Miracle of the Heart

More of the same

2012-02-10T20:56:00.000-06:00

Keegan does have c.diff. Diarrhea to the n-th degree.

Audrey has been super-glued to my side for the last three days. I'm sure the upheaval lately has her legitimately concerned we will leave her again.

Labs this morning looked mostly better. Anemia worsening. Not sure why yet. Electrolytes are still awful. Not unexpected considering the diarrhea, but it meant he had to have a second IV magnesium infusion for the week. Considering the c.diff will likely take a long time to clear his system and that his baseline diarrhea has returned, we have an appointment next week with his GI doctor to discuss going back on TPN soon. Hopefully, it will just be overnight a few days per week.

Audrey threw up in the car this morning. Right as we got on the freeway to go downtown for labs. Nothing like stopping the car on the side of the road to clean-up vomit when you are already running late and still have a 30 minute drive ahead. So far, she has no other symptoms and is happy as long as she is doing what she wants to do. Only time will tell if we are about to ride the virus-flare rollercoaster again.

Keegan is an emotional train wreck. That should probably be italicized and bolded. It could be for a plethora of reasons, but it is absolutely NO fun. He is miserable and making the rest of us feel that way too. Really ready to see my happy Bug again.

More of the same.

Praying next week is where things start to turn around in this house. I hope to get a post up about what we learned in Cinci soon. Things have just been too crazy. Thank you again for sticking by us. It really makes all the difference.

Tuesday too

2012-02-07T20:23:00.002-06:00

After a manic Monday, I could have used more terrific than terrible in my Tuesday.

If you have ever heard the words "c. diff", you probably know what I am talking about. I knew something was wrong as I headed down the hallway (clue #1) to Keegan's room this morning, and when I opened the door, my fears were confirmed.

Needless to say, it was doomed to be a pretty miserable day from the get go. A magnesium infusion didn't help that misery. Hopefully it at least helped his dehydration and magnesium deficiency. Add a possible UTI from little sister at the end of the day (which we think was probably a false alarm after a quick run to the pediatrician)....whew.

I would like a mulligan on this week so far, please. Thank you.

By the way, how is it possible that this little ladybug turned 20 months old yesterday?

Crazy, right?

(Don't ya just want to squeeze her?! Seriously.)

Manic Monday

2012-02-06T21:49:00.002-06:00

Another manic Monday trying to keep Keegan outpatient.

8:30am - Draw last amount of blood that Cincinnati ordered to be sent to a private lab in Canada for testing. Assuming all the paperwork was ok and that we had done this several times, I ask my mom to drop it off at FedEx after she takes Audrey to school.

8:45am - Audrey off to school. Clean Keegan up and throw some clothes on him after his first bad diaper of the day. Head off to Children's downtown for labs.

9am - Stop at Dunkin Donuts drive through for an extra large coffee + expresso shot. Somehow I knew it would be one of those days. Get on the highway before I realize they put sugar in it. Ahhh! Completely undrinkable. Now I'm starting to feel as drowsy as Keegan is. He's already dozed back off in the back seat, after only being awake for about an hour.

9:45am - At the hospital. Get to clinic. Get labs drawn. Still waiting on a return call from rheumatology (which is down the street at Scottish Rite Hospital; 10 minutes from where I am now & 45 minutes from my house) for a post-hospitalization follow-up. Get a call from my mom saying FedEx by her house wouldn't take a "clinical package"with "biological specimens" (despite the fact that I've sent two such packages from there already), so she is headed to downtown Plano to the main office to drop it off.

10:50am - Still no word from rheum, so I head home with Keegan. My mom calls to say she finally got the blood shipped after filling out a second set of paperwork for out-of-country packages.

11:30am to 12:30pm- Arrive home as the phone rings. Appointment with rheumatology at 1pm. Have an hour before we have to head back downtown. Keegan's second bad diaper of the day. Unload the dishwasher, wash syringes; field phone calls from insurance and return emails. Call my mom & ask her to pick Audrey up from school. Keegan finally eats a small lunch...some of which he proceeds to throw up from the retching he's been doing for weeks now as we are about to run back out the door to Scottish Rite.

1pm - Wake Keegan up from his second nap when we get to Scottish Rite for clinic visit with rheumatology. Spend most of the appointment with Keegan curled up in my lap. Until he has another bad diaper. Stand Keegan up. While trying to wrap up our visit with the doctors, I see his diaper has leaked down his leg, onto his shoe and the floor. Learn that most of Keegan's labs are starting to look better, but we need to slow down even more on the steroid wean. Repeat labs on Friday. Leave the hospital not only defeated by the new steroid wean plan but also the need to take Keegan out of the hospital in nothing but a diaper and tshirt.

3:15pm - Leaving Scottish Rite. Get a call from the transplant team that Keegan's magnesium levels are much too low. He needs an IV mag infusion. Seeing that I need to get Keegan some clothes at home, it will probably be at the Legacy ER.

3:50pm - Get home again. Change another bad diaper. Get Keegan cleaned up, dressed, and start laundry. Get a call from team that we can do the 4-hour infusion tomorrow at the Center for Cancer and Blood Disorders.

That only get us to 4pm, but unfortunately, now I need another glass of wine for recounting it all in writing....

Home

2012-02-05T11:37:00.002-06:00

Things got a little busy yesterday, so I wasn't able to post that Keegan actually was allowed to go home in the afternoon. His ferritin level was 4,000, which was a good improvement (still very, very high but coming down). However, his blood counts all continued to drop, while some of his liver numbers worsened also. The hope right now is that these levels are all just "peaking" at a rate slower than the inflammation markers and that they will normalize in a few more days. The blood counts dropping is slightly confusing because steroids should cause them to be artificially high. Steroids generally stimulate your bone marrow to kick everything out, even underdeveloped cells, making all your counts rise temporarily.

There wasn't much we could do about any of these things quite yet except for watch the levels, and we can do that from home. We will recheck labs on Monday morning in clinic. We can do transfusions or start him back on GCSF outpatient if need be by then. For now, there were more risks to being inpatient and picking up a new infection than not drawing labs for one day at home. We will finish the steroid burst today and return to 7mg of prednisone tomorrow.

Keegan is still very, very tired. That is likely to continue for awhile longer. His GI problems are worsening too, which is the opposite of what we've seen in the past on higher steroid doses. Not sure what to make of any of it yet. We will follow up with rheumatology at the beginning of the week. We need to get him settled from this flare before we make any other decisions in regards to his treatment. Since we weren't able to identify a viral trigger, we will probably need to resume the steroid wean and see if he continues to flare each time we get to a certain dose or not.

Thank you for all for each and every prayer said to help Keegan get home once again. We are so grateful. More tomorrow once we know it.

Steady

2012-02-03T22:21:00.000-06:00

Keegan's holding steady with a little more improvement in his ferritin levels today (11,000). The rest of his labs look the same. We went ahead and switched to oral methylpred tonight at the same dose. If he tolerates that over the weekend, his last "pulse" dose will be Sunday morning. Then, he will get no steroids Sunday night. If labs continue to look stable Monday morning, he will go home on 7mg (so essentially, 30mg to 15mg to 7mg over three days) and follow up with more labs in clinic next week. That is a pretty fast steroid wean, so we are praying it goes well. I anticipated we would switch back to prednisone with the switch to oral tonight, but they didn't. We will have to ask tomorrow if he will stay on methylpred/solumedrol for good or just through the end of the pulse.

It's hard to say whether today was an improvement or not in the way Keegan actually feels. He is still waking up around 7:30am and falling back asleep around 10:30am after the first steroid dose. He did not eat breakfast or dinner but managed a bit more than just a piece of string cheese for lunch. What did him in for the afternoon was an attempt to go to play therapy after lunch. Keegan got very excited to go to "Ms. Kristin's playroom", but he only managed about 15-20 minutes of play before he asked to be carried back to his room. Then, he proceeded to take his second nap until 5pm. A brief visit from Audrey (our first since Sunday), and he was asking to go to sleep again by 7:15pm. Remember that this is our boy who dropped his nap almost two years ago!

Anyway, no change in the plan for now. We are praying for good rest tonight and better energy and attitude tomorrow. We should have a phone conference with the doctor in Cincinnati on Monday to flush out what they are thinking based off of everything we have sent so far. We also emailed our team in Boston to see if they had any advice; hoping to hear from them Monday too. Thank you once again for supporting us on this journey.

"Teddy isn't feeing well. Maybe I can help! Dr. Audrey is on call!"

"Let's take your temperature!"

(In your eyeball, of course. Most accurate readings, obviously.)

"Uh oh, Teddy. You have a fever. I think this might be a flare!"

"Aww man, Teddy. I know how that feels. I'm so sorry!"

Watch

2012-02-02T22:22:00.000-06:00

The switch to IV methylpred worked, and Keegan's ferritin dropped to 18,000. The rest of his labs look roughly the same, but we'll take it. The plan remains to stay on IV until we see a bit more improvement. I am hoping that if his labs look better tomorrow, we can switch back to oral prednisone tomorrow evening. Then, we will complete the 5-day pulse before weaning back to 7mg. That is the place in the wean, almost a month ago, that we first started to see GI symptoms. We will stay inpatient at a minimum through a day into the switch to oral to make sure he handles it ok. Maybe a Sunday evening discharge. Then we could follow labs closely in clinic through the step down in dosage. His anakinra dosage will stay maxed out until we decide he is stable enough to go back to the standard dose. More waiting & watching.

I was hoping that the drop in inflammation would help him feel better. I was wrong. He was asleep or fighting sleep most of the day. Still extraordinarily crabby. I poured him into a wagon this afternoon to take a trip outside before the rain comes and stop by the library for some more books. He didn't want to do either of those things but allowed me to pull him around anyway. Then, he wouldn't let me stop. So, we walked at least half an hour around the cardiac and GI floors before he let me take him back to the room. Then he just stayed in the wagon, not wanting to move, watching Nemo, for another hour before he let me put him back in bed. He perked up a bit when Gray came back from work, asking Daddy to build him a tower of blocks. Maybe I'm too boring for him! It was short-lived though. Gray managed to get him to eat almost two chicken nuggets and walk to the nurse's station to say "good night" before he was sound asleep again.

Hopefully tomorrow will be better. We're praying so.

Then again, maybe he just needed to be held by an angel for awhile.

Always there for him, Miss Priss.

Switch

2012-02-01T20:53:00.001-06:00

Keegan's bone marrow laughed in the face of oral steroids. Ferritin rose to 26,000 today. Lymphocytes are nonexistent, but the remainder of his labs, while all continuing to trend poorly, are not worsening at quite the rate we have seen in the past. So tonight we are switching him to IV steroids. We have dropped the "pulse" plan and are proceeding with the methlyprednisolone until further notice. Until we see sustained improvement across the board. Then, we will switch back to oral steroids (dose to be determined later) and will wait to ensure continued stability/improvement before we can go home. The next step would be etoposide, a form of chemotherapy, if the IV steroids do not work.

Outwardly, he continues to look stable. Very tired and increasingly crabby, paler than the bedsheets. But stable. Despite it all, he has managed to keep down a few bites of food here and there, so he's not even on a drip of any kind for once in his life. His heart rate and blood pressure are lower than we are used to, but we aren't sure why. The team is only doing one lab draw per day. Gray and I would like to see at least twice per day of certain levels, but until we can write our own orders, we have to wait until tomorrow to see how things are going.

We received a bit more information from Cincinnati Children's today. Audrey, unfortunately, is not a bone marrow match for Keegan, nor myself or Gray. There are, however, a few possible unrelated matches in the national marrow registry. Again, we are not seeking a bone marrow transplant at this time, but we are doing the initial matching so that we know what our options are if we get to that point.

The team in Cincinnati also asked us to think seriously about transferring Keegan back up to there if he has not shown improvement by the weekend. We are considering it, but at this point, they concur with the recommended course of treatment. As of right now, Gray and I feel that if the treatment will be the same and if they are willing to continue to advise us from a distance, we need to be near our families. The next few days will be crucial in making these decisions. We need to see where Keegan leads us and see how the remaining labs we sent to them come back.

The Lord is going to great lengths to teach me patience lately. For tonight and the coming days, it continues to be "wait and see", "wait and see." Thank you for joining us on the watch.

Pulse

2012-01-31T22:56:00.001-06:00

Keegan's ferritin climbed to 16,000 today, and the remainder of his labs continue to trend the wrong direction (all blood counts have dropped significantly from last week; liver enzymes up; coags up, etc). An expected course for a flare. I have to laugh when I remember one of the cardiologists here commenting to me about routine ferritin checks when we came home from Boston, She said it wasn't necessary because "ferritin just doesn't go up that fast". Wanna bet?!

What it tells us is that the anakinra increase alone is not enough this time, so tonight we are starting him on a 5-day steroid pulse to try to nip this in the bud. This will be 30mg per day, not 30mg per kg, so roughly 1/3 of what we did in Boston when his levels got to 50,000. We are hoping to return to close to his current dose at the end of the pulse, but we will have to see how he responds first. To clarify, published HLH treatment protocol requires 30mg/kg steroids and chemotherapy for levels above 10,000. We are trying a less intense approach this time simply because he seems to be much more stable. Our hope is that if it doesn't work, he is not going to crash so quickly that we have to proceed to the harsher treatments right this minute.

I tried to keep a straight face with the team today when they told us the pulse plan, but my heart dropped through the floor. We were just starting to see glimpses of how Keegan used to look with less fluid retention, "steroid cheeks", and cushingoid features. On top of that, we realized last week that Keegan has not grown at all in the last year. He now falls below the 5th percentile in height, and he won't grow much at all if we have to continue the higher doses of steroids. Not to mention the lovely emotional and sleep problems associated with a pulse. Let me try to contain my excitement...

We know that it is necessary to treat the current crisis first before we can deal with the long-term issues. So, we are trying to focus on that. The current discharge plan is that we can go home once he has been fever-free for 48 hours (beginning this morning), ferritin trending down for 48 hours (not yet), and stable (fluid/kidneys/blood pressure) on the higher steroid doses. Looks like earliest discharge would be Thursday sometime. Outwardly, he continues to not look too bad. Very little desire to get out of bed, minimal desire to play, and very little endurance when we have asked him to walk a bit. But in a better mood today with less agitation. With the fever gone, he has been retching less and actually kept down a few bites of food (not without putting up a good fight to keep it down, bless his little heart). It's mind-blowing to look at how calm he is on the surface, knowing the storm that is raging underneath.

We shipped the labs that Cincinnati requested in regards to this flare this morning. They should have blood in hand by tomorrow morning and results by Thursday. We are hoping to have a clearer picture from them by next week between receiving typing results on Audrey and evaluating the current flare.

Once again, I have to say how words fail us in trying to express our gratitude for your support and prayers for our little family. You renew our strength daily. Thank you.

Feeling crummy.

A blast from the past.

This large framed photo of Keegan (15mo?) was intended for an installation on the cardiac floor.

Ultimately, they picked a different picture of him to use, which still is hung on the unit today.

This one ended up in a storage closet. The nursing staff came across it today and gave it to us to keep.

We asked Keegan who it was, and he responded, "Audrey"!

Better times

2012-01-31T22:23:00.000-06:00

Keegan loves movies. He loves music. He loves to dance. Ergo, he loves Rio.

This was taken last Monday before we left for Cincinnati as we waited on his monthly pentamadine treatment.

Better times. It helps to remember them during the not-so-great times. And this is one to make you smile.

Be sure to watch all the way to the end. It's worth it. Promise.

Relapse

2012-01-30T21:34:00.001-06:00

I know I promised a more in-depth recounting of what we learned in Cincinnati tonight, but I don't have it in me. Keegan's ferritin jumped to 6,000 today, so we officially have another relapse on our hands. He seems stable outwardly with no other symptoms other than the fevers and associated nausea from the inflammation. Chest x-ray and echo were stable. His line cultures and viral cultures for respiratory illnesses are negative so far. Last night, he "simmered" all night but was extremely nauseous (although having to give him IV magnesium probably didn't help that). Because of that though, he got a total of about 45 minutes of sleep, which means I got none.

The fever peaked over 103 this morning, but he has again hovered between 99-101 all day long. If you count Saturday, when he stayed around 100 all day, we are heading into day four. We are going to go ahead with doubling his anakinra dose to see if it helps again. We will probably have to increase his steroids, but we won't do that until we are sure his cultures are negative. The rheumatology team acknowledged today that the anakinra is not going to work for Keegan long-term. They are already thinking about what the next step is. However, the best thing for determining his care plan would be to find out why his immune system is so screwed up. In the absence of a known HLH genetic mutation, the disease process is usually set off by an underlying trigger (generally a virus or an underlying immune disorder/rheumatologic disorder). What is so off about Keegan that would cause such a life-threatening reaction? I have come to realize over the last four years that we will probably never find the answer to that question.

Cincinnati is aware of the situation, and we will probably notify his doctors in Boston tomorrow. It never hurts to have more brains thinking about the situation. Frankly, those two teams are some of the smartest physicians in the world. If anyone is going to "figure Keegan out", it will be one of them. I feel some comfort that we have caught onto this so early, but a lot remains to be seen. We know that over time, Keegan will stop responding to certain treatments. What worked in September and November may not work this time.

Care coordination between the team here in Dallas is still meager at best. Some people are putting more effort than others into it. So, we are doing our best to keep everyone on their toes for Keegan's sake, even at the risk of seeming pushy or demanding. We often get the feeling that certain doctors don't grasp the gravity of the situation because they didn't witness the worst of it and haven't seen anything like this before. We are beyond caring what they think of us. I will go to the moon to make my son better if that's what it takes. This boat is sailing - get in or get out. We don't have the time or patience for anyone who wants to sit on the dock waiting to make a decision. We are trying to remain calm and on top of all the information to make the best decisions for Keegan, no matter how rocky the waves get. We just pray that every one of the doctors join us in that desire.

Labs will be repeated tomorrow, and we'll continue to wait and watch. Thank you again for your prayers. We will update as we know it.

Then they cried out to the Lord in their trouble,

and He brought them out of their distress.

He stilled the storm to a whisper;

the waves of the sea were hushed.

They were glad when it grew calm,

and He guided them to their desired haven.

Psalm 107:28-30

We're back...in more ways than one

2012-01-29T22:49:00.002-06:00

Yes, we are back home from Cincinnati. Arrived safe and sound on Friday afternoon.

Unfortunately, we are also back in the hospital here in Dallas. Keegan was admitted to Children's downtown today after he started spiking fevers again. He doesn't show any signs of having a virus, and his labs drawn today don't look like a central line infection. Still, we have to draw cultures and run IV antibiotics for 2 days minimum while they brew to rule out a line infection for sure.

I apologize for not updating about our trip sooner. Due to space considerations on the tiny puddle jumper of a plane we took, I decided to unplug for a bit and leave my computer at home. I am editing a few pictures and hope to get a more detailed post up about how it went and what we learned some time tomorrow.

Until then, I will answer the burning questions I know everyone is dying to ask. First, we are proceeding with typing to see if Audrey is a bone marrow match for Keegan and if there is anyone currently in the national registry that is a match. We want to have that information ready to go if needed, and we should know what the results are by the end of this week or beginning of the next. However, we won't be proceeding to transplant right away... if ever.

There are a lot of reasons why and what we are still waiting on, which I will try to explain in a more comprehensive post tomorrow. Without an identified genetic mutation for HLH though, the team said they would want to see him flare again before making decisions about transplant. Well, ask, and you shall receive. Keegan's ferritin level spiked to 869 today from the 100s end of last week. We don't have a plan from our team here yet, since it's the weekend, and none of them are around. You better believe we have already contacted the team in Cinci for guidance on the proper steps if this does indeed turn out to be a flare. We will need a few more days to pan that one out unless something extremely glaring pops up. I told Keegan that I was pretty darn sure that when the doctors said they wanted to see him relapse before taking the next steps, they didn't mean the day after we got home!

For now, Keegan is pretty lethargic and easily agitated, but he is stable and on the cardiac floor for now. He responded somewhat to oral tylenol today, so we haven't had to bring out the big guns for fever just yet. I was asked tonight if I had a gut instinct whether this was Keegan relapsing or something else. I'm not really sure. It's hard to argue with some of the numbers, but this is just a little different than before. We know the drugs he is on will mask some of the old symptoms, which just makes it even harder. But he doesn't have any obvious symptoms of an infection, so that seems to support a relapse too. Only time will tell now.

We do appreciate your prayers for Keegan at this time. We were hoping to not have to takle any of this just yet. Keegan's never been great at letting us have time to prepare though. Not sure I would have expected any different from him now. Thank you again, and we'll update soon.

Deep breath

2012-01-23T21:55:00.000-06:00

I think we are ready to leave for Cincinnati tomorrow.

I hope we are ready.

I keep telling myself that we will be ok, no matter what happens, but I'm not convinced of that yet.

But here we go anyway. Lord, be with us.

Kidneys, colds, and catching up

2012-01-19T21:47:00.000-06:00

Kidneys: Keegan had a decent nephrology appointment last week. His kidney function labs and urinalysis have stayed stable, but his blood pressure has not. You may not be aware, but more often than not, your kidneys are the culprits with high blood pressure. Since we just did a heart cath in December, we are more confident that Keegan's hypertension is kidney-related. The nephrologist was concerned that one of his three blood pressure medications (four, if you include diuretics) could cause long-term damage and exacerbate his potassium deficiency. So, we more than doubled another one, which helped just a itsy bitsy smidge, in hopes that we will see even better numbers and be able to reduce or stop the more dangerous medication. We will have to watch his blood pressure, urine output, and weight closely over the next few weeks to see what does or doesn't work. The doctor acknowledged the need to repeat his kidney function testing, but he wants to wait until we have a plan from Cincinnati.

Colds: I am sick of snot. UGH. I want to fumigate my house with antibacterial spray. Audrey picked up yet another cold last week at school. Once she started in with the runny nose, I started us all on zinc and followed her closely with lysol and hand sanitizer. It was all for naught, I'm afraid. One by one, we all fell. I seem to be the last one to go down. Audrey is still just a touch runny. Keegan handled it very well. I actually think his steroids and daily claritin helped this time. We are praying HARD that this has left us by our trip next week.

Catching up on the rest: We really loved the developmental pediatrician Keegan saw at Scottish Rite last week. He really blew me away, and we are looking forward to continuing to work with him. He is going to refine the list of available behavioral therapies to really tailor to Keegan's needs. I will post more about that as we get more information. He also felt there wasn't a need to rush right into anything until we have a solid plan from Cincinnati.

It's been another typically busy week in addition to doing last minute planning for the trip. Keegan will have labs drawn on Friday. Since the last steroid wean, his GI problems have worsened, and he has begun complaining more of joint pain. It should be interesting to see where we stand this week.

Thank you all for your kind words, support, and encouragement after the last post. We feel stuck in a surreal time warp right now. Much like when we left for Boston, it is difficult to look at Keegan on a good day and convince yourself how sick he truly is. So much is in limbo right now. Until we have a plan from this appointment, our whole life is suspended. Are we to resume as much of a normal life as possible because there will be no transplant on the horizon? Just waiting and watching breathlessly every day for him to get sick again? Or are we waiting on news of a match to proceed with a transplant? When? Where? What we do until these decisions are made seems rather pointless.

Luckily, we don't have much longer to wait...although the days are starting to feel like years. In these days of anticipation, fear, and anxiety, we find help when we turn to the Lord. I wrote last that I've found even when my faith is at rock bottom, just lifting my hands in prayer never fails to bring me the comfort and mercy I seek. Lifting your hands can be done in worship, praise, supplication, but also as a sign of needing comfort, of needing love. I realized this the other day when Audrey fell and then ran to me crying, arms lifted as a sign to pick her up, to love on her and help her feel better. Lifting our hands in prayer is the same. A way of saying, "carry me, Lord. I can't do this myself."

I know that each of you who reached out to us in the last week are there to help provide the earthly hands of comfort and love that we need during this time. So once again, we thank you. Thank you so much for being there for us during these uncertain times. It means so much more than we can say.

Lifting my hands

2012-01-11T23:11:00.003-06:00

Since my avoidance issues have prevented an update on Keegan's health for so long, we will once again have to resort to our friends, the bullet points.

Keegan's last set of labs were very stable, and his HLH markers were actually a touch lower too. We are trying to stretch out time between labs, so if all else seems ok, we won't recheck until the 23rd! We have continued to wean Keegan's steroids by a paltry amount every other week, but at least we're still weaning without too many big hiccups.
He has been off of tube feeds since before Christmas and mostly maintaining his electrolyte levels with a few tweaks and supplements. Since the last steroid reduction, we've seen some of his old tummy issues start to come back, but we're not ready to draw conclusions from it.
Keegan has an appointment with his nephrologist tomorrow. His kidney numbers have looked okay, but we haven't been able to reduce his diuretics or blood pressure medications. We're anticipating a repeat of the kidney function test we did last year sometime in the near future.
Friday, he will finish his developmental/autism testing at Scottish Rite hospital. The neuropsychologist at Children's did the initial testing at the beginning of December, and the developmental specialists at Scottish Rite will help us round everything out. I didn't ever really explain all that went into the autism diagnosis. Essentially, the neuropsychologist reviewed all the brain MRIs Keegan has ever had and used them to interpret the examinations and testing that she had Keegan do. Ultimately, Keegan has suffered some extensive brain damage over the years from hypoxic events (lack of oxygen to the brain from his surgeries, ECMO, heart defect, etc), strokes, and the increased pressure on his central nervous system from inflammation due to HLH. Much of Keegan's "autistic" behavior can be directly related to a specific medical issue, however the autism label will help us get the best treatment and education for him. All of that is currently taking a backseat to his medical issues though.

Which brings us to our trip to Cincinnati. I haven't explained much about that yet either.

We have an appointment on January 25th to meet with the immunodeficiency and bone marrow transplant team at Cincinnati Children's Hospital, the world's leading experts on hemophagocytic lymphohistiocytosis. They have been researching Keegan's case and running many additional tests in advance of our meeting. Audrey has an appointment on the 26th to be tested to see if she is a possible match for her brother. There is no doubt we are taking Keegan where he needs to be for the best decisions to be made for his future. But we can't help feeling anxious about it. Mostly because there isn't really a good outcome from this.

No bone marrow transplant would mean that there is nothing more we can do essentially. The cocktail of drugs he is on now is not something he can stay on long term. Not even for another full year. And yet, there are few drugs remaining that hold much possibility for helping him. As "stable" as he appears to be on the surface, he is far, far from it. This is the high side of the wave - the crest. When it will crash down is anyone's guess.

A bone marrow transplant may ultimately "cure" his HLH but won't affect his status as a heart recipient and all that comes with it. Nor would anyone wish a BMT on their child any more than a new heart. The chemo, the complications, the aftermath. It can go well or horribly wrong. That is, if we can even find a match for Keegan. No one seems to know off-hand if it will even be possible given the fact that he has already had one donor that alters his body's antibody make-up.

Just like with much of the last four years, there is no crystal ball to point us in the right direction. No matter what we end up doing, it could be wrong. We're starting to face questions that are less about his health and more about maximizing our time with him.

I suppose I've been avoiding posting about all this information because not putting it in writing meant not admitting it to the world. Not admitting it to myself. I know the odds; I know the options that await to us. We are doing everything we can to take advantage of every minute of apparent health, no matter how superficial, and every day that Keegan has a smile and is feeling ok. But the crushing feeling that it is all fleeting overwhelms us at the end of each day.

It should seem fairly obvious that my faith has wavered over the last few months. The few posts I have managed here don't hide it very well. Every night as Keegan falls asleep, I fight back tears as the uncertainty of his future swirls in my mind. Some nights, I can't summon the strength to fight them. I can't lie about that.

But somehow I have to find the strength to put a smile on each morning when he awakes. As his mother, I have promised him that I will fight for him. That I will soldier on, and that I will do my best to make every day one where he feels at peace, no matter what tomorrow will hold. The only way I can do that is by trusting that God has made the same promise to me. He will not forsake us. He will carry us and provide the strength that is needed each day.

What I've come to realize lately is that I don't have to feel overwhelmed by His promises to know that they remain. Faith does not always mean that my worries will be erased forever. Grief and pain are human nature. Hebrews 11:1 says, "now faith is confidence in what we hope for and assurance in what we do not see." I have faith in this promise that God's plan is bigger than my son's life. He will make this glorified, of that I have no doubt. It doesn't necessarily make living in it day to day any easier, but in hindsight, my faith gives me that comfort. Even if I never see that promise come to fruition in my lifetime, it does not make it any less true (Hebrews 11:39).

Lately though, my faith has not even been as strong as the paragraph above was easy to write. Sometimes faith is even more basal than that. Sometimes I just have to put my knees on the ground, and I can find the strength to stand back up again. It is not me that takes that step. He does it for me. The mere act of being there, of lifting my hands in praise, reminds me of this great love. I can be completely honest and human with God and expect that His response will be no different. I can pour out my disappointment and despair. That's when He loves His children the most. When I don't think that I can make myself believe it anymore, I can do this one act and His mercy comes rushing back. And damnit, even if that is all I have in me for the day. It will be enough. He will always be enough no matter how this ends.

"I Lift My Hands" - Chris Tomlin

Be still. There is a healer.

His love is deeper than the sea.

His mercy, it is unfailing.

His arms, a fortress for the weak.

Let faith arise. Let faith arise.

I lift my hands to believe again.

You are my refuge. You are my strength.

As I pour out my heart, these things I remember.

You are faithful, God, forever.

Be still. There is a river.

That flows from Calvary's tree.

A fountain for the thirsty.

Pure grace that washes over me.

Let faith arise. Let faith arise.

I lift my hands to believe again.

You are my refuge. You are my strength.

As I pour out my heart, these things I remember.

You are faithful, God, forever.

Winter swim break

2012-01-09T22:19:00.000-06:00

Once a week, we take the needle out of Keegan's port to put a new one in. While he is "accessed" all week, he can't get the dressing on top of the needle wet. We have to cover it with plastic wrap and oodles of tape just for a small bath each night. Between his multiple ports and PICC lines and spending most of the summer inpatient, he only got to swim once in 2011.

One night in the tub, Keegan started asking Gray if he could swim. Not sure why or where it came from, but he kept asking and asking. Gray's grandmother lives in a senior living community here in Dallas that has a wonderful, clean, indoor, heated pool. She was able to reserve a block of time in the pool this past weekend for the kids to take an unexpected winter swim break while Keegan's port was deaccessed on Saturday. Needless to say, it was a welcome surprise, and both Audrey and Keegan had a great time. Thank you, Granny!

Well, it's a new year

2012-01-02T22:56:00.001-06:00

Not really too excited about it.

I'm absolutely ready to give 2011 the boot.

2012? I'm not really sure what to think of you yet.

There are so many things I need to explain. So many things I need to process. So many things I need to come to grips with...and quickly.

And yet, I find myself in perpetual denial.

January 24th at approximately 12:30pm, our family of four will board a plane bound for Cincinnati to meet with the HLH and bone marrow transplant team there find out what this year will hold for us.

Sometime before then, I will get it all together. I hope. No promises.

Until then, thank you for your patience and prayers.

In 2012, I'm afraid we'll need them more than ever.

Audrey goes to the aquarium...and the ER

2011-12-29T22:48:00.000-06:00

My sister, Alex, is off of work until next week. So we decided to try a fun outing with the kiddos yesterday and head to the children's aquarium at Fair Park. It's small, not too much to see and not too crowded compared to the World Aquarium downtown.

Keegan is a big Nemo fan, so we thought it would be a hit for him. Not so much. He wanted to grab fistfuls of gravel from the horseshoe crab tank in the entry and soaked his tshirt. He had no interest in the fish, turtles, or alligator but had a meltdown when we told him we had to move on from the empty tank he was apparently fascinated with. Audrey did a lot of pointing, running, and "wow"-ing.

The last stop was the sting ray and shark tanks. Keegan was mesmerized by the rays, as was Audrey by the sharks. She especially loved it when the tiger sharks would swoosh by really fast and spook her. One really caught her off guard, and she stumbled backwards a bit. It was a slightly awkward fall. Audrey didn't even seem that upset, but she wouldn't let me put her down at all. We had some lunch and put her down for her nap at home. When she woke up, she was just beside herself whenever we asked her to stand or walk.

And so, Audrey made her first trip to the Children's ER. Of course, the ER staff at Legacy are our good friends (sad, no?), so Audrey received a LOT of attention at first. A quick exam said she likely had a toddler's fracture. X-rays were ordered, done, and surprisingly, negative for a break! Just a sprain or pulled muscle of some kind. One dose of motrin, and she was toddling out the door. Waving and blowing kisses. That was one really expensive dose of motrin, little girl!

We were instructed to give her scheduled motrin for 24 hours, but I didn't even give her two. She is more than fine today. Truly never a dull moment in the Harrison house!

It was interesting to be there though with her. Driving onto the campus at the speed limit. Not compulsively checking the ambulance bay to see if there is a transport team on hand. Actually waiting in the waiting room for her name to be called, instead of the receptionist seeing us run in and just immediately taking us through the doors to triage or even straight to a room. Knowing that no matter what, we would be walking out the door to go home in a few hours at most.

It truly was different. And refreshing. So thank you, Audrey. For showing us that life can have just normal, everyday childhood moments that can be weathered with a few extra hugs, kisses...and just a little motrin.

Christmas picture overload

2011-12-27T23:15:00.000-06:00

We were extremely blessed to be able to spend this Christmas with our families.

It was a very busy few days, but the kids handled very well with many smiles all around.

Santa was certainly good to Keegan and Audrey this year!

Christmas Eve at Mamie & BD's house:

A new Gator for Keegan!

Unfortunately, the weather was horrible, requiring him to learn to drive (without hitting too much stuff) in the garage.

This will get much love and use as the weather improves.

Snack time with cousin Maya

Luckily, this year we had a few ideas for helping Keegan when he became overstimulated.

Bear hugs and leaving the fray to escape into a video helped immensely.

Playing monkey with Aunt Alex

Rock Star Mickey was a HIT!

Christmas morning at HOME:

A new DVD player with headphones for each kiddo in anticipation of spending more time on the road this year for Keegan's appointments.

The best playhouse ever! It's felt, draped over a folding card table. So easy to store!

A highchair for her baby dolls

Testing out some new MobiGo games

Sneaking in some time on Keegan's cash register.

He has been obsessed with coins (fake, real, anything that's small and round, actually) for the last several months. So, Santa played into it with a play cash register. He loves it so far!

A Christmas morning Sic'Em!

(Gonna have to try to restage a Christmas pj picture. Couldn't get much cooperation this day. See video in the post below for proof!)

Come play, Keegan!

Christmas morning at Oma & Pops' house:

A bounce house just for them!

Keegan wanted it blown up right NOW!

Merry Christmas from Santa's lil' helpers!

Ho Ho Ho!

2011-12-27T22:59:00.000-06:00

Merry Christmas!

2011-12-25T23:47:00.000-06:00

From our home to yours,

we pray that your Christmas was truly merry and bright.

We are so thankful for spending this blessed holiday together with all of our family.

*******

And this will be a sign unto you, you shall find the babe wrapped in swaddling clothes and lying in a manger.

And suddenly there was a with the angels a multitude of heavenly host, praising God and saying"

"Glory to God in the highest,

And on earth, peace and goodwill toward men!"

Luke 2:12-14

Comic relief

2011-12-22T22:14:00.000-06:00

Audrey is 18 months old. I know! I can't believe it either!

This little girl is a ham-and-a-half, the comic relief in our household. (And thank God for that, right?!)

Audrey is doing very well. She still eats well most days and had a nice little growth spurt to show for it. Up to 42 percentile in height! She is sprouting her last baby tooth at this very moment. She babbles constantly with quite a few identifiable words. I honestly believe Audrey is a perfectionist. Just like her walking, she is going to wait until she has down pat and just bust out with it.

Audrey is a little lovebug. When she can tell you need a hug, she lays the best one imaginable on you...even if you didn't know you needed it. She really loves to tackle Keegan and cover him with hugs and kisses. He could care less, but Audrey obviously thinks it's the best thing ever. I really wish he knew how much she loves him.

We sure love you, little Ladybug! Thank you for keeping our days merry and bright!

(And yes, more detailed info on Keegan is coming soon. In the midst of trying to orient ourselves in the autism world, we are still trying to manage his medical issues. The last of the send-out labs are being run, and we are planning our first trip to Cincinnati Children's to meet with the bone marrow team there on January 25. Merry Christmas to us, right?)

Christmas lights...and thanks

2011-12-20T22:34:00.001-06:00

Let me start out by saying a big, heartfelt thank you to all of you for your support and outpouring of love in response to Keegan's autism diagnosis. I promise to update more about all the testing he went through and the doctors' decisions soon, but we are still processing it all ourselves. Gray and I have a lot of mixed feelings about it. For now, what's important is the love and support our family and friends as we sort it all out. And we could not have been more overwhelmed by it all. It is easy to begin to feel that people are used to Keegan being sick and don't want to hear it anymore. This year has been one of the toughest ever, and to be honest, our spirits were sagging terribly as the holidays approached, especially upon hearing the extensive brain damage Keegan has suffered thus far that has contributed to his autism. Your kindness and love has renewed our resolve and given us hope as we face another year that is posed to be just as tough on our little boy and family. So, thank you, once again. We simply can't say it enough.

Here's another thing that brightened our day. A carriage ride to see the lights in the neighborhood south of us. Our friends and neighbors, the Loredos, hosted us all in this evening of holiday fun. As expected, Audrey adored it. She picked a seat in Daddy's lap right up by the horses and literally danced and jumped with joy the entire hour. We couldn't tell if it was all excitement from the lights, the horses, or both. But oh boy, am I excited that horseback-riding may be in her future!

Anyway, I was very nervous because our ride was scheduled during the kids' normal dinner and bathtime. Keegan is very dependent on routines, especially at night. I wasn't sure how he would do with such a major disruption tonight. Overall, he had little interest in the lights or company but had no major meltdowns or problems. We were thrilled with the whole experience overall! Thank you, Loredo family, for such a treat! Merry Christmas!

Our ride for the evening. The horses names were General and Patton. Classic.

A nice try at a family pic. Ironically, a smirk from K and a tantrum from A!

She did not want to stop talking to the horses and her friends to take a picture.

And Keegan cracks me up with that candy cane...he had no idea what to do with it, but it entertained him for a little while.

All bundled up and ready to ride.

Daddy and his troublemakers

"Wooowww!"

This one was in such overload by the time we got home!

She wanted to stand backward on the recliner and "ride" like she had in the carriage instead of eating dinner. Umm, don't think so, little girl!

The tantrum got to be so much that the only thing that calmed her down was some veggie puffs and the Grinch on tv. Not my best dinner moment as a mom. Ultimately, she did eat some PB&J though, so I didn't feel SO bad! Even if I did have to hand-feed her the sandwich and hold her cup for her while she drank. Has me wrapped around her finger much? Yeah.

And just a pic to make you smile. Crazy kiddos totally staged this themselves.

(On a sad note though, this is a horrible picture of Keegan. It hurts my heart to see this transformation. We are SO ready to say goodbye to these stupid steroids and see our Bug look like himself again.)

Do you know Keegan?

2011-12-17T22:44:00.000-06:00

He has autism.

Just one more diagnosis. A pretty big one.

But it is not him.

He is still Keegan, our amazing, strong, courageous, resilient little Bug.

And we love him.

Do you?

Trains

2011-12-16T22:39:00.000-06:00

Well, we've had a pretty crappy week. In too many ways.

Since I don't want to go into all that tonight and to have a better ending to the week for the kids, we made one last attempt at a Christmas outing before school lets out, preventing Keegan from going anywhere public. We started with a quick lab draw at the hospital and then snuck over to see the Trains at NorthPark. Proceeds from the exhibit tickets proceed the Ronald McDonald House, and our transplant friend, Leah Grace, had the honor of presiding over the opening ceremonies a few weeks ago. Keegan was not thrilled with this break in his routine at first, but once he saw them, he warmed up to the idea. He and Audrey both had some "oohs" and "ahhs", a nice end to the morning and week. I didn't bring my big camera, so phone pics will have to do!

A bigger post will follow, but we would appreciate your prayers for our family at this time. Thank you so much.

Audrey & the Advent Calendar

2011-12-13T21:22:00.000-06:00

Rockin' the dress, slippers, and Christmas book as a purse look.

These little doors are so fun to close again every day and watch Mama have to reopen them.

Me: "Audrey, that's your Advent calendar. Your chocolate at dinner each night helps us count down to Christmas!"

Audrey: "What? I didn't know that!"

Oh, that makes me happy!

Merry Christmas!

Breakfast with Santa

2011-12-11T22:58:00.000-06:00

Yesterday, Mamie and Big Daddy treated Keegan and Audrey (as well as me, Gray, and Alex) to breakfast with Santa Claus at Neiman's. Alex and I did this every year growing up, and we have been looking forward to being able to do it with the kids. We had a secluded table at the back so Keegan wouldn't be too exposed. All in all, it went well. Audrey is on a protein craze, and I'm pretty sure she was the only kid present who finished all of her sausage and then some. Sheesh, that child has eaten so much meat in the last week or so, it's unreal! Keegan enjoyed the Mickey-shaped pancake and fruit. He isn't much of a breakfast-eater outside of yogurt and a banana, so we were very pleasantly surprised not only with his eating but also with his mostly pleasant behavior most of the time we waited.

Audrey seems rather fascinated with all the Christmas trappings. She can identify Christmas trees, Santa Claus, reindeers, angels, and baby Jesus in books. She will point to lights and say, "oooohhh". (Quick aside - Ashley F., this reminds me SO much of Karis's "so prrrrreeeetty"!) Obviously, this doesn't mean that Audrey "gets it". Far from it. But I do love how much she is entranced and enthralled with the entire season. It's something we have yet to see from Keegan. He can take Santa references in small doses. He adores the classic Mickey Christmas specials and his Curious George DVD, "A Very Monkey Christmas". Keegan sings part of "Jingle Bells" because Donald Duck sings it in one of the episodes...not because the carol itself brings him joy of the season. While we pray and talk about Jesus all year long, especially at Christmas, so far, it seems they are just words to him. Though his normal speech is scattered and often unintelligible, Keegan's insanely accurate memory means he can recite rather clearly the Our Father, even if it doesn't make sense to him. He could take or leave Christmas and has no idea that it could mean new toys on Christmas Day, although new toys don't necessarily mean much to him either. None of this is for lack of trying. We try to explain it to him in simple, brief ways. Every. Single. Day.

I was aware of Keegan's Christmas oblivion almost two years ago, but this year, at 4 years old, it seems much more pronounced. At four, I can see the other kids so clearly "get it." Maybe not the true meaning of the season but the idea, the splendor, the magic, the joy. They make lists; they wait anxiously for Santa. They have Elves on the Shelf and sing carols and hymns in school programs. They know that baby Jesus was born, whether they know why or not. There are lights and decorating and baking cookies and so many fun things to do. It's the tip of a very, very, very large iceberg with Keegan that I am finally struggling to accept. Over the last two years, the gap between where he is and where others his age are has grown considerably. (We are working on a lot in this area, which I will attempt to update about later this week.) It's just so blatant at this time of year that it makes it very difficult to really enjoy it. My dreams of sharing so many fun activities and magical memories with my child aren't possible yet. And that's heartbreaking.

But back to the post at hand. Breakfast with Santa. Done. Enjoyed. And without too much drama. Audrey was in awe and after some prodding, Keegan gave Santa a hi-five. We ate first and then waited a bit to have a formal picture made. By our turn, Keegan was done, and Audrey had no desire to stop playing with the fun toys she had found to stop for a picture. We did get one that I will try to get Gray to scan at work this week for posterity's sake. Thank you, Mamie and BD, for giving us this one precious moment of normalcy to this holiday season thus far. It was wonderful.

Daddy and his Ladybug

Say what? Santa's coming, and I have chocolate on my face! At breakfast? Oops!

Love pictures of this boy eating. A long time coming.

Bellies full. Waiting for the Big Guy to make his way to our table.

It's really you, Santa!

Why, yes! I have been good. Thank you!

Put 'er there, buddy.

As was his response last year, Keegan said "no" when asked if he wanted Santa to bring him anything special. Makes someone's job easier, if nothing else!

Shucks! I needed more time to go over my list!

A new Hummer perhaps.

Or a Mercedes.

Foosball, anyone?

Or shall I serenade you with Christmas carols?

Results & pictures

2011-12-04T22:38:00.001-06:00

Keegan did pretty well through his cath and cardiac work-up last Thursday. His biopsy results were great - zero evidence of rejection. His antibody levels have weakened, and his coronaries show no further damage from the antibody problems of the late spring. All wonderful news! We were able to come home after a 12-hour stay on Thursday. Whew! More labs on Friday of this week, so hopefully, the good news will continue.

With lots of help from Gray and my sister, we got all the Christmas decorations, including the tree up this year. I'm very thankful for dreary, rainy weather that made it easy to stay inside and get it all done. It is such a pain to do, but I am glad we did it. The look on Audrey's face when she awoke from her nap to see the tree decorated and lit up was priceless. Her favorite ornament so far is our Christmas Elmo. I put it right at her eye level because I knew it would be. She definitely deserves the opportunity to enjoy all the decorations after not having much of a first Christmas last year. Now, Keegan just has to stay home so we can all enjoy it together!

I realized I never posted the rest of the Thanksgiving pictures. I don't know why this surprises me. I promise to get this all together sometime soon. It's just a tad overwhelming at the moment. In order to preserve these family moments here, we will turn the clock back a week and a half momentarily. Hope everyone has a wonderful week ahead.

Hugging...choking....all the same difference.

Technically, this photo was from before Thanksgiving, but I had to share my little turkey with everyone.

Steroids and family photos don't mix.

Playing with Marley and Maya

Audrey and Poppy

Mark and Gray

Chum and Mamie kicking back and relaxing after a super meal.

My mom makes the best Thanksgiving dinner ever. Just sayin'.

Big Daddy and two silly kiddos

Cath tomorrow

2011-11-30T22:04:00.000-06:00

I'm sorry for the delay in updates. I needed to take a short break from posting, but I'm hoping to get back into it soon.

Not tonight though because we have to be downtown at the hospital with Keegan at 7am tomorrow to be admitted for a heart catheterization and biopsy. The whole work-up. He was supposed to have had this done about a month ago, but his relapse delayed it. We are hoping that Keegan will get to come home tomorrow when all is said and done. However, he has been through a lot recently and thrown us a lot of curveballs. It wouldn't surprise anyone if he needs a night inpatient to recover from this.

We had a nice, quiet Thanksgiving and are back into the craziness that is Keegan's appointment schedule. Keegan has been pretty stable. His labs look better. He is back down to one dose of anakinra and even restarted the steroid wean on Tuesday. Unfortunately, we had to double his steroid dose today through Friday. With chronic steroid use at such a high dose, your body stops producing "stress hormones" in response to an injury like a surgery or heart catheterization. So, we have to do it for him artificially, as well as with a pulse dose while he is under anesthesia tomorrow. The good news is that if everything looks ok, we should be able to go back to the current dose by Saturday. It just makes for an even more miserable Keegan in between.

We have also been contacted by the HLH specialists in Cincinnati. We have completed all their paperwork, and once they have his medical records, they want to see both Keegan and Audrey. Keegan to review his history and complete a consultation with us. And Audrey to see if she is a possible bone marrow match for him. This does not mean they think it is necessary for him. We don't know that yet, but it is prudent to go ahead and see if our best chance for a match, Audrey, is possible. That being said, there is a LOT of paperwork for them to go through. We don't know when we will be going up there. Hopefully not until after the holidays, but if they said, "be here December 23rd", we would.

I'll wrap up this post the way I started...with an apology. I'm sorry for how unorganized and scant this update is. I will get back into the groove soon. I hope. Until then, we appreciate your support and prayers for Keegan's stability and health, as well as for wisdom and clarity for his team of doctors. Thank you so much.

Thanksgiving

2011-11-24T21:21:00.000-06:00

Home and together with family and marvelous food for Thanksgiving. Just about everything we could ask for this year.

We pray that it was the same for you and yours. I am very well aware of how many blessings have come our way this year. Unfortunately, it doesn't take away my fear of what is ahead. On this night one year ago, our world came crashing down, as Keegan spiraled into complete kidney failure. The last 12 months have been one struggle after another for my sweet boy. The next 12 could be just as hard. But we are surrounded by loving family and dedicated doctors and nurses who are trying every day to help make sure that Keegan is with us for many more days like today. Hug your family. Tell them how much you love them. You never know what tomorrow might bring. A day like today makes it all worth it, no matter what may come. Thanks be to God, our loving Father from whom all blessings flow!

Pesky gallbladder

2011-11-20T20:29:00.000-06:00

How does the saying go? If it's not one thing, it's another? Yeah.

So, last night Gray and I ended up leaving an absolutely lovely wedding reception (congratulations, Seth & Sheradon!) to go to the ER because I had a gallbladder attack. This was my first one. Not fun....not fun. We weren't sure what to think at first. The ultrasound done in the ER didn't show any stones, but it may be early. Right now, we are taking a wait-and-see approach. Going to do a little research so that I have a doctor to call if they start to become more frequent. Pesky gallbladder. I do not have time for you!

In other news, Keegan is doing about the same. Eating a bit better. We have dropped him back down to one dose of anakinra a day and will recheck labs on Wednesday. If all is going ok, we will restart the steroid wean the week after Thanksgiving. We did go ahead and email the HLH specialist in Cincinnati on Friday and are anxiously awaiting word back. We will surely update when we do.

A busy week ahead for our family, as I'm sure it will be for most everyone. For now, we are thankful that all four of us are home and stable and for every prayer said to help us stay that way.

And for crazy after-nap hair. Yep, definitely thankful for that!

Playdate

2011-11-18T22:18:00.000-06:00

Today, we had a little playdate at the Arboretum with our friend, Rylynn. We are so thankful that Keegan and Ry are both outpatient. Our fervent prayer is that they both stay that way, so that we can have more mornings like today.

Disappointing news

2011-11-14T21:55:00.001-06:00

1. Keegan's ferritin level is down (which is encouraging) but not near what I would have expected with double doses of anakinra all weekend long - 500. I don't have the full set of labs yet to know the whole picture, nor did I hear from the rheumatologist today. My frustration and disappointment with this recent flare is difficult to put into words. I am reluctant to put any of it out here until we have spoken about it all with the team. Right now, that's what is needed. We need to meet with transplant, hematology, and rheumatology to make the next decisions in Keegan's care. Gray and I really feel like the rheumatology approach is not working well enough to control this disease process, but we are unsure where that leaves us as far as daily management goes. Tomorrow was supposed to be a scheduled reduction in the steroid dose, which is still at an enormous dose for his size. Something tells me that won't happen. Having to stop the steroid wean is going to be killer. He is already not scheduled to be off them until February! Hopefully we will have more information tomorrow.

2. We had to restart Keegan's tube feeds today. His electrolyte levels were all sorts of screwed up. His eating has not recovered since the virus, and frankly, it may not. We anticipated his appetite and GI problems would get worse as the steroids were weaned, and this may just be the first step in that process. The tube feeds will be twice a day to start and should help normalize his levels. That is helpful because the deficiencies were starting to cause him some severe cramping and pain. I hope it will help some of the stomach pain he has been having lately too. Keegan has been constantly complaining about his tummy and has been retching all day long. It would be nice if giving him some nutrition with an easier-to-digest food through his tube helped both these problems. However, it was nice to be tube free for awhile. I'm not looking forward to it's return. Tube feeds are better than TPN, I guess.

Thank you for all the kind words and encouragement today. This is a very difficult and uncertain time for our family. It means the world to have such loving support as we slowly work our way through it. Thank you. More as we know it.

Update...kinda

2011-11-13T22:57:00.000-06:00

I honestly have no idea what to title these posts anymore.

As anyone who has read this blog for any piece of time has probably noticed, I am not really in the blogging/journaling "mood", as it were, lately. There are a variety of reasons. None of which I really feel like addressing tonight. Actually, that's probably a good enough description of why I haven't been writing much. I don't really feel like addressing any of it. It's a cycle, ya know. It's sometimes difficult enough to live it. I don't always feel like hashing it out in the written word at the end of the day.

All that cryptic nonsense aside, Keegan seems pretty stable to the naked eye. That's really one of the more disturbing pieces of all this. A child with a ferritin level of 15,000 shouldn't be ambulatory, let alone playing and acting mostly "ok". So the fact that he is home and looking about the same has us a little unsettled, to say the least. We have given him a double dose of anakinra all weekend. We will go in bright and early for labs in the morning. Fingers crossed. He can't stay on the double dose more than for this weekend, so we are praying it worked. What will work from here on out is anyone's guess.

Literally. I don't think anyone has an idea of what his long-term maintenance looks like at this point. Simply maddening for a type-A, planning personality like me.

Keegan is still very tired, is eating minimally at best, and having worsening diapers. He really seemed to feel cruddy all weekend long. I won't be surprised if his inflammation levels have come down with the extra meds, but what does that mean for my ability to monitor him closely enough for flares at home? What is even "normal" for this child anymore? If Tuesday his levels were dangerously high but he acted fairly normally, what on earth are we supposed to think when he actually doesn't act like himself?

Italics are so not enough to do that last question justice.

But I digress.

I do that alot lately. Sorry.

In summary, I suppose this "update" doesn't tell you much at all. Keegan stayed home. We are trying to enjoy it as long as possible. We will see what the team thinks tomorrow after his labs all come back. That's about all we can do right now. I feel like a lot of decisions should be made this week, but I'm not sure they actually will be. Another week. Please, Lord, let it be an uneventful one! More tomorrow...

Just enough!

2011-11-10T22:50:00.001-06:00

Keegan's ferritin was down just enough to keep him home!! A better post to follow with details. Thank you SO much for all the prayers. We are so grateful for God's healing hand and the love of family and friends.

- Posted using BlogPress from my iPhone

Relapse

2011-11-09T22:46:00.002-06:00

We received Keegan's lab results from yesterday earlier today. Unfortunately, Keegan is having a flare of the HLH/MAS. His ferritin level (an inflammation marker) jumped from 455 last Tuesday to 15,000 yesterday. Normal levels are usually under 75. 10,000 is usually the cut-off to start chemotherapy. Keegan's went from 15,000 to almost 50,000 in a matter of just a few days in September. We are unsure if this flare would have happened without the tummy bug from last week. The team allowed us to give Keegan an extra dose of anakinra today, and we will go in tomorrow morning for repeat labs. If there is no change or numbers have worsened, Keegan will be admitted, possibly to the ICU, for another three-day steroid pulse.

Keegan is exhausted, quiet, and having bad diapers again. Given that his numbers were so high yesterday when he showed no symptoms, I would bet the house that labs tomorrow are not going to be improved. He has not spiked an "inflammation fever", but that doesn't surprise me. It's complicated, but we know he had all of the criteria for this condition except for a fever while on his old immunosuppressant, Prograf. We stopped that drug when he was in kidney failure last year. We believe it was the switch to Rapamune that allowed the full picture, fevers included, to flourish. Now that Keegan's back on Prograf, I'm not surprised that he doesn't have a fever.

We are also unsure what the plan for treatment will be from here on out. I don't have the time or energy to go into much detail tonight. There are a few other options besides chemo at this point. None of them are desirable. This news is utterly defeating. And yet, we are gearing up for the fight. We will not let our Bug down. For tonight, we would just really appreciate your prayers that Keegan stays stable and that Gray, the medical team, and I can make the best possible decisions for his treatment and long-term health as possible. Thank you. More than you know. Thank you.

Back home

2011-11-07T22:23:00.000-06:00

Keegan was discharged home yesterday afternoon. His fever did not return, and he woke in a decent mood. His labs stayed about the same, although his platelets continued to fall. It isn't reasonable to sit around inpatient waiting on labs to get better. As a friend once said, Keegan would drive himself home from the hospital if we waited on labs to be perfect.

Today we learned his ferritin level drawn Friday had jumped significantly. Basically to the level that it was when the doctors in Boston started to get nervous. We will follow-up with rheumatology tomorrow to see what happens from here. We did also learn today that the genetic testing run so far is all negative for an inherited version of HLH. That doesn't mean he doesn't have it. It just means either (1) the defect is on a gene we haven't identified yet or (2) he acquired it somehow from a trigger, such as a virus or his medications/illnesses in infancy. Don't quote me on that last part, as we have yet to discuss this fully with hematology. Now that the labwork is close to being finished, we will likely start seeking out second opinions from HLH specialists, particularly in Cincinnati and Houston.

I simply cannot express our gratitude for the prayers and support offered this weekend for Keegan. This was a very stressful time, and we are so grateful that Keegan handled it so well. More soon. Thanks again!

Day 2

2011-11-05T22:44:00.001-05:00

Keegan is still inpatient. He spiked another fever this morning, but once again, it broke on it's own. He ate a little more today and seemed to be feeling pretty good until dinner time. He refused to eat all but a few bites and got a little sick right before bed. Then, he lost everything he had and then some around 9pm. As of right now, he has stayed asleep but is moaning and taking big breaths like he is still feeling nauseous.

His labs were a bit of up and down. His kidney function was better, CRP down a hair, but his blood counts each took a pretty big dip (white, red, and especially platelets). The one level that should tell us if this is truly the beginning of another HLH/MAS flare won't be back until Monday!! The lab gave some song and dance about not having the personnel or equipment to run it except during the week. This was a level that we would have in a few hours in Boston. It is THE key part of his diagnosis and tells us if we have to repeat the steroid pulse and/or turn to chemo to treat. The fact that it "can't be run on the weekend" here is horrifying. We saw it go from slightly elevated to taking a big jump to leaping to staggering levels in a matter of 4 days. There's not much I can do about it this weekend. If he is trending the wrong direction, I don't feel like it's going as quickly as it did before. This is a problem we will definitely have to discuss with the team and plan for in the future. Because it is highly unlikely that Keegan is going to only get sick Monday thru Thursday for the rest of his life. I just don't understand how a pediatric hospital this big can be incapable of running a very important, life-threatening lab on the weekend.

As of this morning, it sounded like if he did well today and overnight and if labs were ok tomorrow, we could go home. I didn't ask what would happen if the labs we can draw stayed the same, but he started throwing up again or spiked another fever. We will have to discuss that in the morning. In the meantime, the bug finally caught up with Gray. Since Audrey was the instigator and already was exposed, she is still with him at home. I think he's getting a little taste of the "moms aren't allowed to be sick" idea being alone with an active toddler while feeling crummy. He should be doing much better by tomorrow, and thankfully, our families will be coming back into town tomorrow, which will definitely be a little relief.

I have a feeling tonight is not going to be as smooth as I was hoping. Thank you for your continued prayers for our family. You truly do make a difference and help us keep up the fight.

29 days

2011-11-04T23:02:00.001-05:00

Guess we didn't have to wait that long to find out if Audrey really had a stomach virus or not. Keegan started throwing up last night. No surprise there. The 101+ temperature this morning was different, though. The team admitted him to the hospital this afternoon for observation, 29 days after he was discharged from the hospital in Boston. This somewhat minor tummy bug is scary for two reasons (1) with so much immunosuppression, he can't fight it as well as Audrey did, and (2) viral exposure can cause an HLH flare. Being that he just recovered from a flare one month ago, he is at a higher risk of another one occurring. If it does, we will be in the tenuous position of having to suppress his immune system further to treat the HLH while he is trying to fight an infection where a healthy immune system is required.

Other than the vomiting, fever, and lethargy, Keegan hasn't acted too poorly today. The fever broke on its own, and he hasn't thrown up since this morning. Hasn't eaten anything all day either. His labs weren't awful. Kidney's not particularly happy, and his CRP (an inflammation marker) is elevated. Still waiting on a ferritin level to come back before we make any assumptions. If he is still doing ok tomorrow and labs are stable, we will talk about going home. Hopefully, we are just being extra cautious and will be able to rebound as quickly as Audrey did.

Speaking of little bit, she is doing much better today. Ate better, played better, slept better. She and Gray are enjoying some quality father-daughter time at home tonight, as both sets of grandparents are out-of-town for the weekend. Great timing, Keegan!

Once again, we can't possibly thank you enough for the prayers and encouragement for our Bug. They really do help us get through every hiccup along the way.

Just because I needed a happy ending to this post.

Long night for Miss A

2011-11-03T22:00:00.001-05:00

Last night, Audrey was fighting some pretty bad tummy problems. She was throwing up for several hours before she finally settled. The only place she seemed comfortable was on the floor of her room. So there the two of us stayed all night long. Poor thing. I've never seen her so miserable. Needless to say, I am too old to be sleeping on the floor. Ouch. Audrey didn't eat hardly anything at all today and was a bit fussy but otherwise seemed ok. She was rightly exhausted. I looked over around 11am, and she had fallen asleep watching Sesame Street on the couch. Wish I could have done the same. I felt achy and a little nauseous today. Difficult to tell whether it was from lack of sleep on a hard surface or something else.

Thankfully, Keegan seemed to be about status quo today. I doubt we are out of the woods just yet, but we are really hoping Audrey's long night was due to drinking some of the bath water last night that had one of those fizzy bath colors in it. A few more days will show how this will pan out, as most stomach bugs have a 3-4 day incubation period after exposure. We will anxiously be waiting to see what happens, and we greatly appreciate your prayers for Audrey's recovery and Keegan's safety. A stomach virus could be devastating to him at this time. Thank you so much. We will update again soon.

Happy Halloween

2011-10-31T22:52:00.002-05:00

In preparation for Halloween, we hit up the pumpkin patch at Storybook Ranch last week.

Back in the saddle again

Audrey's first pony ride

Hay maze

Audrey and Ms. Kelley, Gage and Ainsley's mom.

Audrey became very good friends with Kelley and Abby while we were gone. I love that!

Bounce house fun

This ladybug L-O-V-E-S bounce houses!

For Halloween, Keegan was a heart surgeon (Dr. G, specifically), and Audrey was a pig. Their costumes were a tad thrown together, since I didn't have much time to prepare.

Dr. Harrison with his nurse.

He has Harvard Medical School scrubs, Gray's hospital badge, and a real surgical cap to boot!

My costume was the best ever - scrub pants & a tshirt! Sweet.

So Gray was the farmer to Audrey's pig.

This is the only picture with her hood on. Despised it. Sigh.

Aunt Alex joined the fun.

Abby hosted the pre-trick-or-treating neighborhood party this year. I started it last year to bring the kids together for good fun and food. I think it will become a tradition to hand it off to a different person each year. It has definitely outgrown our little house. Thanks for hosting, Abby! It was GREAT!

Oh, the bounce house!

Being a surgeon for Halloween conveniently camouflages your need to wear a mask.

Alex was a "flamingo dancer"...get it? Flamenco/flamingo! Clever sister, that one.

Audrey with her princess mentors - Lainey (as a character from Barbie Princess Charm School and Ainsley as Snow White).

Goofball.

The ghost cake and the cute mummy pretzels Alex made.

BD and Audrey

Trick or treat!

Keegan and Daddy, Ainsley, Gage, and Landry

Dani, Lauren, Paulina, Ava, Keegan, Dylan, and Gage

Keeg only lasted a few houses, and he really didn't understand what was going on, despite the many repeated views of "Mickey's Halloween Treat". We were just happy to have him home and participating a little bit!

And a little piggy ready for bath taking off with her farmer's hat!

We hope everyone had a good, safe, and fun Halloween! Keegan is doing ok. We are going forward with his steroid wean starting tomorrow on a weekly basis, so this is where things could get dicey. It will take almost 4 months to safely get him off the steroids. So frustrating because we are beyond ready to say good-bye to them. I want to see my precious boy's face again...as well as a sunnier disposition. I do promise to get a better medical post up soon. Thank you for your prayers over the weekend for Keegan. He isn't necessarily doing better, but he is not any worse. Hopefully the changes we have seen are steroid-related. Big transplant appointment tomorrow. Fingers crossed for a good report!

Nervous

2011-10-27T22:59:00.001-05:00

Going to bed nervous tonight...not because of the Rangers. Honestly, I could care less right about that now.

I'm nervous because Keegan had a rather questionable day. His tummy was not very happy today. Not bad per se, not even as bad as what used to be his "normal". Just not what we have been used to the last three weeks. He has been begging and begging for water every waking moment. In the past, this has been a bad sign for his kidneys, but so far, he is still urinating ok. Had a few bouts of gagging and choking throughout the day at mealtimes. Again, not terribly unusual but more than the norm. The really freaky part was when he started shivering and shaking for a good 30 minutes at dinner. I've never seen him shiver...ever. Not even when he's been playing outside in brisk weather or fresh out of the pool.

But his temperature is normal. His heart rate blood pressure were the same as always. His attitude was about the same. So, Keegan's asleep for now and hopefully just had a slightly off day. I may not sleep so well. Praying my nerves end up being a waste of energy. Would you join me? Thanks.

Wrapping up Boston

2011-10-24T22:44:00.000-05:00

It's hard to believe that it's been almost three weeks since Keegan was discharged from Children's Hospital Boston. I realize that I never wrapped up our pictures and final tidbits from discharge day and our final appointment day. Here's a little love for our home away from Dallas.

Keegan's window on the cardiac floor is the second from the top on the far right.

Waiting to leave on discharge day

He was SO not happy when he realized we were actually leaving the hospital.

I think Ashleigh was quite possibly Keegan's favorite nurse.

She was there for us on some really rough nights...and days too!

Shanda and Ricky got to say good-bye.

We missed quite a few other friends, especially Liz who also rode out some scary times with us.

You learn alot about each other when you're taking hour long turns holding pressure on a gushing wound, ya know.

The cardiac ICU. A wonderful group of people beyond those doors.

So glad we were just across the hall when we needed them.

Kinda funny that the cardiac floors at CHB and CMCD are both on the 8th floor.

Keegan loved his room. More than I think he has been previously attached to any other hospital room in the past, although he does get very comfortable...too comfortable.

The amazing staff on the floor held his room while he was in the ICU so he wouldn't have to adjust when he came back.

On the last day of appointments outpatient before we flew home, he was getting tired late morning and asked "number 8? Room please?" When we got to the floor to say our goodbyes and wrap up some paperwork needed for travel, we had to keep him from running back into his old room, which was of course being occupied by another kiddo by that time!

In the cab leaving the hospital on discharge day.

"No leave hospital, Mama!" Keegan could not understand why we were so happy to be going!

Our awesome cardiologist, Dr. Blume.

She loves Keegan so much. Leaving her and the simply amazing transplant team at CHB was absolutely the hardest part of coming home.

Wish we had a picture with the whole team!

The ones that finally put Keegan's puzzle together!

Dr. Henderson and Dr. Sundel, the rheumatology team (and Dr. Kim too!).

Words fail me when I try to explain how blessed we were to have them on our side, along with the hematology team. How do you thank someone for saving your child's life?

And Dr. Sundel also has the rare honor of getting Keegan to eat a lollipop for the first time.

Took him a month, but I think he had two at that last appointment!

Keegan's favorite activity in the hospital - throwing coins in the waterless "pond" in the lobby.

Daddy and Keegan in front of the Bippity Ball Machine in the lobby, another favorite.

As loud as that thing is, there is something about the cadence of those balls bouncing, whoosing, and pinging that is reassuring and calming, even on the worst days.

Mama and Keegan

After lunch in the garden

Thank you to each and every CHB doctor, nurse, and staff member that cared for Keegan.

The words are wholly inadequate to describe how desperately grateful we are for your compassion, expertise, and dedication. We are forever indebted...yet again.

Hugs

2011-10-22T23:11:00.000-05:00

Aaaaand, we're back

2011-10-20T22:06:00.000-05:00

Online, that is.

After a very long week without internet access (my WORD, are we not overly dependent on technology these days?), we are finally back online. Come to find out, thanks to our trusty DVR, that our internet has actually been out since about 2 weeks after we left for Boston. I'm SO not paying for that month, thank-you-very-much.

I still have a lot of photos to organize and post and am slightly overwhelmed by the amount of "updating" is necessary for this little blog. I am still unpacking/organizing/cleaning, not to mention trying to locate/wash/iron fall clothes and shoes. If you know me at all, any one of the above three problems, not to mention the combo of them, is enough to give me an anxiety attack.

Some highlights from the last week:

Watching my kids "remember" each other again.
Sitting down to eat dinner together as a family. For the first time...EVER.
Mexican food and margaritas. (Yes, it tops my list. Yumm.)
Deaccessing Keegan's port on Saturday to let him swim for the first time this year last weekend.
Picking Audrey up from school for the first time. She has a fascination with the play vacuum cleaner. Definitely my child.
Coming home Monday afternoon from going to pick up 2 boxes (yes, big brown boxes) full of Keegan's medications to find Audrey walking, just like she had been doing it forever. Tuesday, her teachers said she was walking on uneven surfaces on the playground. By yesterday (Wednesday), she was standing up on her own unassisted from sitting in the middle of the room, without pulling up on anything. Today, she was almost running. The girl is a perfectionist, I suppose. Waiting until she had it all down to take off on her own. Hmmm...wonder where she gets that?
Going to sleep at night knowing that Keegan is not connected to any tubes or wires!!! His last feed ends before Gray and I go to sleep. So very, very wonderful.
Attending a very productive, reassuring team meeting with all of Keegan's specialists on Tuesday. It feels very good to know that we have an umbrella for the next storm. We may not know how sturdy or reliable our "umbrella" is yet, but we are not stepping out without some sort of plan. That, in and of itself, is very reassuring. He is now actively followed by 8 specialists, plus his pediatrician and therapists.
Relatively stable labs for Keegan. His numbers are definitely taking longer than expected to normalize. Either that or they are not going to really normalize as we continue to wean steroids. That remains to be seen. I will certainly do a better update on his medical status soon. I think one of the transplant cardiologists said it best last week...Keegan still has enough steroids on board (which is a LOT) to keep him doing really well, considering what he just went through. We're on the "high" side of the wave essentially. As we continue to wean the steroids, we are unsure how he will do. Still, we are going to ride this wave and enjoy it as long as we possibly can!

Pictures and more regular updates to follow soon! Thank you again for your prayers!

Be right back!

2011-10-16T23:11:00.001-05:00

Pardon the interruption: your regularly (or not so regularly) scheduled blog posts have been interrupted by a lack of internet access in the Harrison household. Please talk amongst yourselves while we work diligently (and Verizon, evidently, does not) to remedy the situation. Keegan is stable & happy. Audrey too! We will resume updates as soon as we possibly can. Thank you for your patience!

- Posted using BlogPress from my iPhone

Home again

2011-10-12T21:19:00.001-05:00

...home again, jiggety jig!

We made it! All together under one roof tonight. Thank you, Lord, for your many blessings!

More to come!

- Posted using BlogPress from my iPhone

Going home!

2011-10-12T10:17:00.001-05:00

We are on the plane & ready to go HOME! More details to follow soon. Thank you to every doctor, nurse, & staff member at Children's Hospital Boston who helped make this day possible. We wish we could take you all with us! Thank you to everyone who has prayed for us on this journey. We could not have made it here without you.

Audrey, here we come!! We love you!!

- Posted using BlogPress from my iPhone

Fun in Boston

2011-10-10T21:43:00.000-05:00

This afternoon, we headed to a place that absolutely makes me, the germaphobic mother of an immunosuppressed child, shake with fear...Boston Children's Museum. In the end, I hope that our reliance on masks, hand sanitizer, and fervent prayers for Keegan's safety were sufficient to protect him because oh boy, did he have fun! Thanks to Keegan's medication schedule and Gray's attempt to work from the hotel room, we were able to capitalize on the half-price-admission-after-4pm deal and limit Keegan's exposure to one hour. But trust me, it was an action-packed hour!

Bubbles!

KID POWER!

Shooting hoops with Daddy

Water table. His shoes were SOAKED!

Arthur's kitchen

Workin' the Bobcat in the construction zone

Cars and trucks

Master architect

Peas in a pod

The fun food stand outside the museum. Hood is a dairy, much like Borden or Schepp's back home.

After the museum, we stopped by a playground near the hospital. This playground is where Keegan suffered his first skinned forehead back in 2009.

We ended the evening with dinner at Matt Murphy's Pub. A lovely neighborhood pub where Gray happened to sneak in one night the first week we were in Boston looking for a football game. He didn't find one, but he did find some awesome, caring people in the owner and bartender (leave it to Gray, right? Never found a stranger!). They listened to Keegan's story and were genuinely caring and supportive. When we returned with Keegan in tow tonight, they treated us like family. A big thanks to Siobhan and Doug!

Tomorrow will be whirlwind of labs and appointments. Praying Keegan gets the all clear to return home...especially since we just bought airline tickets. There are still a lot of "game-changer" labs pending, but at this point, it's time to go home while we wait on those pieces of information to fall into place. Thank you for your support through all of this. We've really enjoyed the last 5 days outpatient in Boston. Thank God for Audrey and family...Boston would be near impossible to leave without them!

Boston weekend

2011-10-09T21:51:00.001-05:00

I apologize for being a bad blogger as of late. The hotel wi-fi is horrendously slow, so uploading the important things, i.e. pictures, takes forever and a day. We have no blood pressure or heart rate monitors or daily labs to know how Keegan is doing outpatient. He is acting much the same as he has for the last week and a half, just out of the hospital instead of inside it. Although this morning, he had about two hours of a generally good mood. Gray and I commented several times how amenable Keegan was being. Alas, it was short lived. Stinkin' steroids.

He continues to eat about the same as he was while inpatient, mainly baby food purees and dry carbs with three small boluses of a new formula each day. I can only imagine what waitresses must think when we say, "no, we don't need to order him anything", and then proceed to pull out the baby food and goldfish arsenal. The nutritionist would like to see Keegan take more tube feedings during the day, but cardiology has placed him on fluid restrictions in our effort to help his blood pressure and fluid load. Despite that, he has maintained his weight well. We are on a flexible diuretic plan, which means we weigh him every day (yes, the team gifted us with a brand new digital scale for our hotel room) and adjust his diuretics whether he is up or down. We are not worried about his intake right now. Whatever he eats or wants is fine, but we are anxious to restart feeding therapy while we have steroids on board. It should be interesting to see if his weight remains stable after the steroids are gone.

We have one more day outpatient to run through some more sights, arrange travel plans, and pack/ship items back home. Tuesday will be a full day of outpatient appointments with the team here in Boston, and if all looks well, we should be able to fly home Wednesday!! Just 6 weeks after we arrived. Fingers crossed! Our two goals before returning home were to get Keegan eating by mouth (and tolerating it...details, details) and to get Keegan's new team of doctors set in Dallas. First one seems to be ok, and the second is in the works. It will be even harder to leave Boston this time than in 2009.

And for the finale, our weekend in pictures:

Saturday we went apple picking in Ipswich, Massachusetts at Russell Orchards

I've never done anything like this. I can't say I was surprised, but these apples were fresh, crisp, and shiny. Thanks to being a pesticide-free farm, we could put what we could fit into a bag and eat what we wanted right off the tree! We plan on taking our bag back to the hospital's community housing, the Yawkey Family Inn, where Gray and I had a room so one of us could get rest at night.

Raspberry fields

Cider donut time!

The orchard was about a mile from Crane Beach. Keegan had never seen the ocean, and it was 80 degrees. Perfect day for your first trip to the beach!

On the way back in, we drove through Salem and ate dinner. It was the annual haunted happenings fair, so there were plentiful people dressed as zombies/witches/etc around. So odd.

Sunday, we ventured into Cambridge and toured Harvard.

On the steps of the library at Harvard.

He looks smarter already!

Then we cruised by the Boston Navy Yard to catch a glimpse of the USS Constitution.

Sunset on the harbor.

We capped off the day with dinner in the Italian North End.

Overall, a weekend in Boston that was hard to beat!

Friday photos

2011-10-07T22:18:00.000-05:00

Today was our first "free" day in Boston - outpatient and no appointments at the hospital, much to Keegan's dismay. He was a very unhappy camper yesterday until we pulled up to the hospital. His frown turned upside down, and he practically skipped inside. You could tell the familiarity instantly took a weight off his shoulders. The port reaccess that followed quickly thereafter changed all that for a bit though! I wonder what people think we do to him at home that makes the child scream and holler "no go home" when we leave the hospital.

We didn't want to push Keegan too much today. We got our Legal Sea Foods fix for lunch, hit up a farmer's market in Copley Square, and wrapped up with a visit to see the Ducklings in the Public Garden. A walk down Newbury Street capped it all off. Not too shabby. I do wish Keegan felt better on these outings. He is an emotional wreck from the steroids (still on an extremely high dose, the same as the 4-week mini-pulse in June). His belly is horribly distended, and he complains about it alot. He also has very little energy and still struggles with stiff joints. But we are making the best of it. I think tomorrow we are going to rent a car and drive to an apple orchard outside of town for the day.

A few pictures to wrap up this post. I have tried to write updates during the day, but things have just been too nonstop lately. By the time I sit down at night, my brain has reached it's bottom-of-the-barrel productivity rates. One day, I will get it all together again! I still need to post pics and details from discharge day too.

38 days after I sat at Legal waiting for Keegan and Gray to arrive in Boston

Keegan and Mrs. Mallard, back together again!

A beautiful day in the Public Garden for a nap

Day 1

2011-10-06T21:13:00.001-05:00

First day of outpatient life in Boston went well. Labs, EKG, & clinic appointments were ok. I will update more tomorrow. Tonight, I am thankful for Rylynn's new heart and that my only alarm clock (besides Keegan waking up too early) is 9am meds!! Details tomorrow. Promise!

- Posted using BlogPress from my iPhone

Discharged!!

2011-10-05T20:39:00.000-05:00

Keegan was discharged from Children's Hospital Boston today after exactly 6 weeks inpatient. We are staying in a hotel locally for at least a week, possibly flying home as early as next Wednesday! More details tomorrow. We need to get some sleep for an early day of outpatient appointments tomorrow. We are so beyond grateful for every prayer and well wish for Keegan that made this day possible. Thank you!

What to do?

2011-10-03T22:48:00.003-05:00

Not a lot new to share today. Keegan had a pretty quiet end/beginning of his week. His labs on Sunday continued on a track that makes us very antsy. Are we seeing the beginnings of another flare or a reaction to the IV iron from last week? It's a bit too early to tell. We had to supplement his potassium and minimize his diuretics a bit today, even though his blood pressure remains high. His liver is still enlarged, tummy still a tad uncomfortable, and his joints are a bit stiff today. All in all, he is headed in the right direction to be discharged from the hospital.

The question is where do we go from here? Outpatient in Boston while we sort things out or wait inpatient here longer while we try to get care coordination hammered out at home. At the end of last week, we were pushing for straight to home. We quickly realized today that a fly-straight-home plan might not be the easiest or safest option. Not to mention that getting his care coordination situated at home is turning into a nightmare. Politics...ugh. We had a nice discussion with the transplant cardiology attending tonight and will regroup with another team meeting tomorrow afternoon. By then, we should have a better timeline for the coming week. Thank you once again for all your love and support.

A happy boy on Sunday morning

Play-doh!

(An inside joke for my Dallas DA friends...I almost entitled this post, "what am we to do?" I then realized most people would think I had lost my mind. Although I did have a good role model for that, I suppose.)

Busy, busy

2011-10-01T23:14:00.001-05:00

I apologize for the delay in posts. The last few days have been quite busy here in Boston, both with Keegan's care and discharge planning. Yup, you heard me right. Lest we get ahead of ourselves though, I should start with how Keegan has been doing. I will resort to my friends, the bullet points, for clarity and because, frankly, I feel unable to put together any well-drafted paragraphs at this point.

First, a general recap of the main points:

Keegan has been doing well overall. His labs are starting to stabilize. Inflammation markers are almost normal, which was earlier than the team anticipated, but his liver function is not, which is taking longer than we anticipated. His blood counts are all holding steady due to the boost from steroids. Today was declared a "lab holiday" for Keegan, but he will have a very big draw tomorrow. After daily labs for so long, even one day has me on edge. Keegan's liver is "down" even more today, which means it is enlarged to the point that it can be felt by pressing on his tummy. We are hoping it is due to fluid retention and nothing more sinister at this point, which brings me to our next point.
We are trying to aggressively treat Keegan's fluid retention/weight gain over the next few days for several reasons. His belly is very distended; he has been very "positive" (more intake than urine output) the last few days, and his blood pressure is way too high. He is now on three different diuretics, in addition to increasing his blood pressure medication. The goal is to get him to lose about .4kgs per day (0.8lbs). I don't think we will get there tomorrow. This is all assuming that what we are seeing truly is fluid retention. We are also walking a fine line with these diuretics and his kidney. Drying it out too much could be dangerous, but we will return to daily labs tomorrow, along with watching his weight, appearance, fluid balance, and blood pressure.
Keegan's GI system is giving us all the shock of our lives. He has continued to have soft stools daily since Wednesday. Simply amazing. He has been successfully eating small meals of baby food and dry carbs each day and has tolerated three bolus feeds of 2 oz per day of a new tube-feeding formula. This has allowed us to stop his TPN for the last two days!! So far, so good. Even better? As part of our fluid-retention-plan-of-attack, we are holding ALL fluids overnight, so Keegan is only attached to his heart rate/saturation monitors tonight!
While we have been ecstatic to see some huge progress on the GI side, it seems to unfortunately be coming at a pretty good price. It hurts to see his belly this big. You can see every vein in his abdomen clearly against the skin, and at times, it is so tight it seems you could bounce a coin off of it. Sometimes he can't even sit up straight because it makes him uncomfortable. Keegan doesn't speak much and rarely can communicate with us what exactly hurts when he is in pain. Not now. He is moaning and groaning, grabbing his belly and saying "it hurts, it hurts" many times a day. Although the "quality" of his stool is improving, there seems to be way too much of it and too difficult for him to pass (I know that's a bit graphic, sorry). We have been putting him on the potty several times a day to help encourage him to pass it, but he has such a difficult time and is so stressed about it, that I worry we will end up making him fear the potty completely. Again because of Keegan's communication limitations, we don't know if this is all just due to to his GI system learning to work properly or a true problem. Our GI doctor heard our concerns on Friday and shares them with us. He wanted to stay the course unless Keegan became inconsolable or constipated (ha!) over the weekend to get a bit more data, as it were, before going forward with any more testing.
Keegan is now down to the same amount of steroids he was on during the "mini-pulse" in June, which is still a very large dose. We haven't seen a fever out of him yet, but his heart rate has gone back up into what used to be "fever warning range" a few times. We will just have to wait it out a bit longer.
Not sure why yet, but Keegan seems to be utterly exhausted. He wants to sleep a lot, doesn't want to walk, wants to be carried, etc. We've been limiting him to one afternoon nap, and he is still going to bed about on time.
The steroids have made him miserable. There are a lot of crying, plentiful screaming, and abundant meltdowns coming from room 819. He, himself, is miserable, and he is plain miserable to be around. I hate steroids.

Second, some of the bigger events of the last few days:

Thursday, Keegan had to have his first IV iron infusion. The level of iron in his blood stream has been virtually zero since about April. Now that he is more stable, hematology said it was time to proceed. IV iron carries a very, very big reaction risk. So much so that a doctor has to physically be present in Keegan's room for the first 30 minutes, and a nurse has to take his vital signs every 30 minutes for the entire 4-5 hour infusion. Keegan tolerated it very well overall. We will see how well it worked with labs tomorrow. More than likely, this will become a fairly regular treatment for him.
Friday, Keegan's evening just before bed was quite eventful. He was overdue for his monthly inhaled antibiotic used to prevent a certain type of opportunistic pneumonia. At home, this is given in the Center for Cancer and Blood Disorders in a filtered, negative pressure chamber to keep it out of the general air circulation. It's a bit different here, so we attempted to outfit his room with a HEPA filter and administer it on the floor. Keegan wasn't too happy about that idea and let everyone within ear shot on the surrounding floors know about it. Around the same time, he received a new IV blood pressure medication. Between the two (probably the BP med), Keegan had a mild allergic reaction. He broke out in some hives, started retching and throwing up, and was complaining about his tongue. IV benadryl seemed to nip it in the bud before it got bad. Needless to say, we learned quickly about a new drug allergy!

Lastly, the magic word ... "discharge":

Tuesday, the plan was to leave the hospital but stay in Boston for a few weeks. By Thursday, we were reevaluating that idea for the just flying home next week. GI was a main reason to stay here outpatient, but so far that is going well. The other reason to stay was to make sure a team of doctors are in place at home to take over Keegan's care.
If Keegan doesn't throw us any more curveballs, we may be able to stay inpatient through next week and fly home next weekend some time!!
I don't have energy to go into the care coordination issues at home right now. Another post for another time.

A tuckered out Keegan.

The iron infusion on the right.

TPN under the amber bag on the left.

In hindsight, writing smaller updates more frequently would have been a better idea! Oh well. Hope I didn't leave anyone or anything out. Thank you once again for each and every prayer and well wish. I simply cannot put into words how much each prayer means for my precious Bug. Keep them coming!

4 weeks

2011-09-28T22:06:00.000-05:00

Today marked four weeks of Keegan being inpatient in Boston. What a roller coaster these four weeks have been! I can't believe that after years of looking for something, anything, to tie all of the pieces of Keegan's picture together, we are finally on the right road. We still aren't sure exactly whether to call this MAS or HLH, but that will work itself out in time. Management of this condition will be life-long and a entail lot of ups and downs. But at least we have a better idea of what it is we're fighting, and sometimes that is more than enough.

It's been a very busy last few days, and it is only going to get busier. Because Keegan might be discharged from the hospital by the beginning of next week! We will be staying in Boston for a little while after that, but we would at least not have to be in the hospital. I will certainly share more information as I have it, but we are very excited that this could bring us one step closer to home.

A few notes about how Keegan has been doing this week:

His inflammation markers have stabilized, but they are still slightly elevated.
His liver numbers and coagulation factors are taking longer to normalize, but there has been drastic improvement.
Neurologically, we think Keegan is almost back to his old self. We are going to hold off on a repeat brain MRI until we got home. The immediate threat is gone. We still will likely need imaging to determine what long-term damage was done, but again, that can wait.
From a GI standpoint, we are learning as we go along. At the risk of sharing too much information, Keegan has actually had some better "quality" to his stools, as it were, the last two days. That's encouraging and could give credit to the theory that his underlying MAS/HLH was causing additional GI problems. We will continue to watch him the rest of the week closely, and we may have to proceed with the motility study anyway. We have rarely seen him this distended, and he is complaining of his stomach hurting for the first time in his life.
His walking is getting better. Keegan still doesn't have much energy...hardly any at all, really. But each day is a bit better. Over the weekend and through yesterday, he was using a little kid-size walker to get around, but today we took slow laps around the floor three times successfully.
I'm sure there's more, but I'm having trouble remembering everything right now. I really need to attempt day-time blog updates!

Decked out and ready to roll!

Getting his haircut by a lovely volunteer who cuts kids' hair while they are inpatient.

Keegan wasn't too happy about it, despite this calm picture, and the bath that ensued afterward was even worse!

HATE the steroids!

Thank you again for all the love and prayers sent Keegan's way. You keep us all going strong!

Ladybug Goes to School

2011-09-26T07:48:00.000-05:00

I'm just a touch behind in posting about it, but Audrey has been going to "school" or Mother's Day Out while we have been gone. The doctors at home thought sending Audrey to school was too much of an infection-risk for Keegan while we were all living together. Since Keegan is not around her at the moment though, Mamie has been taking her twice a week, and it sounds like she's having a blast. Audrey is attending the program at a local church where Keegan went last year for a few months, where many of our friends attend. We love the staff there, and they are taking such great care of her for us! She's doing art projects, singing songs, playing with other kids (a first!), learning new words, and getting lots of socialization.

First day of Mother's Day Out

Super excited to find Angel Baby in her "school bag"!

Luckily, the last day we were home was the first day of school.

I had to be at the hospital with Keegan most of the day.

We chose to not take her ourselves, so she would have some consistency with Mamie bringing her the following weeks.

Taking a ride in the buggy around school.

Her teacher and the administrative staff have been taking pictures to email to us, so Gray and I don't feel like we're missing as much.

"Washing" cows with paint.

The finished cow. Audrey's first true art project.

Enjoying an after-school snack after a long day!

Overall, Audrey is doing very well. She is developmentally on target and still eats like an absolute horse. Wonder where she gets that? Ironic because Keegan, at 4 years old, is just now eating pureed food and dry, simple crackers, but Audrey, at 15 months old, can eat more than me some days. She is still a tiny little thing though, weighing in at just 21 pounds. Audrey still lacks the confidence to walk independently but will go forever holding one finger. I can't believe that the day I get to see her again, she will likely walk right into my arms.

Audrey has been a little fish in Mamie & BD's pool and swims or plays outside every chance she gets. When Alex and I were little, we went straight from bed to the pool most mornings, eating our cereal from the side of the pool. Audrey does the same whenever she can, and that makes me so happy. She absolutely loves to dance - no matter what else she happens to be doing! She seems to have a particular love of Maroon 5. We were video chatting with her, and my dad had the radio on in the background. In the middle of talking and playing, she heard the beat to "Moves Like Jagger" and busted out with her signature shoulder shake, lovingly called "the Audrey". Too cute!

Playing with her BBQ by the pool after a morning swim.

I wish I could give more details about my adorable Ladybug, but I feel terribly out of the loop when it comes to her. She also went to the zoo this weekend for the first time with Aunt Alex, Mamie, and Big Daddy. Keegan has never even been to the zoo. I will try for a post with some of the pictures from that soon. We video chat with her constantly, but it is no substitute for loving and squeezing on her. I can't believe how much Audrey has grown just in the last month, and I'm so excited for her. I left her still very much a baby. There is no doubt that she is now a precious and precocious little toddler.

Keep getting bigger, stronger, and smarter, Miss A. We miss you so much more than words can express. We can't wait to wrap our arms around you again. Love you, love you.

Baby steps

2011-09-24T22:11:00.000-05:00

Keegan is making baby steps of improvement each day. His labs have been up and down, but that is not unexpected at this point. We are doing our best to make small changes each day, so that he handles it better and is able to achieve these baby steps without too many set-backs.

Tidbits from the last two days:

His inflammation markers are showing the most improvement. Some of them are actually measurable now!
His platelet levels have taken some drastic swings, but his hemoglobin/hematocrit are only dropping by small bits each day. This makes us more confident that the "eating" of his blood cells by the macrophages has slowed down. His liver numbers are starting to normalize a bit too.
We don't think he has any more "active" bleeding. The dressing on his bone marrow biopsy site was changed yesterday. It looks like a scab has formed at the skin level, but some bleeding occurred underneath that from the bone. Again, it's not wholly surprising and will take some time to heal after such a trauma.
Due to the coagulopathy (inability to clot), he is bruised everywhere. Not just on his hips, backs, and legs. Anywhere he was poked, prodded, or moved in the last week has developed a horrible, nasty bruise. We were joking with the nurse earlier when we were leaving the room that we needed a big sign or some certified letter from the doctor that we weren't beating him.
The bleeding, bruising, and inflammation are all making it very painful for Keegan to walk well. Today, he made it about 10 yards, two different times. He also walked down a few stairs, which was probably a bit too much today. A little each day will help though.
So far, he has had almost two days worth of doses of the tacrolimus without a big reaction by his kidneys. Another problem with the tacro is that it is given by mouth. We know Keegan doesn't absorb well through his gut, and that was evident this morning when the level of tacrolimus was virtually undetectable in his blood despite a good-size dose. Increase and try again tomorrow.
His echo yesterday was stable from earlier in the week. He has been experiencing some bradycardia (slow heart rate) at night that we have never seen before. Keegan has always actually been tachycardic (high heart rate) for his age. Right now, the doctors aren't too concerned but are watching closely. His respiratory rate isn't changing as much as it was a few days ago with these episodes, so it isn't quite as alarming as it was at first glance.
We are slowly switching medications back to g-tube versions from IV. He has eaten a few goldfish crackers the last two days and a bit of applesauce today. Keegan's tummy has not been too happy with this situation. He hasn't thrown up in about 24 hours, but the diarrhea is back in full force.
We are seeing small gains in his neurological status each day. His eyes looked better today. He interacted with the tv and with us more. He is still struggling to get words out. It seems most obvious when he is trying to string together a few. We will talk again with the team on Monday about timing to repeat his brain MRI. We held off on the CT scan because the neurologist thought it wouldn't show us everything we wanted and would expose him to more radiation. Now that he is more stable, he will be able to better handle anesthesia for a good MRI scan. To follow up on his MRI from the other week, it was relatively stable compared to the one in June. The radiologist did say, however, that the cluster of bleeds was not consistent with clots showered from the bad port. Those would be scattered along the periphery of his brain, and these are "deeper". This means they are likely spontaneous bleeds more consistent with the HLH and another reason a repeat scan would be helpful.

All in all, everyone is very pleased with how he is recovering, especially us. We did finally have a nice, long sit-down with the oncologist specializing in HLH. She said it is hard to say that holding off on the chemo was a bad decision considering how well he is doing right now. However based on his history and the severity of this last week, she did not think, nor does anyone really, that this will be his last "flare". That's where bigger decisions will come into play. We are waiting on some genetic testing to come back, but if it is negative, as we anticipate it will be, for a genetic predisposition towards HLH, we will just have to wait and watch. She said it would be a good idea to have a follow-up appointment with a specialist at home, just to make sure we don't miss anything. We will just have to see how that all lines up when we actually get close to going home.

Making a run for it!

... A coffee run that is, but it was good to get out of the hospital for a brief minute!

I think that's enough for tonight. Gray is staying at the hospital tonight so that I can go get some rest. Keegan slept a touch better last night, and so far, he is doing much better tonight. Here's praying he keeps it up! Thank you so much for all the prayers. I'm going to try to get a post or two about Audrey up tomorrow. It sure is fun to talk to her on the video chat and see pics taken by my parents and her teachers. But oh my goodness, how I miss this precious little girl!

A screen shot of our "bathtub cam" video chats after Keegan is asleep.

Please ignore my creepy face in the corner!

On the move

2011-09-22T22:26:00.001-05:00

He may not be "out of the woods" yet, but Keegan is out of the ICU!

Will update more tomorrow. For tonight, we are asking for prayers for sleep! Sleep for Keegan and our buddy, Rylynn. Keegan hasn't slept but about 3 hours total in the last 48, and Ry has been fighting sleep all week. These kiddos need their sleep to heal, so please pray for some zzzz's! Thank you!

- Posted using BlogPress from my iPhone

Steady

2011-09-21T22:35:00.000-05:00

I'm not quite ready to label a post "stable", so "steady" will work for now. His labs were up and down, again trending back to his old cytopenias. I'm not sure anyone believed that was something we could correct for good. We are just hoping he won't need long-term intervention to keep his counts at baseline. We did not have to give IV fibrinogen for the first time in four days, which was huge. Levels are still super low. His bleeding has slowed down a lot though, so we are trying to not expose him to any more blood products at the moment.

The team meeting this afternoon went very well. We were surrounded by many people who genuinely care about Keegan and our family, who were thinking critically and creatively about his treatment. Overall, the team is mostly pleased with the direction Keegan is going. They are still not ready to say he has completely turned the corner, but he is slowly moving towards it, at least.

Here are the main points from today's meeting:

It appears most everyone is characterizing this as more of an HLH than MAS, but again the characterization is arbitrary at this point. It's the treatment that is important.
We are going to do a much slower steroid wean and a higher dose of anakinra than usual for the next few weeks, in an attempt to ease Keegan's body toward recovery.
Tomorrow, he will also go back on a small dose of tacrolimus. The heart transplant anti-rejection drug that we had been avoiding because of his kidney. Since we now think that he didn't truly have HUS, we may not need to be as scared of the tacro. Keegan's kidney is still very, very fragile. We will be starting him off slowly and keeping it at a minimal dose. They will be trending his kidney function labs daily, and we will stop the second they go more in the wrong direction than he already is from the HLH.
The team is cautiously optimistic that we made the right choice in anakinra over chemotherapy (a drug called etoposide, specifically). He will be getting daily labs for awhile, and we are hoping that Keegan would give us some sort of a warning either in labs or clinical symptoms of relapse, allowing us to jump right in with the etoposide quickly. The HLH specialist is still reviewing Keegan's case, and she will hopefully be able to weigh in with greater certainty tomorrow. It is still on the table as a course of treatment. We were also told today that while it would be much farther in the future, Keegan's heart transplant does not preclude him from having a bone marrow transplant. The donor would have to not only match Keegan, but his heart donor also, and that could make finding a marrow donor virtually impossible.
An echocardiogram done yesterday showed a few changes from the one he had when we arrived in Boston. Not necessarily concerning changes, but any drastic change in a transplant is something to watch. So he will have a repeat echo every 3 days, especially as the tacro is added back in to his drug regimen.
Keegan is still retching and would be throwing up if we weren't venting his g-tube constantly to gravity. Not sure what that's about quite yet.
The biggest concern of the day is Keegan's neurological status. My brief mention of it yesterday was quite an understatement. Probably because it's a very difficult thing to admit. Keegan has been a little more than just "zoned out", and it's not from the pain meds he was on (been off the PCA pump for two days now). He is minimally responsive to some basic questions at best. His pupils are extremely dilated all the time, no matter the light in the room. He is having some breathing and heart rate changes that could be indicative of an increase in pressure in his brain. From a bleed, a stroke, seizures, or from inflammation, we're not sure. The team felt the risk of taking him out of the ICU and giving him anesthesia for a scan was too great today, but we are planning on making it happen tomorrow. We will probably go for a CT scan instead of an MRI because it doesn't take nearly as long. All that being said, he had a promising 20 minutes or so this morning where he sat up a bit and seemed to focus on the TV better than before. Once today, Gray was leaving the room, and Keegan softly said "bye" to him when prompted. He spent much more time awake today, and he waved and feebly tried to blow a kiss to Audrey, Mamie, and BD over FaceTime this afternoon. We will see what tomorrow brings, but this is by far our number one concern at the moment.
If his bleeding is minimal and his counts are stable tomorrow, we should be able to go back to the recovery floor and leave the ICU after the scan.

On a positive note, Keegan happened to be free of any infusions running for a few moments just before bedtime. Our lovely nurse grabbed a go-kart, loaded Keegan up, and led him on a "sticker hunt" throughout the ICU. There are boxes of stickers located at different nurses stations around the unit, so we walked around them slowly. At each one, Keegan pointed to a sticker he liked and even was able to grasp one himself. HUGE gold star for the day!! Definitely have a long way to go, but it was really good to see.

In the end, there was truly no decision to be made today. We are still waiting on some more information and frankly, we're waiting on Keegan. This could still go either way. We know that. I'm not sure we're prepared for it, but we know it's a possibility. For now, we have to trust that the Lord knows Keegan's struggles and journey. We will continue to ask for clarity and wisdom as we stumble through each day, and we will thank Him each day for surrounding us with the love of family and friends who help us get through it all. Thank you.

Decisions

2011-09-20T22:42:00.001-05:00

Keegan is still in the ICU. He is still bleeding from the bone marrow biopsy site, but it has slowed to an ooze from a gush. And just to correct myself from the other day, he is actually bleeding from the bone. It's not marrow, nor is it bleeding from the tissue. So, we can't just stitch him up, or the bone will continue to bleed internally. The only real chance we have of stopping it is to treat the underlying disease. And therein lies one of our problems.

We are still on the fence of making a diagnosis for what Keegan is facing. If you happened to do a web search when I first mentioned Macrophage Activation Syndrome (MAS), you would have found that it is virtually indistinguishable at presentation from Hemophagocytic Lymphohistiocytosis (HLH). In theory, MAS is a complication of juvenile rheumatic diseases (such as rheumatoid arthritis, vasculitis, etc), and HLH is a blood disorder that can be genetic, reactive (in response to an infection), or idiopathic (no known trigger). Both are, to put it in hugely simplified terms, conditions where the immune system goes completely nuts. The worst of both conditions results in what is called a "cytokine storm", where there is so much inflammation in your body, that it feels like the worst flu you have ever had....times about a million. Again, if you looked up cytokine storm when I first mentioned it, you probably would have seen that it was likely a cytokine storm that was the cause of death in otherwise healthy people after H1N1 exposure.

The criteria for diagnosis are the same; both cause raging inflammation in the body, decreased blood counts across the board (white, red, and platelets), coagulopathy, etc. But the treatment differs widely. MAS is treated with a variety of immunosuppressants, including high dose steroids and a drug called anakinra. Keegan has completed the three-day steroid pulse and been on anakinra for two days now. HLH, however, uses the same drugs, but also involves a round of chemotherapy and possibly bone marrow transplantation. People with underlying rheumatoid disorders can have recurrent MAS flares that are not fatal. Children with HLH rarely survive a relapse, prevention of which is the reason for transplantation.

While Keegan meets every one of the criteria for diagnosis of either or both, his medical history makes it more difficult for us to decide at first glance which one this is, and thus, what the next step should be. No one knows what either looks like under the umbrella of an immunosuppressive regimen after a heart transplant at 7 days of age. I mentioned a few days ago that the many doctors here working on Keegan's case where rethinking his many ups and downs over the last four years. It appears that what we thought were a strange combination of isolated incidents could have been recurring flares of MAS/HLH that were mediated by his heart transplant anti-rejection drugs. A few examples:

Keegan has been cytopenic his entire life. This means he has never had enough of any type of blood cell (hemoglobin, white blood cells/neutrophils, and platelets). At times this has caused him to be dependent on blood transfusions, GCSF and epoetin injections, and platelets replacements.
Two of his three liver biopsies showed evidence of hemophagocytosis.
We thought he had a rare form of hemolytic uremic syndrome (HUS) induced by one of his transplant medications, tacrolimus. By reviewing some of the labs from that timeframe, it is possible that this may have actually been a MAS/HLH flare that we inadvertently stopped by switching him to a drug called Simulect. High IL-2 levels in your blood are a criterion for HLH, and Simulect is an IL-2 interceptor. This may explain why Keegan made such an unexpected recovery from HUS. We also gave Keegan five doses
Most recently, Keegan's bone marrow biopsy results show signs of hemophagocytosis. He also has had some recent bleeds in his brain that may have been aggravated by the MAS/HLH.

Some of Keegan's labs have improved with the current course; some have not. His inflammation markers are slowly trending down. One level, ferritin, has normal values of 0-75. Levels over 10,000 are the cut-off for chemotherapy in general according to hematology's protocols. Keegan's levels peaked on Saturday at 48,000. His blood counts improved slightly with steroids and the blood transfusion, but they have already returned to their old levels. He is still bleeding, and his blood has not regained the ability to clot properly, despite three transfusions of fibrinogen and a blood transfusion. He is either asleep, completely agitated, or "zoned out". It's this last development that has been of most concern. Tomorrow, we may have to run an EEG of his brain activity in order to know for sure that he is not experiencing seizures of some kind or repeat his brain MRI to ensure the bleeds have not grown.

Tomorrow the entire team of doctors will meet again to discuss Keegan's case. This time, the oncologist specializing in HLH at the hospital will attend, as will the ICU attending. These "team meetings" are astonishing to me. This never happens at home. I think we knew last week that what we were dealing with was extremely serious when we walked into a room of 20 doctors with drawn faces telling us how sick Keegan was. And we hadn't even seen the worst of it yet. Yet, it's really hard to admit that your child is sick when they are on the regular cardiology floor, not intubated, not on dialysis. There is obviously no denying it now, but it still could be lightyears worse. And that is what everyone is scared of, right now. Because "lightyears worse" could happen tomorrow, two weeks from now, a year from now. And what will that look like? Will it be something Keegan can come back from the next time? What should we or can we do to prevent a "next time".... especially one that he can't fight.

The decisions we will have to make tomorrow and in the coming days are ones that no parent wants or should have to make. It is not nearly as clear cut as it was at first glance. No one wants to do too much too fast if it isn't necessary, but we have no crystal ball. There is no way to know what will happen, no matter which path we choose. It appears that no one thinks it is as much of an "if" as a "when", but how much are we willing to risk now if we don't know when the "when" will come. We are trying our hardest to gather as much information as we can and surround ourselves with as many people who are experts in their fields as possible. And right now, I think that's the only thing we can do. No one is going to be able to make this decision completely for us, and unfortunately, there are enough different ways of treating MAS and/or HLH that not all of the doctors will ever be in complete agreement. Anything we do will be taking a huge risk. I just pray that we choose the one with the biggest benefit because I can't afford to lose him. And I refuse to let him go through this ever again.

So once more, I will ask for your prayers not only for Keegan but also for me, Gray, and every doctor working tirelessly to help him. We have no doubt that he is in the best possible hands. Those of his doctors, his family, and every single pair of hands lifting him up in prayer to the Father. Thank you for helping us to help him on this road.

Risk more than others think is safe. Care more than others think is wise.

Dream more than others think is practical. Expect more than others think is possible.

-Claude Bissell

Four Years

2011-09-19T23:11:00.000-05:00

It's quite surreal to sit in the cardiovascular ICU four years after our lives changed forever. I don't think I ever fooled myself into believing that life was going to be perfect after transplant, but I never in a million years have imagined where this road would take us. Keegan amazes me every day - whether at home and feeling well or here fighting to get through. We could not be more grateful for the ultimate gift that Johnston gave to you four years ago. We wouldn't trade a single minute. We love you, Bug.

Eye of the storm

2011-09-19T10:34:00.002-05:00

Another long day in the ICU. I can't quite get an eloquent post up tonight. Just wanted to share a few things:

Bleeding seems to have stabilized without any more IV clotting factors today. His blood counts did drop to critical levels this morning, but we transfused him, which gave him a good little bump today.
Keegan remains pretty well sedated today and will through tonight. We will start to wean his pain meds down and wake him up a bit more tomorrow.
He is not urinating spontaneously right now, only in response to IV diuretics. His numbers are trending the wrong direction but not dramatically. He begs for water when awake. We are praying this is a temporary set back that his kidneys recover from without much more intervention.
His inflammation markers are starting to slowly go down. His coagulation factors are stable today.
The team is rounding out a bit more research at the moment, but they think he may have been fighting this for a long time. It might even explain away the HUS.
This morning was a bit tenuous, with hematology and rheumatology having a vigorous discussion about the next step. The bleeding and continuously increasing inflammation had everyone on edge today. Hematology was ready to come down with "the hammer" to treat him (i.e. chemotherapy), and it sounds like cardiology was on board with that. Rheumatology made a great case for trying one more drug today before we go there. So we gave him a big dose of a second drug this afternoon to target the protein that "eats" up the platelets and attacks the protein release of antibodies. Tonight, he gets the last dose of "big gun" steroids.
The team is not ready to say Keegan is out of the woods yet. They are calling this the calm in the eye of the storm. Many kids relapse, and hard, as the steroids are weaned. There is not a lot of data supporting the "milder" drug we started today, but what exists is quite persuasive. At any rate, we are all willing to try the least harmful treatment first while it appears he is handling things ok.

That's about all the news fit to print today. We had to have a lot of other serious discussions today with the team, but we are all cautiously optimistic that Keegan will beat this, just as he always has. Thank you so much for your prayers and support. We are truly blessed.

Getting blood and pinking back up

Bleeding

2011-09-18T08:47:00.000-05:00

Keegan's bleeding has slowed down significantly this morning, after 18 hours of nurses, doctors, Gray and me all taking shifts holding pressure on the site. We ended up having to give him two different types of clotting factors to stop it. So far, we haven't seen any results of stroke or seizure, but it is a little too soon to tell. He has hematomas developing on both hips, his lower back, and down his legs. Despite that, the doctors actually think he has been "bleeding" actual bone marrow, not peripheral blood. The danger with that is that while he may have relatively "stable" blood counts right now, as the circulating blood is used up, his bone marrow may not be able to replenish it after so much loss. We won't know that until it happens though, so as with almost everything else, for now, we just wait and watch.

Holding pressure last night

We are starting him on a PCA (pain) pump of dilaudid this morning to help keep his pain and agitation more under control. The more upset he gets, the more he bleeds. His GI system is also taking a hit, so they have stopped using it altogether. If there is not an IV form of a med, it is not being given. His is not urinating without the help of diuretics right now, but we are hoping that will self-correct in time.

We are waiting to see the rheumatology and hematology teams this morning to see if they will proceed with the next immunosuppressant drug today to slow down the MAS or wait until tomorrow after the last dose of steroids are given. We will be in the ICU for a few more days at least. In the coming days we will see more results from the bone marrow biopsy, then we may have a slightly better idea of which drugs will work the best to treat him in the long-term.

More later. Words are simply not enough to thank you for your prayers for Keegan right now. We are beyond grateful.

Resting this morning

Back to the ICU

2011-09-17T23:59:00.000-05:00

Keegan was transferred to the cardiovascular ICU here in Boston this afternoon. We were able to stop the bleeding from his bone marrow biopsy site last night for a little while. It started up in earnest gain around 11am, and it hasn't stopped since then. He is also pooling blood into his hips and back because of the bleeding problems. They are doing what they can within the realm of the Macrophage Activation Syndrome (MAS). If they give him certain things to help his anemia, his body won't be able to use it. If we give him clotting factors, his bone marrow is so confused that it could risk forming a clot somewhere besides the site where we want them too. The doctors truly believe that if we can treat the MAS, we can get his blood/bone marrow to behave a bit better and at least benefit from any therapies we start.

Keegan's blood counts have continued to drop and inflammation markers have continued to rise. Despite this, the doctors think that it's a good sign that the fever has not returned since last night.

*****
That's all I was able to write earlier. Keegan has been bleeding for over 12 hours continuously now. Giving IV clotting factors because we have to now. Fentanyl and valium too, as he bleeds more as he gets upset. Will update more tomorrow. Thank you for continued prayers for our Bug and our family.

Earlier this morning, snuggling with his Bummer Bear and puppy.

He should be pinging off the walls with this steroid pulse, but he has spent more time asleep than awake.

A good thing because he is so uncomfortable otherwise.

Biopsy and steroids

2011-09-16T22:19:00.000-05:00

I really, really want to write a good update that explains everything that's going on. I hoped that I would have had time to do so tonight, but things have just been too hectic. I'm not sure my brain can put it all together, but I will try my best. As I type, the first dose of the steroid pulse is being hung.

First, let's catch up from yesterday to this evening:

After Keegan's fever started getting out of control last night, it became apparent that this disease process was progressing faster than we could treat with the "benign" option of IVIG. I won't even go into describing what that spike was like. It was terrible to watch. He finally stabilized and fell asleep. We restarted the IVIG because we knew it was necessary, and he did ok through the night to stay out of the ICU. Temperature started to spike again around 5:30am.
We tried to keep him comfortable all morning. Very difficult to do. Because his bone marrow biopsy was an add-on, it took awhile to get an OR time, and he finally went back around 1:30pm.
Some bleeding is expected after a bone marrow biopsy, especially on some of the drugs that Keegan is on already. The inflammation has started to "consume" Keegan's platelets, which are the part of your blood necessary for clotting. Even the ones that are still there are damaged enough to not perform well. Keegan continued to bleed heavily for over four hours after the biopsy. After pressure had been held on it for over an hour and topical agents failed to work, we had to give him IV clotting factors. It is still oozing but not gushing like it was. There is a possibility this steroid pulse will cause the biopsy site to start bleeding again, but if so, we have a plan to treat it per hematology. It's a good thing we gave him blood a few days ago. He will likely need more in the next day or two.
The initial read on the bone marrow biopsy didn't show signs of a leukemia or an infection. There are a slew of more tests to run on the marrow, but that will take a little time. With those two off the table, we were clear to proceed with the steroid pulse.
The doctors have carefully planned out the best way to administer the steroid pulse, which will include more diuretics and some other drugs to help him tolerate it as best as he can. The first dose should end around 3am. More labs will be drawn then. Another drug will likely be started tomorrow to treat the inflammation, along with two more days of steroids.
Keegan is starting this pulse out without being in the ICU. He will have to have vital signs (blood pressure, heart rate, temperature, etc) taken every 15 minutes for the next four hours. If at any point he starts to worsen, he will be transferred to the unit though.

Again, I will do my best to get a post up in the next day or two with more information. The most likely answer right now is that Keegan has developed something called Macrophage Activation Syndrome. The inflammation in his body is such that the steroid pulse has to be started tonight. He cannot even wait until tomorrow morning. This is the same three-day steroid pulse he would be getting if he was rejecting his heart. The drugs we will need to use after the steroids are very similar to chemotherapy, and it will be a long road to recovery.

If my eyes weren't completely crossing, I would right more, but that's about all I can handle right now. An even longer night awaits us. We are so extremely thankful for the outpouring of support and love that has been shown to us this week. We are scared, and your presence on this road as we stumble along means so much. Thank you.

Tonight

2011-09-15T22:18:00.000-05:00

I don't even know where to start this update. It will be a brief one for tonight. Keegan is not doing well. He had a very rough day of high temperatures. The IVIG we gave him last night did not stop the inflammation occurring in his body. The markers continued to rise, while his blood counts continued to fall.

We had a long meeting with all the doctors today. The plan at 4pm was to repeat another dose of IVIG and get him scheduled for a bone marrow biopsy tomorrow morning. Keegan did not follow that plan, unfortunately. I don't have the energy for all the details from tonight, but he is toeing the line for an ICU admission right now. He spiked to 105.3 around 8pm and was not initially responding to anything we gave him to stop it. He has come down to around 103 now and finally is resting without pain.

Keegan really needs this bone marrow biopsy in the morning. They are trying to move him to first case. We need the biopsy to rule out a few things before taking the next steps. If we see what we are anticipating seeing in his bone marrow, Keegan will go to the ICU tomorrow to start a three day pulse of very high steroids, usually used to treat rejection. After that, he will get one of two other drugs to stop his immune system from going nuts, like it is now.

Prayers for tonight:

That Keegan is able to tolerate the rest of the IVIG infusion and that it buys him enough stability to get through the biopsy in the morning.
For his safety through the steroid pulse. Many complications can arise from this, as the steroids and the drugs he will need after that, will essentially wipe his immune system out completely. The coming weeks are going to be even more difficult.

I will update tomorrow with more detailed information and explanations. Right now, I want to wait until we have the biopsy results and a clearer plan before putting anything out there at the moment. Thank you for your understanding. And thank you, more than anything, for your prayers for our Bug. We love him more than life itself, and we are very, very scared right now. Thank you for supporting us all during this time.

"I have made you, and I will carry you.

I will sustain you, and I will rescue you."

Isaiah 46:4

Prayers

2011-09-14T23:00:00.000-05:00

I know I was sleepy writing last night's post. Guess I didn't realize how badly until I read it again just now. Sheesh. Pardon my poor grammar there.

Tonight, I'm just as tired, but it's going to be a long night ahead. For those reasons, I'm posting two prayer requests only tonight. I will try to update in more detail tomorrow after we have a big team meeting with all the attending doctors.

A few extra prayers for Keegan. His labs took a huge nose dive this afternoon, and inflammation markers were through the roof. This prompted an immediate response from the cardiology and rheumatology teams. They wrote for a whole slew of new lab tests that required an insane amount of blood. We'd been dancing around a blood transfusion the last few days, but now, it is an absolute necessity. Again, I will explain more soon, but it appears Keegan is in a cytokine storm (you can look it up). There are two explanations for his disease process. The doctors think what he is fighting now has been an ongoing process for at least the last three years, if not from birth. Again, we will know more about which one they are leaning towards tomorrow. Neither one is a "good" thing. Let's leave it at that for right now.
To stop the inflammation without having to put him in the ICU tonight, they immediately wrote for IV tylenol, benadryl, IVIG, and then follow it all up with a blood transfusion. That is a lot of fluid on his already swollen little body, so we will chase it afterwards with diuretics. We are extremely thankful that the team caught this early and responded quickly. Otherwise, things could have gotten very scary. Despite all this, he actually seemed to feel a little better than yesterday and was in generally good spirits all day. His temperature stayed between 99 and 101 again.
Please pray for guidance, clarity, and wisdom for the medical team, Gray, and me tomorrow as we try to get a good plan in place based on what we know.
Lastly, please pray for our little friend, Rylynn. She has had to have a chest tube and abdominal drain put in to help her out and is now back on a ventilator. Praying she stays strong and brave waiting on her angel heart. Mostly, we're praying for that miracle to come soon!

That's literally all I can muster tonight. I will do my best to explain it all as soon as I can. Been a bit busy around here. Thank you so, so very much.

Busy day

2011-09-13T21:20:00.000-05:00

Keegan had a rather busy day. Our suspicion that the fever wasn't going to really follow a "cycle" anymore, but rather become one constant fever, appears to be true. He had a brief reprieve yesterday afternoon, but he came back up to the high 101s last night. Today, he stayed between 99.8 and 100.5 until early evening when he started to get back into the 101s. It's not looking good for tonight.

He looked downright awful today. He is very swollen, and his weight is up even more. He has absolutely no color whatsoever. His eyes are red-rimmed and puffy. Keegan didn't get out of bed but once today. Despite having a great stash of new toys, he barely played with anything at all. After getting some good smiles and lower temps out of him yesterday, I think we were all hopeful that today would be a better day than it was. I hate to see how this disease process is taking so much out of my amazing little boy.

Keegan did have another brain MRI and angiogram today. They were trying to track down the images from the last one in June before making any determinations. They didn't see any signs of inflammation in his brains. There was evidence of quite a few micro-bleeds, but we won't know if there are more/bigger/etc until today's scan is compared to the previous ones. We also took him over to the opthamology clinic this afternoon to have his eyes checked for inflammation. The doctor didn't think she saw much, but he wasn't a great participant in that testing either. There are a few other lab tests hematology wants to run, and they are trying to decide whether or not to do a bone marrow biopsy. Keegan hasn't had one since January 2009. Then, all the attending doctors on Keegan's case will meet by the end of the week to make some decisions.

I'm not sure what to think in all this. Some of the last remaining ideas they have for a definable diagnosis are very scary, but I'm almost more scared that we will likely end up calling it some kind of immune disregulation that leaves us just guessing on the best course of treatment. Even then, the drugs that are being thrown around are no laughing matter. It's starting to feel like a no-win situation, no matter what. As we wrap up two full weeks of being in patient here, we can see that this is going to be a very long road either way.

Thank you for keeping Keegan in prayer, along with everyone working tirelessly to help him feel better. We are praying the Lord will keep his loving hand over us at all times as we make the difficult decisions ahead. Thank you so much.

Keegan is 4!

2011-09-12T23:36:00.003-05:00

It would be lovely to say that Keegan had an awesome fourth birthday. But I can't. It did end better than it started, so that is something to be thankful for, I suppose. Last night, he had another rough night of 103-104 degree temps with lots of discomfort and nausea. This morning, he spiked again and got sick. He felt horrible, was hardly moving, and would barely let you touch him. One more IV tylenol and dose of zofran brought him back to life. He spent most of the day with a temperature in the 98s - a first since Saturday. His attitude improved, and he showed a little more energy, even getting out of bed to the playground for a bit. With the fever down, his joints felt somewhat better, and he was able to get almost to a run momentarily. Keegan's temperature came back up a little before bed. This could be the end of this cycle, but I don't think anyone will be sure until he makes it through the night without a spike.

Tomorrow, Keegan will have another round of general anesthesia in order to do a special type of MRI of his brain called an magnetic resonance angiogram (MRA). We are getting closer to having a plan to treat Keegan's immune dysfunction and hopefully stop the fevers. It is going to require a trying different drugs until we find what works. Unfortunately, that will involve a lot of waiting and watching, so we will have big decisions coming our way soon. Before we take such big steps though, the doctors are trying to rule out everything they can and learn as much as possible about Keegan first.

What was awesome about Keegan's birthday was the amount of cards, messages, emails, and phone calls that he received today! Not to mention quite a few cool toys that happened to come his way. The Child Life specialist brought balloons and a neat racetrack toy for him. She also ordered him a cake, and we all sang happy birthday. No candles allowed in the hospital, and he wanted nothing to do with the cake. But we tried, and the nurses were more than happy to share his cake. Thank you, thank you to everyone who reached out in one way or another. You truly made this day special. Even if Keegan didn't understand, Gray and I did, and we are extremely grateful and humbled by your love and support for our Bug.

Keegan also received one more cool gift today. There is a great guy who volunteers at the hospital here. He spends time with families and kids who are inpatient and helps them make movies or slideshows as a special way to remember, commemorate, heal, cope, or celebrate. Whatever the patient or family feels like doing. So today, he helped us make a special birthday movie just for Keegan. It was a perfect way for us to celebrate Keegan today. Keegan watched it several times. I loved hearing him say, "that's Aww-duh-wee" or "it's Keegan!" when the pictures scrolled by.

Happy birthday, Buggy! I hope your coming year brings healing and growth that we are yet to even imagine. We love you so much more than you could ever know, and we are amazed every day by your strength, courage, and resiliency. You are an inspiration to everyone you meet. You are a shining example of God's boundless love. Keep fighting, Keegan. We are with you every step of the way.

So much for hoping

2011-09-11T21:58:00.001-05:00

Last night, I was marginally hopeful that Keegan's latest fever cycle (#13) would break overnight. I was wrong. Very wrong. He had two 104 spikes overnight requiring IV tylenol, and once again, he did not come below 100.5 during the day. He did spike up once during the day. We held off on the tylenol, and he came back down to this new baseline on his own after an hour or so. Just like last night, he is sweating madly, has come down to 99.8, but his heart rate has stayed the same. Not looking good for tonight. Tomorrow will be day 6 of this cycle....if it is truly a cycle that will break, rather than some strange new permanent fever.

Perhaps as a direct result of the new intensity of the fever, Keegan has completely stopped eating and is hardly drinking water anymore. We ordered him a pizza for lunch because he seemed to be in good spirits late morning, despite his temperature. We put it on his tray, uncovered the plate. He barely had time to smell it before he threw up horribly. GI lifted all restrictions on his diet, just to see if he would eat anything at all. We had told him that his absolute favorite food of all time used to be quesadillas. We ordered them two nights in a row. Last night, he took one feeble bite of tortilla, but tonight, he told us to take it away immediately. I think Gray and I were both hopeful that he would have some appetite for at least something, but now even goldfish crackers are being refused. I can't say I blame him though. Who does want to eat when fighting a fever?

The other significant change recently has been the beginning of inflammation in Keegan's joints, most prominently his ankles. We noticed it first on Thursday. The video below was taken on Friday. He is now having trouble just walking and cannot run anymore at all. Rheumatology is aware of it, but they are trying to round out their last ideas before doing anything just yet. Obviously this is something autoimmune, just not sure what yet. They are waiting for the images from his last brain MRIs tomorrow, but it sounds like Keegan will have a repeat brain MRI (actually an angiogram) and possibly a CT of his legs. For now, he doesn't feel like getting out of bed much anyway, so we are doing our best to massage his legs to keep him comfortable for now.

We did not draw any labs today to give his body a break. Tomorrow, we will see if he is in need of a blood transfusion again. My gut instinct is he will. The motility study will not happen tomorrow either. It may not even be this week due to scheduling conflicts. We certainly need to proceed with it to address his multiple GI problems. However as this fever has evolved and the inflammation has begun to attack his joints, it is seeming less and less likely that whatever is causing the fever is also causing his GI issues.

Tomorrow morning at 6:24am, my baby boy will be four years old. An eternity in the blink of an eye. I'm praying tomorrow will be a good day for him. We are ready for one of those. And he deserves one of them too.

Day five

2011-09-10T21:22:00.000-05:00

This was day five of this fever cycle. Keegan is really keeping us all on our toes. This one came on more slowly than before but stayed higher for longer than any previous fever cycle. Another very rough night last night followed by a rough morning before he evened out a bit. He hasn't come below 100.5 all day long. His heart rate is a smidge lower than it was at bedtime, so maybe we are headed in the right direction here. Here's hoping.

Ideas

2011-09-09T21:44:00.000-05:00

I have got to be more careful about what I post. Never a good idea to tempt fate with putting something in writing.

The great "oral tylenol + ibuprofen" experiment was an epic fail last night. Other than the night after Audrey's birthday party, this was his absolute worst fever so far. As I posted yesterday, Keegan's fever simply laughed at the oral tylenol. It temporarily bowed to the ibuprofen, only to come roaring back about two hours later bigger and badder. He climbed to 103.9, probably higher, but we were too busy trying to control his horrible nausea and vomiting to continue to check his temperature. We know that Keegan's body restricts blood flow to his stomach during the fever episodes, causing retching and vomiting. Ibuprofen can also cause stomach upset. The combination of the two probably put him over the top. I don't know if it is the ibuprofen or the fever or both for sure because that was literally the first time in his life to ever receive ibuprofen. At any rate, we finally were able to get him an IV tylenol dose and zofran around 3am, and he was finally asleep again just before 6am.

Our brave Bug this morning

I think Keegan's fever was ticked off that we attempted to change the plan of attack, and it came out for another round around 8am, reaching 104 by about 10am. Another IV tylenol dose worked, and he finally got some sleep until around 2pm this afternoon. He "simmered" as usual all afternoon, and by bedtime, he was back at 102. We've held off on the meds so far though because he is asleep. We are trying to treat the symptoms as needed and not just react to the fever by overmedicating him. Sitting here watching his heart rate continue to climb, I know that he will need another dose. We're just trying to be safe with the bigger gun medications.

If the last two weeks' pattern persists, this should be the last night of big fevers with more "simmering" tomorrow before it finally stops. This one has done a number on his labs. We drew some repeat numbers just now, but he will probably need to have a blood transfusion, at a minimum, tomorrow. He looks like crud and is refusing to leave his bed or room for the most part. It was an absolutely beautiful day here today after a few rainy ones. We convinced him to go to the garden, and ultimately, he walked around for about 10 minutes before giving up. The last two days, Keegan has been walking with a very pronounced limp/stiffness. We thought it was a fluke yesterday, but it was very prevalent this evening. Another thing to talk to the team about tomorrow.

Playing yesterday from bed

If we can take away anything positive from a particularly bad fever cycle, it is that the team here is getting to see it in full swing. Hopefully, that will help provide more insight to them as they try to figure this out. I mentioned yesterday that there are a few things brewing as far as ideas for diagnoses (or lack thereof). Basically, it comes down to one last possibility that could unify both his GI problems and fevers, or GI and transplant addressing the two separately.

On Monday, Keegan will undergo a motility study that should give us a bit of insight as to how well the nerves in the end of his colon are working. If there is a problem with the nerves, we will confirm with a biopsy before considering which way to treat it surgically. This is the one last idea that would unify the fever with the GI issues. Basically, children with this condition are very susceptible to translocation of bacteria within the gut that can cause fever fluctuations. To avoid a complicated explanation of this problem that may not actually be a problem for Keegan, I will leave it at that until we get the results of the study on Monday.
If the results of the motility study are "normal", then GI has pretty much run the gamut of every possible test or idea for treating Keegan's diarrhea. We will have to have a very lengthy discussion with the team, but it sounds like the overall plan at that point would be no different than what we had talked about with our doctor at home. Reduce his TPN to the absolute minimum needed to maintain growth; assuming that he will have diarrhea with formula or solids, allow him to eat normally without the tube feeds. I use the word "normally" cautiously though because we are getting absolutely nowhere with eating. A serving a day of goldfish crackers or pretzels is not going to cut it for very long.
The transplant team is calling upon the immunology and rheumatology doctors here to help them continue to research the fevers independently of the GI problems over the weekend. If the results of the motility study are "normal", then they want to be sure they haven't wasted any time working up the fever. We met with rheumatology today. They were going to meet with the entire team, but they at least think we need to repeat the MRI of Keegan's brain to check on the micro-hemmorages there. They also were going to consult hematology about his many different blood issues, and what they might tell us about the overall picture. We had discussed needing a bone marrow biopsy a few months ago, and that is something we are leaving on the table for now here. The immunologist here was still reviewing the results run by the immunologist we saw in Dallas, but we anticipate seeing them in the coming days.

A visit from the clowns yesterday

Bubbles

All the other little bothersome things that need to be addressed with Keegan, like his fluid retention/blood pressure/immunosuppression levels, have been somewhat placed on the back-burner for now. We are addressing the most serious issues at hand first. This latest fever cycle has only reiterated the need to stop this out-of-control spin Keegan is taking us on. All that being said, I think you can gather that this is shaping up to not be a short stay in Boston.

But Keegan is stable tonight. So, I am asking you to save your prayers for him and instead focus on our friends Leah and Rylynn. Leah's transplant went well overnight. However, she is struggling with a post-surgical fever, and her lungs are having a difficult time adapting to her new heart. Rylynn, who is on a Berlin heart while waiting for her miracle, suffered a very serious consequence of the pump yesterday when she had a small stroke. It looks like she hasn't suffered any major complications from it, but today, she did have to go back to the OR to have the pump replaced. It was done successfully, so we are praying that prevents any further issues for awhile. Please, please keep these precious girls in your prayers. They both need them desperately tonight. Thank you, friends.

#13

2011-09-08T23:02:00.000-05:00

I apologize in advance, but this is going to be a short post. I will try to round it out a little more tomorrow when I have a little more energy. Just didn't want to leave y'all hanging.

Keegan's thirteenth fever started Tuesday, but the high temps (102s) didn't kick in until earlier tonight. The team here in Boston wanted to try to switch Keegan back to oral tylenol. We tried that when he hit 101.5 and watched his temperature sail right through it. So, then we tried the oral ibuprofen (well, through his g-tube really, but you know what I mean). The good news is that the ibuprofen worked - temperature down into the 98s but heart rate still high. The bad news is that Keegan's kidneys can't handle ibuprofen on a regular basis. While it's useful for at least letting him be comfortable and sleep for the next few hours, we probably won't be able to use it again this fever cycle. I guess we'll take what we can get for now.

"I don't feel so great, so please get that camera out of my face, Mama."

Even though it felt at times the last few days that we were just waiting on a fever, wheels have been turning and ideas formulating. Right now, we are trying to get Keegan scheduled for a particular motility study on Monday that may tell us if there are missing/underdeveloped/damaged nerves in his colon that could be responsible not only for his GI problems but also for the fevers. The down side is that Monday is Keegan's fourth birthday. Having this test then is kind of the equivalent of scheduling a colonoscopy on your 50th birthday. The up side is that Keegan doesn't know it's his birthday or what that really means. He will like his toys and being sung to, but other than that, he is oblivious. If this test is "positive" for a nerve problem, there will be a difficult road ahead for Keegan involving some surgical adaptations. If it is "negative" (and I'm way simplifying things here for convenience's sake), that puts us back at "square one", with no other ideas on how to deal with the fever and resorting to just finding Keegan's balance between TPN/tube feeds/solid food/diarrhea/growth, etc.

Again, I will try to clarify more, if I can, tomorrow. I have a long night ahead of me, unfortunately, with Keegan's fever, so I need to sign off. I would like to ask you to include our sweet little friend, Miss Leah Grace, in your prayers tonight. Leah has been waiting on her angel heart for 2 years now, and today, she got the call! She is in the OR right this very minute. Please pray for her smooth recovery and continued health with her new heart, as well as prayers of comfort and strength for the family grieving the loss of their child tonight. Thank you so much, friends.

Tuesday

2011-09-06T21:49:00.001-05:00

Brilliant title, I know.

Keegan had a pretty good Labor Day for being in the hospital. He went outside and played in the playroom. His port appears to be doing well, so there was no need to reassess his line situation since losing the PICC on Sunday. He was generally in good spirits. Until we decided to order some mashed potatoes for him at dinner time. I think we had all been operating under the assumption that once permission was given to eat, no matter what was offered, he would eat it because he hadn't been able to for so long. We were sorely mistaken. We have offered potatoes, pasta, chicken, and vegetables. So far absolutely everything offered has been not only rejected but resulted in major, screaming meltdowns ... except goldfish crackers. And only goldfish crackers. That's not really helping GI make any determinations about the state of his intestines. They need him to eat some more complex foods, which he is flatly refusing to do within the realm of his allowed diet.

He has tolerated about two full servings of crackers per day so far without throwing up. The diarrhea took a definite up-tick. We were unsure whether or not to attribute to the food or not. I think we are still unsure, but at least today we were able to collect the last few stool samples for labs. These labs may give us a little guidance as to where in his intestinal tract the problem is occurring, i.e. large vs. small intestine. The team is also weighing the need to proceed with a full motility work-up. It's not a fun process, and frankly, the results might not tell us much anyway. For now, we will proceed with adding more dry carbs and offering real food daily, even if it is declined. All bets are off during fever cycles though. We know he doesn't tolerate minimal tube feeds during them, so solid food is likely not going to work either.

As Keegan's fever pattern has morphed recently, we weren't sure whether to expect it last night or tonight. Only a few times has he been inpatient and on monitors for the initial spike. The silent indicator of the fever is his heart rate. It is normally in the upper 90s-100s while he is asleep with no fever (a little higher than the average for his age but not uncommon for a transplant kid). At the highest temperatures, he will reach the 170s, and he will not come below the 130s until the entire cycle is over. The benefit of being inpatient on the monitors is that we can theoretically see them coming. Usually, he will "simmer" for a day before the first spike. He will get flushed, feel warm even if his actual temp isn't too high, and slow down energy wise. We saw all those things happen today, so everyone is a little on edge, Gray and I especially, waiting to see what Keegan will do. Since he has been asleep, he has stayed mostly in the upper 110s, and he has already had one moaning/crying-in-his-sleep episode that we are familiar with the night of the first spike.

The good thing is that we feel like the transplant team has had time to catch up and prepare for the next fever cycle, whenever it hits. The plan is to try oral tylenol again and possibly a dose of ibuprofen (just one, and one only since it is a big risk for his kidney) before going back to the IV tylenol. They really want to see what he does in order to determine if the fever follows a particular pattern that could clue them into the source. But we won't let him get uncomfortable either, and unfortunately, the highest temps tend to be at night when he should and needs to be asleep. The team hasn't clued us in to any thoughts they may be having at this point in regards to a cause for the fever, if any. We will just have to wait and see.

Thank you to everyone for your continued love and support for our little family. We are so appreciative of every prayer and well wish. I am really struggling with being apart from Audrey. I miss her every minute she is not with us anyway, but to top it off now, she has her very first ear infection. I don't think my heart can break much more knowing that I can't be there for my little girl when she needs her mama most of all. She seems to be responding to the antibiotics, but I would adore it if y'all would join me in prayer for my little Ladybug. I hope she knows how much we love and miss her and would scoop her up and never let go if we could come home today. I love you so much little girl...to the moon and back!

Bye-bye PICC

2011-09-04T22:07:00.000-05:00

There truly is never a dull moment with Keegan. Never an opportunity to have just a normal, fun or relaxing weekend.

There is a small playground at the hospital. Friday afternoon when we finally convinced him to get out of bed, we showed him the playground. Keegan probably hasn't been to one (except for his little fort in the backyard) since at least January. He went down the slide a few times and walked around, but he was fresh off his last fever and not feeling quite right yet. So, we went back yesterday afternoon and did a little better.

This afternoon, the hospital's air conditioning had a brief slow-down, making it a little steamy in the room. Since Daddy had stepped away to get some work done, I asked Keegan if he wanted to go outside. This time, he said "slide?" immediately. There were a handful of kids out there, and he was slightly apprehensive about all the running around they were doing. So, he took a seat on one of the benches to watch. The benches are made of a metal "mesh" with round holes...exactly the size of the end of Keegan's PICC line. He tried to turn around on the bench, and in an instant, his PICC got caught in a hole and stayed there. Even as Keegan stood up. Ugh.

I put pressure on his arm, and scooped him up to run back to the room. The nursing staff and resident handled it very well. We stopped the bleeding and did an xray to be sure none of the line broke off in his vein. They measured the line and called Dallas to see if there was any documentation of the length of the line. There wasn't, but from our best estimates and a clear xray, it seemed like a little blood from the pulled stitch and insertion site was the worst of it.

Luckily, he still has his port. It was still leaking two weeks ago, and the original plan here was to worry about that after everything else was over. Our hand has been forced into using it for now. Maybe we'll get lucky, and it will start behaving now. The oncology charge nurse came over from Dana Farber and accessed it. Different type of needles here, which might help. That wasn't a fun cap to the day, but at least he gets his IV nutrition tonight. He will need it considering the minimal eating he is doing during the day.

GI instructed us to pick one new food every two days. Simple carbs, no fats or fruits, and no leading tummy-upsetting/allergy culprits, i.e. dairy, eggs, soy, shellfish, etc. That basically leaves us with plain crackers, vegetables, and bland meat. We started with the ubiquitous graham crackers. There was no additional enthusiasm for them yesterday. I think he ate two, instead of one. So, today we went with an old standby...plain goldfish crackers. He ate 30 this morning and then tried to throw them up on the cardiologist. He refused them the rest of the day until around 5pm. With George to distract him on the TV, he downed 60. And kept them down! It's better than 2 graham crackers!

Unfortunately, he has had quite a few bad diapers yesterday and a few today. I think it's too soon to tell if it's directly related to the food or not. We need a few more days to be sure. It's not as bad as it could be, I suppose, so we'll take that. I'm not sure what any of us were expecting to happen, which makes it a bit tougher to decide what it means. Tomorrow we will offer more goldfish crackers. Then, it will be about time for another fever spike, and any conclusions about his reaction will be clouded by the effects of the fever. One day at a time for now though.

This photo is a bit random, but I thought it was fun.

There are two big maps on the floor, of the US and the world, and you get to pick a sticker to show where you live. If you notice, there is only one blue dot from Dallas. That's Keegan! The first one on the map! There are dots from everywhere, even Iceland. Really fun.

We had a great discussion with the cardiologist on service this weekend yesterday. She spent a lot of time answering our questions, discussing the differences in their approach to transplantation, and going over our expectations for this trip. Even though she's not our primary here, we really enjoyed having her spend uninterrupted time just getting to know Keegan and talking shop. The transplant team is trying hard to get caught up in time for the next fever cycle. They want to be able to evaluate it based on what they know and not just react to the fever itself. Just like with the port, there are a lot of things they want to just leave alone while they focus on the fever and the related autoimmune reaction, i.e. his immunosuppression regimen, his blood pressure, his fluid retention, etc. The fevers top the list of to-do's with Keegan, so we are trying to keep everything else stable for now. That being said, we likely only have one more day to get ready if the pattern from the last two weeks hold.

A little drama here. A little drama at home. Audrey has been running a low-grade temp. She doesn't want to eat and feels pretty crummy. I feel worse knowing my baby is sick (for the first time), and I can't be there to make her feel better. Absolutely breaks my heart. Please say a little prayer that it's just a little virus that passes quickly.

And also a very, very big prayer for our sweet friend, Rylynn. She is relying on a Berlin heart (a ventricular assist device) while she waits for her angel heart. Ry had to go back to the ICU today because her kidneys were starting to get a little unhappy. Please join us in praying for lots of wet diapers and a better day ahead for Ry and her family. Thanks friends!

Happy Friday

2011-09-02T23:01:00.002-05:00

Keegan's fever broke early in the morning today. He was amiable but refusing to leave bed for most of the day. All in all though, it ended up being a pretty happy Friday. We had our first big meeting with GI today. As always, Dr. Kamin is tackling things head-on with Keegan once again. They are still reviewing his history, his old procedures and labs, and reviewing with us to ensure they have everything right as to how Keegan has been doing from an intestinal standpoint. So, they haven't come up with an overarching plan or any new possible diagnoses just yet. Dr. K was trying to collect all the data before jumping to any conclusions. In order to do that, he felt we needed to at least jump on the opportunity of no fever over the weekend and try to challenge Keegan's gut a little. The fevers throw a big wrench into the picture. Since we are all in general agreement that the fevers are indicative of an immune response/inflammation process, it is no surprise that they do affect his GI system. They probably would in a normal person, even more so with a not-so-normal one.

So, how do you challenge Keegan's gut? For the last several months, it has seemed that even minimal amounts of a very diluted, very elemental formula challenge him on a daily basis. The team felt that since the tube feeds were doing absolutely nothing for Keegan and he is stable on TPN for nutritional support, we should try to stop the tube feeds and add in some solid food in a very planned and controlled environment.

And so, Keegan ate a graham cracker today!

His first food by mouth since late February!

"Wait...you want me to eat that?"

He looked at it for awhile. He licked it. He nibbled it.

Not so sure about it.

Kinda remembering what it tastes like. Still not so sure.

"Really funny, guys. You can take these back now."

Not exactly the response we were hoping for. I can't say that I am wholly surprised though. Keegan ate a little bit of food back in January to February, but he really hasn't eaten consistently since early last November. He did ultimately eat one square of the graham crackers. We offered more multiple times today, which was declined each time. Hey, at least he was polite about it. We will offer more tomorrow and possibly Sunday, just to watch what happens with his diapers. He had a few bad ones after the graham cracker, but I wouldn't say just yet that it was a direct response. Could just be "normal Keegan" fluctuations.

But the tube feeds are gone, at least during this trial. Due to his kidney function and the steroids, he is extremely fluid positive right now and needs to get rid of a little over five pounds. The tube feeds were very, very minimal, only about 10 ounces per day. Losing them won't make much difference, but he has some wiggle room of healthy weight loss to cushion him too. This is not a permanent solution. No one is suggesting we just lose the tube feeds forever and continue with diarrhea and TPN long-term. That might end up being what happens, but for this weekend at least, we are just doing a test of sorts to see what will happen. Collect more data, as it were. As Dr. K said today, once you have all your dots, you can keep throwing them against the wall to determine if you see a new way to connect them.

Around 4pm, we finally convinced him (after a lovely little meltdown, of course) to take advantage of his newfound freedom and leave the room. He did fine once we were out and about. We found the playroom and grabbed some toys to keep him busy over the weekend. Then, we ventured outside into this beautiful 67-degree, ragweed-free evening.

"Hold my hand, Daddy!"

The Prouty Garden

It should be an interesting long weekend between him (hopefully) having a little break from the fevers and challenging his tummy. It's difficult to imagine being inpatient on his "good days" and keeping him busy. Other than the GI test, we have no other plans for the weekend. Transplant is going to let GI have these fever-free days to take over the show for a bit while they continue researching and pouring over Keegan's history. Then, they will ideally be more ready to tackle the fever when it reappears next week.

And in case you were wondering how Ladybug is doing, well, she is just as cute as ever.

Swimming at Great Uncle Tim's for Great-Grandpa Max's birthday

Sun and water are two favorites of this little fish! Takes after her mama that way.

Miss these sweet chubby cheeks!

Big Daddy and Audrey...and yes, she's just in a swim diaper.

It's the baby equivalent of "skinny dipping"!

Oh yeah, and......

SIC'EM BEARS!!!

How's THAT for a season opener!?!?

Back in Beantown

2011-09-01T21:51:00.000-05:00

Well, we made it back to Boston. It was a very long day preceded by a very long night. Keegan's fever returned Tuesday morning, and it really hit him hard overnight. He could not get comfortable, even with IV tylenol, and was awake from 1:30am on. Yes, on. Gray and I didn't finish packing and getting things situated at the house until almost that time, and then, I would have had a little over two hours of sleep before needing to get up to make my flight. But those two hours were spent trying to help Keegan fall asleep. I just barely made it to the airport, and an angel must have been on baggage duty, as that's the only possible explanation as to how all of our bags also made the flight!

My flight left at 6:30am with a brief stop in St. Louis. I landed in Boston right at noon. Only to find out that Keegan's fever made the transport team have to take a few extra precautions, and as I was arriving, they were just leaving Dallas...two and a half hours behind schedule. Their projected arrival time was 7pm, which meant Keegan hadn't been assigned a room at the hospital in Boston yet and left me nowhere to go for the next six hours. Luckily, some lovely staff members on the cardiac floor at Children's agreed to lock our bags in an office while I waited. Being that it was a gorgeous 80 degree day outside, I took advantage of the almost thirty degree difference between here and home and just set out walking. I made it all the way to the Public Garden (probably a good two and a half miles), where I spent time with the Make Way for Ducklings statue, Keegan's very favorite spot in all of Boston. It was difficult to sit there alone and watch other families playing and laughing with each other. But it also reminded me of why we are here - in hopes that once again we will be one of those families too.

Well-loved Mrs. Mallard with her ducklings,

Jack, Kack, Lack, Mack, Nack, Ouack, Pack, and Quack

Keegan had a pretty difficult time on the first leg of his flight from home to Lexington, Kentucky. He got sick to his stomach right before they landed. It must have lifted some of his discomfort, and he was finally able to sleep for a few hours on the second half of the flight. When they left Lexington, Keegan wasn't looking very good, and the transport team was concerned they would need to stop in the ER on the way in. Luckily, he perked up enough to avoid that. He and Gray arrived here with the medical air transport team around 7pm and were taken by ambulance to Children's Hospital. This fever is mimicking last week and is heading into day 4. At least the team here is getting to see how these cycles go. He was quite uncomfortable last night and threw up this morning again when his temp spiked up high. We've only given one dose of the IV tylenol though because the team wants to see what he will do without it. Does it go on longer than 4 days? Do the peaks get higher? So far, he's looked pretty much the same. We will just have to wait and watch. We convinced him to go outside for a brief bit this afternoon, but he doesn't have much energy still.

Waiting in the Solid Organ Transplant office at CMC at home for the transport team to pick him up.

They took the exact same plane they took 2 years ago.

Looking at that tiny thing (rather than the larger CMC jet), I am thrilled with my choice to let Gray go with Keegan and fly on Southwest myself!

Loading up

Feeling yucky but always a trooper

Part of the crew

Today was another very busy day, trying to meet with doctors and fellows and residents. They are trying to get a good picture of Keegan's history and sort through all the records that came with us. He had an echo, EKG, and xray today. Our transplant cardiologist here, Dr. Blume, is not on service technically, but she is still leading the show. We learned some wonderful news today that our gastroenterologist, Dr. Kamin, is actually going to stay on Keegan's case, even though he now heads the ethics board for the hospital rather than see patients clinically. We can't wait to see him tomorrow, and hear what he has to offer. It will likely be a few days (which unfortunately includes a holiday weekend) before we see a "grand plan" start to come together. We anticipate visits from immunology, infectious disease, hematology, and possibly nephrology and genetics over the next week, too.

Safe and sound in Boston

We are extremely grateful for the prayers and well wishes that were sent our way yesterday. Every one was felt in earnest, and you truly helped ease our minds and hearts as we traveled separately, yet surrounded by love and support. It humbles us daily to know there are so many friends and strangers pulling for our Bug. Thank you for continuing with us on this journey. We have gotten weary at many points along the way, and it would have been just as easy for many to walk away, rather than trudge along with us daily. You help us carry on every day, especially the days when we could not imagine continuing alone. We are anxious to see the plan the Lord has for Keegan and our family on this new part of our journey. Thank you for traveling it with us.

"For I know the plans I have for you," declares the Lord,

"plans to prosper you and not to harm you,

to give you hope and a future."

Jeremiah 29:11

Perhaps...

2011-08-30T13:13:00.000-05:00

I hesitate to even say it, but it looks like we are good for Boston tomorrow. I just got a call from the transplant cardiologist there (yes, the actual doctor!), and she said they are ready for Keegan. The rest of the day will be crazy here. In addition to packing and getting the house ready, it was Audrey's first day of Mother's Day Out, and Keegan will be at the hospital downtown all afternoon for last minute infusions, nebulizer treatments, dressing changes, transport team meetings, etc.

Oh, not to mention that Keegan's fever has started again. Just in time. Please just join us in prayer for Keegan's safety on this trip now that he will not be feeling well in the air. Gray will fly with Keegan on the hospital's fixed wing plane, and I will be flying out commercially tomorrow morning. Very early mornings for all, so tonight will be the last time I get to see Audrey for awhile. Gray will be staying in Boston about one week and then will be back to work and taking care of Ladybug. While we are anxious to find answers for Keegan, we are also disappointed in having to separate our family and leave our home. We are confident this is a step in the right direction. Just one we take with great caution and trepidation.

And perhaps when we return, we will see these adorable siblings back together once again with big smiles and both feeling well and happy.

Hurry up and wait

2011-08-29T00:02:00.000-05:00

I realized my last post probably didn't make much sense unless you happen to keep up with our twitter or Facebook feeds. I intended to write a nice, well thought out post tonight. However, Keegan has been moaning/crying/rolling around in what might be GI pain or "looming fever" pain intermittently since about 8pm. Things never go quite as planned around here thought, so I can't be too surprised.

So in lieu of a lovely narrative, I present you with bullet points of the last few days:

WEDNESDAY NIGHT: I posted that Keegan's fever had gone on 4+ days and may or may not be gone. It was gone thankfully.
THURSDAY: Keegan refuses to get out of bed and seems to be feeling crummy despite finally not having a fever. We know that many of his labs were off. Between this apparent turn for the worse and Hurricane Irene barreling toward the east coast, things went into hyper-drive to try to admit Keegan to the hospital here, get insurance approval for medical air transport, and fly him to Boston Friday morning. I spend all day getting the house ready while trying to accommodate Keegan from bed. Without going into detail, suffice it to say it was sheer craziness trying to do all that and stay in constant contact with the two hospitals to see what would happen. Around 3pm, it seemed we were going. By 5pm, it was a maybe not, but they admitted Keegan anyway due to everything else from the week. By 7pm, it was a no-go.

Thursday in bed. Reading and resting.

FRIDAY: Keegan seems to be feeling much better. We decide to hold off on his infusions for a few more days and recheck levels Monday. We decide to go back home to wait out the storm and spend as much time as possible together as a family. There wasn't anything to be done at the hospital that we couldn't do at home. We left with instructions to return immediately if another fever hit, although we can't really predict them anymore since last week's was so uncharacteristic of the previous ones. Around 5pm, we receive word that insurance approval is in and good to go. Now, we just wait on Irene.

Keegan had a pretty good weekend overall. It's hard to look at him on a good day and realize how sick he can be the rest of the week. We know this trip is necessary, but it doesn't make it any easier. Fever hasn't returned yet, but we think he may be in the "simmer" stage. With the apparent discomfort he has been dealing with all night, we may be in for a whopper here soon.

The bright light in all the craziness of this week is spending some extra snuggle time with this beauty.

I sure am going to miss her.

I will post more tomorrow as I know it, but the goal is to be Boston-bound by Tuesday or Wednesday. If Keegan stays out of the hospital, we will try to do an outpatient-to-inpatient transfer, where he is picked up by the transport team from clinic. If he doesn't, we will do inpatient-to-inpatient. The hard part now will be getting a commercial flight for the parent that doesn't fly with Keegan (likely me, as my tolerance for small planes is virtually nonexistent). Ok, more tomorrow. Better get some sleep while I can, since it looks like a very long night ahead for our Bug.

Oh Irene...

2011-08-28T00:29:00.001-05:00

Just a quick note to say Keegan is back at home while we wait on hurricane Irene to clear the east coast. Insurance approval for travel is complete. (Yippee!!) if not for the storm, we would be in Boston by now. The goal is Tues/Wed based on storm predictions at this time. I will post more tomorrow....no really, I mean it. I hope. Been a little nuts here. Trying to spend as much time together as a family as possible the next few days while we get ready to travel, leaving little computer time. Thank you for all the well wishes, inquiries, & prayers. More soon!

- Posted using BlogPress from my iPhone

Got a map?

2011-08-24T23:10:00.000-05:00

Remember how I mentioned in the last post that if the fever went any longer, we would be in uncharted territory? Well, welcome to the wild. If we felt lost before, that feeling is magnified tenfold as this fever has started to morph in front of our eyes. It truly reemphasizes the need to get Keegan somewhere new... and quickly.

Keegan's last fever went on for four and a half days (whether or not it is truly broken remains to be seen). I had to talk our way out of being admitted last night. Everyone, us included, was worried about this new shift. But we felt like he was stable at home. With the promise that we would be in the ER the second we lost that feeling, we put him to bed. Last night, he stayed between 99.5 and 100.1 all night long. Until now, Keegan would really retch and throw up at the highest temperatures (103+). He did not process his 10ml/hr night feeds at ALL last night, and ultimately threw them up around 5am despite his temperature not being that high. He stayed in the 98s most of the day, which is still above Keegan's normal. Fingers are crossed and prayers said for a much, much better night tonight.

We spent most of the day at the hospital drawing labs, doing dressing changes, and meeting with the transplant team about planning issues for the trip. Keegan's PICC is still hanging in there, which is good news since the port is still leaking. We are going to continue to use it for a few more days at least to see what happens, but it doesn't look good. Unfortunately, his labs have taken a hit from these fevers. Keegan will need to get an albumin and IV lasix to give him a boost. He may also need blood soon, but that would be a lot of volume to give him in one day. At the end of the day today, I think the doctors were on the same page with allowing us to do this outpatient in the infusion lab. We are trying our best to keep him at home until the last possible minute. We need more time to get our lives in order before leaving, and frankly, we want to spend as much time as we can together as a family too. We have not nailed down a day for travel yet. The goal is still for the end of this week, but there are a lot of logistics left to handle.

To wrap up tonight, I wanted to share some pictures. Maybe not from "better" times per se because he had just recovered from a massive surgery, days from several other big procedures, and in between fever cycles. Despite all that, he looks lightyears better than he does today, and my heart needed to see his beautiful face tonight.

I think you can tell from the next picture how it breaks my heart to see how the last few months have affected him. He is still my beautiful little boy, and I love him so much it hurts. I just pray that we can find a way to help him feel like his happy little self again soon.

Another day

2011-08-22T23:03:00.000-05:00

Today marked another day of fever for Keegan. Last night was not easy on him. Consistently in the 103s. After another IV tylenol dose at 6am, he "simmered" at 99-100.5 all day. At bedtime, he was at 100.8. This was a marked change from all 10 of the previous fevers. It has already gone a good 12 hours past all the other fever cycles. If he spikes again overnight, we will truly be in uncharted territory with three straight nights of very high fevers. Keegan's baseline normal body temp is about 96.9, so you can add at least a degree or so to any temp he has. Other than IV tylenol, we have no other way to help him during these cycles at this point.

Today marked another day of line problems. Since he has been at his maintenance dose of steroids for a week, we attempted to access his port tonight. The plan was to use the port for a week with good results before pulling the PICC line. We know that his PICC is on it's last legs. After last week's dressing change, it became very sluggish. So Friday night, we ended up in the ER. After an xray showed the PICC had migrated a few centimeters, we determined that it was probably embedded against the vein wall. A power flush knocked it loose and allowed us to go home then. That little incident made us realize we needed to start using the port. Tonight, I tried twice to get blood return from the port. Then Keegan ended up in the ER again because it wouldn't budge. The hem/onc team was able to finally free it up after a lot of work. We do not have much confidence in this line for now, but we will just have to see how it all pans out.

Today was another day spent trying to arrange for our trip to Boston. This morning, the plan was to gather information and documentation with the goal of traveling next week. By this afternoon, the plan shifted to trying to get Keegan there by the end of the week. Nothing is set yet. Lots of work done today and hopefully lots more to be done tomorrow. We are working on securing an inpatient transfer again this time, which would involve admitting him here in Dallas to the hospital and transporting him there to the hospital via medical air transport. Keegan will be inpatient for an as of yet undetermined amount of time when we arrive. We have no timeline at this point for how long we will be gone, but we are betting on a month at least. I am paralyzed by the choice I have to make between my children. My son desperately needing this requires me leaving my daughter behind. A choice no parents should have to make.

Today was just another day of the same craziness for Keegan and our family. For almost everyone else though, today was the first day of school. I didn't have time to really think about the implications of today until I scanned the 5 million Facebook posts tonight of everyone celebrating the first day of school. I am only slightly ashamed to admit the bitterness I feel. We were there just one year ago. He was there just one year ago. How could our lives have changed so much? I knew then that Keegan going to school was risky, that it opened up a world of infection that he hadn't had to deal with before then. I never, ever, ever could have imagined that after 2 and half months, it would all be over. That our lives would be so drastically changed. Kidney failure, dialysis, ICU, intestinal failure, bleeding, early stages of rejection, fevers. A year ago, I tried to put my fears behind me. Little by little, I saw them come to reality, all but slapping me in the face and laughing at my audacity over the last year. Today, my grief is raw. That small sliver of hope was jerked away so quickly, it almost seems as though it never existed. A few weeks ago, we were told that Audrey couldn't even have a small dose of normalcy in going to Mother's Day Out because the risks were too great for Keegan. She might only get to go for a bit this fall while we are gone. I am ecstatic for her, for every child that experienced that glorious first day for themselves today, and for every child that enjoyed returning to school one more time. But it doesn't reduce the regret I have for the day that my son did not, and honestly may not ever again, get to enjoy.

But this is our life. This is the path God has chosen for us. Another day. Another step forward in the journey. We may slow down on days like this, but we have not given up. Another day survived. Another day lived. Another day to praise Him for our blessings and ask for strength for the challenges yet to come.

Care Calendar update

2011-08-22T10:44:00.000-05:00

Dear Family and Friends of the Harrison Family,

We have decided that it will be extremely beneficial if we extend the Care Calendar to resume providing meals for the Harrisons. As we mentioned before, our family is well aware that having meals provided even when home from the hospital proves to be a fantastic way to provide relief. After all, once home, all care rests on the parents and trying to even think about preparing meals can be a daunting task.

You may sign up at www.carecalendar.org. In Calendar ID, enter 82405 and in Security Code, enter 8772. Meals will begin Tuesday, August 23^rd and continue every other day through Sunday, September 4^th. Please click on the day on which you would like to provide a meal for the Harrisons and sign up at the bottom of the page for that day. If the date still says “MEALS”, it is still open. Please bring meals between 4 and 6 PM, but keep in mind that the family is unable to eat until around 8:00 PM, due to Keegan’s scheduling needs.

I know that Gray, Maddie, Keegan and Audrey appreciate your support through encouragement, prayers and messages on this website. Let’s extend that love further by doing something little to make a big difference!

Thank you,

Paul, Angie and Avery Grace Akeman

*Many thanks to the Akemans for wanting to continue this care calendar for meals. Many, MANY thanks for the meals so far. They are right that eating is usally last on our list these days. Taking that off our plate, no pun intended, certainly lightens the load. If we leave home before this calendar ends, we will keep y'all updated for sure.

11

2011-08-21T23:01:00.001-05:00

I miscalculated last week. Keegan's fever this weekend is the 11th overall. The 6th since we started the steroid pulse. It seems to have been about the same as last weekend's so far. There was a few hours earlier today where we got pretty worried about him. He was even more ghostly pale than we had seen in awhile. After an IV tylenol dose, he seemed nauseous, distant, and unnaturally cold on his head and hands despite an underarm temperature in the 98 range. We waited it out for a bit, and he seemed to shake that. At bedtime, he was starting to spike up again. Another long night awaits us here in the Harrison house.

We anticipate a return call from the transplant team in Boston tomorrow sometime to discuss planning. On first thought last week, they believed it best to do another inpatient transfer to get Keegan to Boston. This would mean admitting him to the hospital here, a medical air transport up there, and staying inpatient there at least for the first week. The plan was to try to do it before the next fever spike. That's a lot of planning in a short time. We will see what happens tomorrow and will update then. Thank you so much for every prayer and well wish for Keegan and our family.

Limbo

2011-08-17T23:05:00.002-05:00

A state of uncertainty.

I'm not exactly sure what I should or can update about at this point. Nothing is really certain. We don't know anything new. The fact that we don't have a single doctor/specialty that is the "owner" of Keegan's issues has become more and more clear every single day. So, let's start at the beginning of last week, if no other reason than to give us somewhere to start.

Fevers - Still happening on a weekly basis. Still seeming to get harder on him, and all of us, each week. We don't have a single doctor who seems to want to help us figure out why. We don't even have a single doctor we can call each week to make decisions about how to treat the fevers. When it hits, I have to call at least two different services, each who inevitably asks "well, what did so-and-so say? did he/she want labs/admit him/etc?" Grr. We still have IV tylenol at home, which is the only thing we can do for him here. IV tylenol is so new on the market that half the hospital doesn't even realize it's available. And it's so strong that we have kids coming out of open heart surgery only on it for pain management. So the fact that we have to give him 4-6 doses of it every week to keep him somewhat stable through these fevers terrifies me. And it's not even really helping him be comfortable through the fevers anymore or keeping them away longer than a few hours at a time.

GI - A little background. Keegan is pretty much completely TPN (IV nutrition) dependent at this time. It runs 16 hours per day. He gets a minimal amount of very low calorie formula through his g-tube 20 hours per day to keep his stomach and GI system "alive" and hopefully "learning" how to process something or anything at this point. Back in early April, a colonoscopy showed what looked like graft-vs-host disease or autoimmune enteropathy. Whatever it was, we knew the best way to stop it was a high-dose steroid pulse over 4 weeks, with a 6-week wean after that. Unfortunately, Keegan started having having a fever every single week. Steroids help stop inflammatory responses because they are an immunosuppressant. The flip-side of that is they will fuel any infection where you need your immune system to respond. So, we put off the steroid pulse for a month while we ruled out an infectious cause for the fevers and dealt with a port that was in Keegan's subclavian artery.

When the steroid pulse was over a few weeks ago, Keegan underwent a repeat colonoscopy to see if the pulse had helped at all. His symptoms did not change; arguably, they got worse. He still has mucousy diarrhea. Only now, he cannot pass it without the help of a stimulant. However, the amount of apoptotic cells in his intestinal tissue was vastly reduced. That means that the steroids did what they were supposed to do. Either it wasn't strong enough to allow his symptoms to improve, or his diarrhea is indicative of a separate problem. Yet, we don't know what to do about either of those situations. We know he can't tolerate the steroids for a longer amount of time or a stronger dose. Too many side effects - from wound healing to growth inhibition to fluid retention. And the other possibility? Well, it's pretty difficult to treat what you don't know.

Basically the gastroenterologist said he was out of ideas. Maybe we should just let Keegan keep having diarrhea, reduce the TPN to a more manageable amount, and let him start eating solid foods. We know that the last two steps would be a better quality of life for Keegan than the current sneaking around and keeping food from him with him dragging IV pumps around in a rolling backpack all day. And truthfully, the diarrhea does not seem to bother him that much. The sheer volume is difficult for us to keep up with on the days we give him medications to get it out, but the other days, his belly becomes very distended. It looks horrible, but it doesn't seem to affect his disposition or cause him pain.

However, Gray and I are not quite ready to accept that situation. We still believe we can seek out help from other programs to help figure out how to treat him and help him improve. Constant diarrhea for life puts school and potty-training and a million other things on hold. Despite the fact that it has dictated our entire family's lifestyles and habits. And giving him food is not as easy as it sounds. What exactly should I feed him? What is likely to be easiest or hardest on his system? The doctor says to ask the feeding therapist; the feeding therapist says to ask the doctor. Adults can make a decision to eat a certain food, knowing that it might or will cause them intestinal pain later. But we, as Keegan's parents, have to make that decision for him. How much are we willing to put him through? Not to mention that at the developmental level of a 2-year-old, he does not understand why he could eat a particular thing one day and not the the other. He doesn't understand that what he eats is what comes out. If ultimately nothing helps, then we will deal with that then. We are just not ready to accept this as is quite yet. All that being said, GI is arguably the lowest problem on Keegan's totem pole.

Immunology - To be fair, we have not had an opportunity to speak directly to the doctor since the first round of labs came back. They took 18ml of blood from him last week and 28ml this week for all the different labs. The initial impression I got from the nurse today was that the doctor didn't find a smoking gun in any of that so far. The immunologist spent a lot of time catching up on Keegan's last two years with us last week. She was very thorough and supportive. It appears Keegan does have some antibody-production fueled issues, but nothing that fits a cut and dry pattern with a defined diagnosis. The doctor had a few "outside the box" ideas for drug treatment though, even if she can't figure Keegan out completely. Of course, that was tempered by a "after I talk with his other doctors". Yeah, good luck with that. We should talk to her sometime tomorrow. Her final advice to us last week though was that seeking out a second opinion with a fresh set of eyes is always a good idea.

Heart - Well, we haven't had an echo in a few weeks, and no appointment to do another one. I'm not sure if the team is waiting on the head of the heart transplant program to come back in town to make these decisions or not. In fact the day Keegan was discharged two weeks ago from the third fever post-steroids, we were told that they didn't need to see us in clinic until October. Yeah. October. Despite weekly high fevers. Despite being TPN dependent and having a two central IV lines (port and PICC). Despite his freakishly high blood pressure. Despite the antibodies circulating in his blood against his heart and diastolic dysfunction (which the immunologist was highly concerned about). Despite his kidney issues, unusual immunosuppression regimen, blood issues, and fluid retention. I apologize to all the nurses and staff in the transplant center that we love and respect. We truly feel that Keegan has been let down from a heart transplant perspective. We can and probably will be going elsewhere for this very soon. The problem is what happens when we come back. I hate even putting all this into words. It literally breaks my heart in two. Without this transplant program, Keegan would not be alive. We have done everything we feel possible to give back and be supportive. This hurts more than absolutely anything else in Keegan's entire picture. Simply crushing. I am not an overly emotional person, but this has brought me to tears way more than I would like to admit lately. I have no idea what to do or think about it anymore. This is, in our opinion, the most important part of Keegan's health and life and arguably the most life-threatening. This is where we need to feel most comfortable.

Sigh.

That's a very, very brief overview of all that we've been dealing with the last few weeks. We wanted to make sure we gave the immunologist a chance to weigh in before we started the second-opinion process. Today, we went ahead with the first round of calls and messages to Boston. We just can't wait any longer. Keegan can't wait any longer. We feel like we are caught in limbo with all this. So much uncertainty in this entire picture. There was a time we would have left town at the drop of a hat to seek out answers for our son. We will still go to the ends of the world to help him, but leaving is a whole different beast now that our family has grown. Nor do we want to question whether what someone somewhere else does could have been done here. But the daily level of angst and uncertainty involved in Keegan's care here at home has literally worn us into the ground. As we try to make plans and figure it out, we are deeply appreciative and humbled by every prayer for Keegan's health and stability and for our peace as we important decisions that will affect our family for a long time. Thank you doesn't even begin to describe it.