Wednesday, September 28, 2011

4 weeks

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!

Monday, September 26, 2011

Ladybug Goes to School

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.

Saturday, September 24, 2011

Baby steps

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!

Thursday, September 22, 2011

On the move

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

Wednesday, September 21, 2011

Steady

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.

Tuesday, September 20, 2011

Decisions

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

Monday, September 19, 2011

Four Years

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

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

Sunday, September 18, 2011

Bleeding

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

Saturday, September 17, 2011

Back to the ICU

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.

Friday, September 16, 2011

Biopsy and steroids

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.

Thursday, September 15, 2011

Tonight

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

Wednesday, September 14, 2011

Prayers

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.

Tuesday, September 13, 2011

Busy day

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.

Monday, September 12, 2011

Keegan is 4!

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.


Sunday, September 11, 2011

So much for hoping

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.

Saturday, September 10, 2011

Day five

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.

Friday, September 9, 2011

Ideas

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.

Thursday, September 8, 2011

#13

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, September 6, 2011

Tuesday

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!

Sunday, September 4, 2011

Bye-bye PICC

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!

Friday, September 2, 2011

Happy Friday

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!?!?