Paisley went to the neurologist today. Of course, that came with more bad news. We went a week earlier than our scheduled appointment because of Paisley's more severe and frequent seizures. She's on the highest dose of Topirimate (Topamax) she can be on right now and it's barely scratching those infantile spasms. Gammeltoft finally recommended ACTHar, a 4-week hormone shot therapy. Now before I tell you all about ACTHar, Gammeltoft does not really expect it to stop the spasms. Since it is a hormone steroid, she can't be on it very long. We're in trial and error and it's a good drug for kids with "more normal" brains who have the spasms, but it's worth a shot because the next drug, Vigabatrin, is harmful to eye sight.
Here's some info from the ACTHar website:
ABOUT ACTHAR: Acthar is a prescription medication containing the hormone adrenocorticotropin (“a-DRE-no-cor-tico-tro-pin”) that has been used for over 50 years to treat children with infantile spasms. Acthar may work by both helping the body produce natural hormones and by having a direct effect on the brain. It is designed to slowly release the medication into the body after it is injected.
HOW ACTHAR WORKS IN INFANTILE SPASMS:
Approved by the FDA, Acthar was proven to stop spasms and hypsarrhythmia. In one clinical study, 87% of children had no spasms and no hypsarrhythmia by week 2. Some parents reported that their children’s spasms disappeared as early as day 1 following the start of treatment. On average, children responded to treatment at day 3. Children with an underlying disease or injury to the brain also benefited from Acthar. However, every child will respond to treatment differently.
If the ACTHar shots do not help or her spasms return after the therapy is over we will most likely resort to Vigabatrin.
Gammeltoft said that the when geneticist told us that Paisley's lissencephaly was mild and that she may do better than originally thought and may have a normal life span was she was totally wrong. He got kind of offended at the optimistic prognosis and that really upset me. I feel like he takes every little bit of hope that I have away. He said she's about like a Miller-Dieker patient even though she doesn't have the rest of the deletion, and that if she never learns to roll over her lungs will give out prematurely.
I just hope this ACTHar knocks the spasms out so Paisley can go through therapy better and learn motor skills! Today was a bad day.
I just came upon your post when researching ACTH. My 5 month old has been diagnosed with infantile spasms. As of now, it's idiopathic, meaning there is no cause. I was appalled at your neurologist's response and would urge you to find another doctor who is more compassionate ... There is one out there for you! No matter what, never lose hope! miracles happen every day! God bless you and your beautiful little girl!!
ReplyDeletePaige - First of all, you are an amazing parent, so courageous and loving. I think you've got a terrific blog. I am conducting a survey about specialty drugs used to treat various neurological disorders. We are interested in speaking to parents about their experience with various medications, with an emphasis on Acthar Gel for the treatment of infantile spasms. The call takes no more than 30 minutes. If you would be open to speaking I can be reached at mrich@tecoppscapital.com Thank you. - Marian
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