Miss A and the Seven Doctors


Once upon a time, there was a little girl who, every three weeks, for three days in a row, went to the infusion clinic at the hospital for IV immunoglobulin treatments, IVIG for short. She arrived with her Mom (and home nurse) at 8:30 am and they would not leave until 2:30 pm...if they were lucky.

Fun fact, these time-consuming treatments are NOT to treat her epilepsy. Like so many other kiddos with seizures, epilepsy is not Miss A's only diagnosis. So, this week we sat in the pediatric infusion room treating two of Adelaide's other symptoms/diagnosis: Mast Cell Activation Syndrome and Dysautonomia. Two more disorders that doctors understand little about and, of course, have no cure. It is most likely that all of Adelaide's ailments are caused by a genetic problem that as of yet has not been identified, but that's a whole post in and of itself. What I'm getting at, (focus, Kelly, focus!) is that many pediatric epilepsy patients are often battling more than just seizures and you never know when the next poisoned apple will arrive at your doorstep.

Adelaide has a team of seven doctors that work together to treat her regularly and three additional specialists that we see a couple times a year. Which makes sense if you think about it: the problem, whatever it is, is originating in her brain -> the brain controls the rest of the body -> so if there is a problem in the brain its likely affecting other systems. Prior to Adelaide's first seizure, she was already missing milestones. She had been diagnosed with hypotonia (low muscle tone) and we were laser-focused on trying to figure out the cause and to treat her. So, when Adelaide was diagnosed with epilepsy it felt like a distraction. If we could just control the seizures then we could get back to focusing on her hypotonia, then she could start meeting those physical milestones with which I was so obsessed. It did not take long to realize that the physical delays paled in importance to Adelaide's cognition which was devastated overnight when the infantile spasm seizures began two months later.

But just focusing on seizures would be too easy! When it comes to managing a medically complex illness, or trying to anyway, we've learned to be on the lookout for the next unwelcome visitor. In February we were fully focused on adjusting meds to control Adelaide's seizures when she was rushed to the hospital because her heart nearly stopped along with her breathing. This was the second time she was resuscitated in four months and we still don't know exactly what happened. It was terrifying to say the least, and suddenly every doctor appointment was spent focusing on how to prevent this from happening again. We THINK the life-threatening events were related to her dysautonomia so we began the IVIG treatments and, thankfully, have avoided trauma centers since. Juuuust when we've thought we had a treatment plan or had a symptom in check something else pops up and makes itself an issue. So, we remain flexible, focus on inch-stones not milestones and take life a day at a time. If that gets too hard then focusing on the moment at hand is ok too. We may not have a magic mirror that can give us the cure for Miss A but we do have seven doctors and are armed with bushels of  love to power through. 

Side note: While, Adelaide's other diagnosis can be life-threatening, we are fortunate that we have been able to find treatments that improve her symptoms but that is not the case for all those afflicted. To learn more about mast cell disorders, The Mastocytosis Society is an excellent source; as is Dysautonomia International for dysautonomic disorders.