Tuesday, January 31, 2012

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.


Monday, January 30, 2012

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

Sunday, January 29, 2012

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.

Monday, January 23, 2012

Deep breath

I think we are ready to leave for Cincinnati tomorrow.  

I hope we are ready.

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

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


Thursday, January 19, 2012

Kidneys, colds, and catching up

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

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

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

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

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

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

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

Wednesday, January 11, 2012

Lifting my hands

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

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

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

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

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

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

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

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

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

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


"I Lift My Hands" - Chris Tomlin

Be still.  There is a healer.
His love is deeper than the sea.
His mercy, it is unfailing.
His arms, a fortress for the weak.

Let faith arise.  Let faith arise.

I lift my hands to believe again.
You are my refuge.  You are my strength.
As I pour out my heart, these things I remember.
You are faithful, God, forever.

Be still.  There is a river.
That flows from Calvary's tree.
A fountain for the thirsty.
Pure grace that washes over me.

Let faith arise.  Let faith arise.

I lift my hands to believe again.
You are my refuge.  You are my strength.
As I pour out my heart, these things I remember.
You are faithful, God, forever.

Monday, January 9, 2012

Winter swim break

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

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











Monday, January 2, 2012

Well, it's a new year

Not really too excited about it.

I'm absolutely ready to give 2011 the boot.

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

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

And yet, I find myself in perpetual denial.

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

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

Until then, thank you for your patience and prayers.

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