Treatment for Infantile Spasms

When Baby J. was diagnosed with Infantile Spasms a few weeks ago, the doctors were adamant that treatment begin immediately. We knew a little about the drug ACTH because we had read about it before even bringing J. to the hospital, but we didn’t know much about the supporting drugs she would need to be on, as well.

I hated that we had to start her on treatment so quickly because I didn’t have time to research our options, or the side effects of the drugs. I still haven’t really researched alternative treatments, mostly because I’m afraid that I’ll find something that indicates we should never have given such a powerful steroid to our daughter because there are less dangerous treatment options out there. We’re already halfway through her course of ACTH, so why make ourselves feel bad about it, now?

On the other hand, however, I believe in trusting your doctors (until you have a real reason not to) and so far J. has done very well on her medications and they seem to be working (our next EEG will tell us how well).

So far, J. has been placed on four different medications and is also undergoing therapy for her delays. This is what our schedule for the last two weeks has looked like:

7/15	Admitted to the hospital; EEG	7/30	E.I. Physical Therapist assessment
7/16	Diagnosed with IS; began meds	8/1	Follow-up with the pediatrician
7/18	Removed EEG leads; MRI	8/2	E.I. Vision Therapist assessment
7/22	Meds arrived; discharged	8/3	E.I. Developmental Specialist assessment
7/23	Visiting Nurse (introduction)	8/4	Visiting Nurse (weight, blood sugar, BP)
7/26	Early Intervention assessment	8/4	E.I. Occupational Therapist assessment
7/27	Visiting Nurse (weight, blood sugar, BP)	8/8	E.I. Speech/Feeding Specialist assessment

As you can see, post-diagnosis is a very busy time. It’s good, though. Each appointment gives us something to look forward to and it makes us feel better to have all of these professionals checking in on J.

The two branches of treatment J. is receiving at this time are medication and therapy.

I mentioned above that J. is currently on 4 different medications. They are: ACTH (Acthar), leucovorin (folinic acid), B-6, and ranitidine (Zantac).

ACTH

The worst, by far, is the ACTH. The other three drugs are prescribed to support a baby’s health while they are on ACTH. (The Wikipedia page is very informational.)

ACTH stands for Adrenocorticotropic hormone. It’s a corticosteroid this is used to treat, in addition to Infantile Spasms, pituitary disorders, Addison’s, Cushing’s, and some kinds of cancer.

The side effects can be severe. The top three things to be careful of are high blood pressure, high blood sugar levels, and infection. Our visiting nurse comes twice a week (unless we are seeing a doctor) to monitor J.’s blood pressure and blood sugar.

ACTH suppresses the immune system is why infection is major concern. It’s easily the scariest side effect, in my opinion. We decided (and our neurologist agreed it was wise) to pull J. out of daycare for the duration of her treatment. We haven’t decided yet if we’ll put her back in when she is done with the ACTH, but we felt that it was important to keep her away from other children. Especially because she also cannot receive her vaccinations until her treatment is over. Since she was diagnosed just after she turned 6 months, the last series of vaccines she received were her 4 month shots, so, now 7 months, she is quite behind.

ACTH is also very difficult to get. As we were told, ACTH is made by one company and distributed by one pharmacy in this country. We ended up having to have it overnighted to our house, and were then required to bring it in to the hospital before we could be discharged. The whole process took about five days. If you are unfortunate enough to not have insurance at the time your child needs this medication, be sure to inquire about special programs the manufacturer may have to help you receive the drug for free. ACTH is prohibitively expensive without the help of insurance (we’re talking in the range of $30k-$50k per vial--we were prescribed 2 vials). We were also told that insurance companies will sometimes refuse to cover ACTH due to exorbitant cost, but those situations are rare. We haven’t received our bill yet, but our prescription was covered. Most insurances will comply because ACTH is virtually the only drug used to treat Infantile Spasms (unless the underlying cause is discovered, for instance, if your child has Tuberous Sclerosis, vigabatrin (Sabril) may be used, instead).

Prepare yourself for learning how to administer ACTH to your child at home, it’s given by injection. Your nurses will teach you to give the shot and when you receive the drug, you’ll also receive syringes, needles and sharps container. Ask your nurse to find out what to do with the sharps container once it’s full.

We are on a taper schedule with ACTH. The first week we were giving her 32 units (.4ml) twice daily. The second week was 32 units once daily, third week 32 units once every other day and fourth week is 16 units (.2ml) every other day. We will be on the fourth week when we return for the second EEG, so we are hopeful that J. can come off the ACTH after that week, but they told us that she may be on it for 6-12 weeks.

Leucovorin

When you Google leucovorin, don’t panic when you see it listed as a drug used to support patients going through chemotherapy. During ACTH treatment, leucovorin is used to protect intestinal tract from a GI bleed.

This comes in pill form, which we crush up with the B-6, mix with pureed prunes and spoon feed.

Leucovorin is 30mg once a day.

B-6

B-6 is prescribed for Infantile Spasms because, in some cases, seizures can be caused by a B-6 deficiency. Our doctors told us that sometimes they will administer B-6 first to see if it has an ameliorating effect on the spasms before going to ACTH, but in our case, they prescribed it to J. in addition to the other drugs.

This is also in pill form, which we give by crushing and adding to prunes.

In the hospital we tried mixing both the Leucovorin and the B-6 with apple juice and giving that by syringe (it would clog a bottle nipple) but J. really hates being force-fed with a syringe, and sometimes that would get clogged, too. We’ve had far better luck giving it to her mixed with prunes, which she already loves, and I add just a touch of pure maple syrup to cut any bitterness from the pills.

B-6 is 300mg once a day.

Ranitidine

Ranitidine (Zantac) is a drug used to treat reflux. This is also to protect J.’s digestive tract. GI bleed is another side effect of the ACTH to look out for, but the leucovorin and ranitidine are employed to combat that risk.

It comes in a minty syrup which we administer with a syringe.

Ranitidine is 3ml once a day.

Therapy

J’s therapy has not yet started. A diagnosis of Infantile Spasms makes J. automatically eligible for Early Intervention services in New York, but she is currently being evaluated for which kinds of therapy she’ll need. This whole process (unfortunately) takes upwards of 30 days. Then we’ll be able to review the report the therapists have written, and approve it, or ask for a second assessment. Once the report is submitted, we’ll have a meeting with our EI coordinator who will go over the whole thing with us.

Once J. is assigned therapists and seeing them regularly, I’ll post more about this part of treatment.