Okay, so some may have already read, or maybe not since i'm new on here, but my daugher was diagnosed a week ago with Tuberous Sclerosis and Infantile Spasms. Interestingly enough though she is stumping the doctors because she is responding so well to the drugs.
They started her on phenobarbital alone 2 weeks ago before they got the MRI results and knew for certain it was IS (because of the TSC). They say she for sure has IS b/c of all the tumors in her brain from the TSC, yet she had 2 EEGs, one of which was 24 hours, and they never saw hypsarhythmia, though her EEG was irregular. Before they saw her MRI though they put her on phenobarbital. After her first dose, she never had another seizure. Once they saw her MRI they also put her on ACTH, 40 units; .7 mL of Topamax and 110mg Vit B6, and she's on a probiotic she's been on since she was 2 months old and they said to keep her on it. She's been on the ACTH for 8 days and after 2 weeks total they'll start to wean her off of it.
She is actually doing suprisingly well. She is not overly irritable with the ACTH, in fact LESS irritable than before starting the medications (she has always been a VERY colicky baby). She did have some eating issues at first but we've now got her back to normal with eating.
I guess I'm wondering, do most people see better attitude, etc when slowly weaning off ACTH, or does mood get worse from the weaning off? Just wondering whast I need to prepare myself for.
Also, she is getting blood draws twice a week to check electrolytes and CBC. I thought it was b/c of the ACTH, but then they said no, its to check kidney funciton b/c of the Topamax because the Topamax can cause kidney stones....
My understanding is that they will probably keep her on the Topamax, B6 and Phenobarb after weaning her off the ACTH as long as they are working and she has no bad side effects from it. But does that mean that she will FOREVER need blood draws twice a week for as long as she is on Topamax? They ahve a very hard time getting blood from her, even with the best pediatric crisis nurse at the hospital. It always takes them at least twice to get the blood they need, but i feear getting her a picline or port for fear it will get infected since her immune system is down from the ACTH????
They did say that after the ACTH she wont have as many doc appointments as she is having now, (at least twice a week appointments plus daily nurse visits) but I'm just curious what i can expect as far as blood draw frequency??
Thanks for sharing what you know and your experiences... (ps my daughter is 4 months)
When Lij was on Topamax long term, they eventually spread out blood draws to every 3 or 6 months, (I don't remember which, since ther've been so many drugs. lol)
.With Topamax we did get the no sweating side effect, which mena that he couldn't control his body tempurature and could over heat very easily, so make sure to watch out for that.
Almost all seizure meds require blood draws, but usually only every few months, so things should calm down for you. If they have to continue with twice a week, maybe you could request a picc line, so they don't have to poke her every time, though that does require more home care...
As you know phenobarb isn't supposed to help IS, so we've never tried it, but quite a few of my friends have, and when their kiddos come off of it they do MUCH better, developmentally. Not so floppy and able to sit better and stand better and everything, so I kind of hope she's not on that one long term, but if it keeps the seizures away, it might be better for her than being off of it... Always such a fine line to walk between seizures and meds. ugh.
February 14, 2010 at 10:15 AM
If she can do okay just on Topamax, I'm okay with that; I'm just hoping we can get her back on phenobarb until she's off the ACTH because it makes the ACTH much more bearable. She was also very floppy when first put on phenobarb loading dose but she seemed to even out a bit after her system got used to it.
I can handle blood draws even once a month, heck even twice a month is better than twice a week. Twice a week doesnt even give her time for her bruising to go away.
However, she is doing MUCh better with blood draws from her forehead....little to no bruising, and they've gotten blood from there twice without having to restick her! YAY!
you're right, even now its so hard to tell what symptoms are from the TS and which are from the drugs.......
I see her neuro again Tuesday, so we'll see how it goes. She would be weaning off from ACTH now if it werent for having to take her temporarily off phenobarb and topamax b/c of the rash (i'm almost certain the rash was from the amoxicillin).