More of the same

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

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

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

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

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

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

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

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

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

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

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.