Monday, June 10, 2013

What to say about Keegan?

I've been sitting here trying to start this post for the last 5 minutes, but I am really struggling how to explain or make sense of what's going on with Keegan.  It's likely because no one really seems to know exactly what is going on with Keegan.

Most recently, we spent all weekend waiting on him to spike a full-blown fever.  Fevers are tricky for Keegan.  They could indicate a normal virus (which could result in a MAS flare), a flare just in response to his immune system attacking itself, or a central line infection.  Because he has a central IV line, protocol is usually to admit him for 48 hours of IV antibiotics and culture the line when his temperature goes above 100.5 degrees.  On Friday, he got to 100.4, but by the time we contacted the team and had a plan, he had come back down into the 99s without intervention.  We had already drawn labs that morning, so the doctors agreed to let us keep him home until the fever came back up, as we all assumed it was just the beginning.  Keegan was tired, had horrible coloring, and was not acting like himself, but other than that (and some tummy issues I will address in a minute), he showed no other signs of needing to be inpatient.

So, we waited.  And waited.  And waited.  We took his temperature every 30 minutes.  Every night, we prepared for needing to run him to the ER in the middle of the night.  He "simmered" in the upper 98s and 99s all weekend, but he never hit that magic number.  By last night, he still looked terrible, but he otherwise seemed to feel pretty well.  What was it?  Not a line infection, thankfully.  It wouldn't have gone away on it's own if it was a line infection.  It could have been a virus that the anakinra helped keep from becoming a full flare, although I find that unlikely since no one else in the family was sick.  Or it could be a small flare up of his underlying immune disorder that may happen more often as we continue to wean his steroids.  This may just be the beginning of something bigger, or maybe it will be a one-time weekend full of anxiety that never reveals anything more.  As agonizing as it was, let's hope that will be the case.

He didn't want to eat much this weekend, but a lemonade sounded good yesterday.
Safety during your lemonade run is of utmost importance.
Hard hats required.

In the last few weeks, Keegan has been sliding back into some of his old disease patterns, which has been obviously difficult for us to process.  We have been unable to raise his magnesium levels at all, but we are continuing with the IV mag infusions anyway.  Last week, we were allowed to start administering them at home.  Now in addition to his two IV medications per day, he gets TPN three nights per week and mag infusions twice per week.  Maybe in time, Keegan will actually benefit from all of this work.

When we came back from Boston with our new MAS/as-yet-unnamed-autoimmune-disorder diagnosis and on the IV drug anakinra, the rheumatologist Keegan sees here at Scottish Rite (who actually developed the drug) warned us that there is always something that your main drug won't fix.  For some kids, it may be a rash or joint pain.  For Keegan, she suspected it would be the attack on his GI system. We know that on higher levels of steroids, we can quiet the attack on his intestines and allow him to function somewhat normally.  On the biggest doses, he could eat and process food fairly normally.  Around the beginning of the year, we reached a point in the wean where although he was eating, his intestines couldn't properly obtain nutrients from his food, and we had to start back on IV nutrition (TPN).  The more steroids we have weaned, the more TPN and electrolyte management he has needed.

However, we hadn't seen a huge change in his output.  Until now.  It may be TMI, but if you've been reading about Keegan for any amount of time, you should be used to it.  About three weeks ago, we took another milligram of steroids off his daily dose, and over the last three weeks, his diarrhea has returned.  As the rheumatologist predicted, we believe this is just his underlying disease without steroid management (or as we slowly wean him off completely).  We were warned this would happen, but it is still a blow to see it come back.  The anakinra keeps him from massive flares that could kill him, and that is most important.  He needs to get off the steroids in order to grow and stop the atrophy we have seen in his brain over the last few years.  Those things are more important than a bad tummy that can be managed with diapers and TPN.  It's not life-threatening.  It's just disheartening.

I kept putting off this update because I kept hoping that the answers from his labs and from the doctors would be different.  I apologize if little of the preceding information made sense to most people.  I wish there was a concise answer, but there's just not.  Keegan will always keep challenging us, and this disease process will always keep evolving and keep us guessing.  This certainly isn't the end of the story.  I have a gut feeling things may start to reveal themselves a bit more clearly in the next few weeks.  We will just have to keep praying for patience as we wait.  Thank you so much for continuing to support us through it.  There is just no way we could continue to travel this road without your prayers and love.  More soon.


Sarah Kay Hoffman said...

I feel for you! I am praying for you, your son and your family.....all my love!

Jennifer said...

So sorry to hear of the setback. Like you said, not life threatening, just disheartening. I know you guys have had enough disheartening news to last a lifetime. Hugs and prayers, always!

Greg said...

Sorry to hear - complicated kids are so frustrating.

We have one with an immune deficiency, and it's really difficult to get your head around the concept that a bunch of MD/PhD's from the best schools, sitting at a great academic center, don't have the answers.

At least if you get a bunch of law professors in a room thinking about a problem, they'll all tell you that they know *the* answer (even if they're all wrong).

Good luck...


Julie said...

Praying for you!