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.
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"!