31 Jan 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.