Isabelle is Going to the Hospital
My wife and I just returned from seeing Isabelle’s pediatric neurologist.
First of all, the enormity of that sentence just hit me. Nobody takes their kid to see a pediatric neurologist when everything is going well. You don’t just wake up one morning and say to your spouse, “hey, let’s take the kid in to see the pediatric neurologist to show him how awesome our kid’s noggin’ is!”
Isabelle, who turns one year old this month, has been on a course of medication for her seizures for several weeks. A week ago the doctor added an additional medication. Then a couple of days ago he upped the dosage. Though Isabelle initially responded to the medication (her seizures went from around 80 per day to 20 per day), the fact is the seizures have continued. The thing about infantile spasms is that they have to stop completely or they will continue to do long term damage to the child’s brain. Since they haven’t stopped completely, we had to see the doctor again.
It started this morning with Isabelle getting another EEG. The bad news is that it was very difficult for them to get a good reading because Isabelle was squirming so much. The good news is that they had a hard time getting a good reading because Isabelle was squirming so much. Before the first drug Isabelle was essentially in a state of what I can only describe as an “awake coma.” She didn’t make eye contact, she didn’t make noise, she didn’t move, she just sat and stared at nothing. Once we started the first medication she became more alert, started rolling around in her crib at night while she slept, making noises when she wanted to be picked up, and even made occasional eye contact. So the positive side to all of this is that the original medication seems to have started her in the right direction, but unfortunately, the addition of the second protocol did not have any effect.
Today the doctor told us that he was bringing in a second pediatric neurologist to consult with him on Isabelle’s seizures. Though she has all of the classic signs of infantile spasms, her EEG does not seem to match what they look for. But with that said, he told us that pediatric neurologists will get together at conferences and essentially fight about what to look for when diagnosing infantile spasms. I guess that comes with the territory when your seizure disorder is this rare.
So tomorrow it looks like Isabelle will be hospitalized. The main reason is so they can give her a longer EEG and monitor her brainwave patterns and hopefully catch several of the seizures on it so they can better see what they are dealing with. If it turns out after looking at the longer EEG and consulting with another expert that they are confident Isabelle has infantile spasms, then she will immediately start on a new medication. This is the one I was telling you we hoped to avoid. It has some nasty side effects including a suppressed immune system; a simple case of the sniffles can put her in the hospital while on it. Plus it tends to really drive up the blood pressure, so she would be on blood pressure medication to control it. There are also a host of other potential side effects like extreme bloating, etc. that I won’t go into detail about yet. Worst of all, she would need to be on the medication for at least 10 weeks, administered by injection twice a day. She would be home with us for all but the first few days, but we would be the ones stabbing her twice a day. No parent wants to do that.
And a little about this particular medication, ACTH. It ain’t cheap. Turns out if Isabelle goes down this course of treatment (which has about a 90 percent success rate with infantile spasms) it will cost upward s of $100,000. (ONE HUNDRED THOUSAND DOLLARS!!!) I asked the doctor why it was so expensive thinking it must be difficult to manufacture. He told me through clenched teeth, “we can thank the drug manufacturer for that, which I see as nothing short of criminal.” Turns out a couple of years ago, the company who makes this drug came to the conclusion that they were the only ones and could charge whatever they want for it. Used to be that the treatment cost $10,000. But a couple of years ago when a new president took over the company they increased the cost to $100,000. You can do that when you’re the only game in town apparently. I have never heard a doctor so angry before as when I heard Isabelle’s doctor talking about this drug company. It’s apparently the one thing that really knocks him off his normally quiet, calm bedside manner.
But…
If it turns out after the overnight hospitalization they decide that it is likely NOT infantile spasms, then they would approach Isabelle as if she has a different seizure disorder. If that turns out to be the case, they would try a different medication with more benign side effects. But the course of treatment would be for several years rather than weeks. And if it turns out after years of this other treatment that it is infantile spasms after all, then we would have lost valuable time to save Isabelle’s development and prevent permanent brain damage just to go on the ACTH anyway. I asked the doctor, “I know it is what it is, but if you were me which would you be hoping for… infantile spasms or a different seizure disorder?” He told me that one is a relatively short, horrible treatment… the other is a long, not so horrible treatment that might not be effective. That, I decided is the best definition I’ve ever seen for “rock and a hard place.”
I share all of this because I know many of you care about Isabelle and have been praying for her during these first two drugs. As we move on to the EEG and inevitably one of the drugs tomorrow, we just ask you keep our family in your prayers; especially Isabelle’s big sister Tessie. As we were explaining that Isabelle would likely be in the hospital she said “but they can’t take her! She’s my baby!” While my wife was pregnant with Isabelle, Tessie had a lot of plans for her little sister. We had gotten past the initial shock and learning curve that Down Syndrome brought when Isabelle was born. In fact, the Down Syndrome is no big deal anymore when faced with these seizures. But Tessie needs a supernatural boost to get her through this. We’d also ask for you to pray that the doctors are guided to make the right diagnosis of Isabelle so we can start the right treatment plan and make these dang seizures go away. And finally, we’d love prayers for strength for my wife and me so we can stay positive during this unsure time.
As always, thanks. And I’ll keep you updated as soon as we know anything else.
R
