Wednesday, August 17, 2011

Limbo

A state of uncertainty.

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

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

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

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

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

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

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

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

Sigh.


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

2 comments:

Cody said...

Maddie - again, thank you for sharing your heart. Unfortunately, I do understand your frustrations ... Keegan is such a treasure and his heart reaches out to so many through your voice. We continue our prayers for Bug and your family.
With love - Cody, Gary & Griff

Stacie said...

I am so very sorry that there are so many different areas of Keegan's health with such great uncertainty. I am praying for you all, for complete wisdom as you take the necessary steps to help Keegan. Thank you for the detailed update. It helps us know how best to pray.

Hang in there.

Much love,
Stacie Smith