One 45 minute test equals 4.5 hours in the hospital.

Ah, the electroretinography (ERG). a routine test you are supposed to have done every three months while taking Sabril (vigabatrin). You are also required to have an eye exam every three months because this particular drug carries a risk of loss of peripheral vision. Sabril has only been approved by the FDA for a few years. Before that you had to order it from Canada. But then it received approval here, the cost skyrocketed, it became heavily regulated, and it’s only available through specialty mail-order pharmacies. Many people resent being treated as if we’re too stupid to understand and take on the risks.

Given the risks, should we keep a close eye on the vision of our children? Certainly. But the intrusive nature of the ERG makes many families angry. It’s no simple test. We took Connor in for his second one yesterday (mmm hmmm, we’re behind schedule). We had to be at the hospital by 9 and get admitted through day surgery for a test that won’t start until 11. Yup, I told you it’s not a simple test. Then there is lots of information gathering, weighing, measuring, and finding a vein for the IV. Connor likes to make things difficult by hiding his veins. He always requires the IV team, rather than a regular nurse. Then we take him down to the room where they will place contacts in his eyes that test his reactions to stimuli. But he won’t be awake. He must be sedated with propofol (yup, Michael Jackson propofol). Once it’s over, we must wait for him to wake up and drink before they will pull out the IV and we are allowed to leave.

These tests and the frequency with which we are expected to undergo them frustrate a lot of people. We know there are risks to the peripheral vision. But we didn’t put our kids on this drug just for the heck of it. If someone is taking it, it’s for pretty dire reasons. The primary reasons I know of for its use are infantile spasms (why Connor is taking it) and frequent compex partial seizures that have not responded to other medications. Most people (based on my interactions on message boards) wouldn’t take their kids off this drug, even if they were told vision was being affected. Infantile spasms, left untreated, can cause major brain damage. What good is fantastic vision if your brain is fried? And the people taking it for other seizures have likely tried every cocktail in the book and are having so many seizures that their lives are being adversely affected to the extreme. So is being sedated every three months a bit much? You decide.

I’m not really freaked out by Connor being sedated, I guess because I’ve seen so much in his 10 months. I also know he’s being closely monitored. It’s not like Michael Jackson, who was abusing it with the help of a shady doctor. I just think the overregulation is an American agency assuming we are idiots. Should we keep an eye on vision? Yes. Every three months? Some people are on this drug for years. What hell. Especially if you are getting it through Accredo Pharmacy, specialty pharmacy of Medco. If so, you are probably already going through the monthly hell of securing your refill from this incompetent place.

We recently increased Connor’s dose of vigabatrin again in hopes of putting a dent in these eye rolling seizures. We are seeing some improvement. We actually had a day recently where we didn’t see any, which hadn’t happened in a while. We are also having more days in which we only see 1-2 clusters of eye rolling. But other days we see up to 4 or 5. Then again, for a while we were frequently seeing 4-6, so it’s definitely an improvement. His other seizure med is keppra. His neurologist is considering adding Onfi, and if it seems to help, we will wean him off the keppra.

Any TSC families reading this, I will be going to DC at the end of the month with volunteers from all over the country to meet with members of Congress regarding TSC research funding. I’m in need of personal stories and letters to your congress people. If you can help, e-mail me at pin.the.map@gmail.com. I can give you more details.

Thanks to everyone who has been voting for me to the right by clicking on the Top Mommy Blogs button.