Next Step: ACTH

June had an EEG on Monday morning, and it showed no real change since we started the diet.  As you can imagine, Matt and I are disappointed.  We knew her EEG wouldn’t be normal because we have seen her continue to have spasms, but we’ve seen such a dramatic improvement in June’s awareness and temperament that we believed we would see some corresponding progress on her EEG.

However, I realized the moment the technician turned on the EEG machine that her EEG had unfortunately not improved.  I understand it takes years of medical school and training to learn how to read an EEG, but as soon as I saw the large spikes, and disorganization on the screen, I knew June’s brain was still in a state of chaos. Later that night, her doctors called and told us what we already suspected, that June is still having spasms and the diet is not controlling them. After the doctors went through our options, Matt and I decided to start weaning June off the diet so she can start ACTH, which is the frontline medication to treat infantile spasms. You may wonder if ACTH is the frontline medication for IS, why didn’t we start June on it in the first place?  I ask myself that question almost everyday, and it is one that haunts me in the middle of the night. The first reason is because ACTH has dangerous side effects and risks, the main one being a compromised immune system. While on ACTH, a common cold could send June to the ICU. Second, June was only 2 months old when she was diagnosed – most babies are diagnosed around 6 months – and the doctor we had at the time advised us that it was too risky for a baby her age. He told us that we could do a daily hormone injection, which is dangerous with lots of potential side effects, or an oral steroid, which is much safer and does the exact same thing as ACTH. We, of course, chose the safer drug to start. We now know that statement is not entirely true. Studies show that prendisolone is comparable to ACTH, but there are babies who do not respond to prendisolone that go on to try ACTH with success. Additionally, at that time of diagnosis, we didn’t realize how serious IS was for June. In fact, I didn’t even know June had IS until we saw a different doctor for a second opinion. Our first doctor kept referring to them as “spells” and didn’t do a good job of explaining the condition to us. In fact, I remember saying to him, “Oh, June has seizures?  No big deal. The Supreme Court Justice Roberts has epilepsy and look at all he’s done.”

If our first doctor had explained June’s condition to us the way her current doctor has, I think we would have done ACTH to start.  Once we switched doctors and realized the oral steroid did not work to stop June’s spasms, we thought we needed to try a different kind of drug because it was important to get the spasms to stop immediately. We didn’t want to waste time trying another drug in the same family as a drug that didn’t work. That’s why we tried Vigabatrin next.  By the time we realized Vigabtrin wasn’t working, flu/cold season was upon us and we were scared to try ACTH at that point because it seemed too risky, so we decided to give the diet a try until after flu season.

As I write this I can almost hear Matt saying to me, “Genny, you don’t need to defend yourself.  We did the right thing based on what we knew at the time.”  Logically I know he is right, but I feel guilty.  I feel like I let June down because here she is eight months later still having spasms. Before having a baby, I heard sayings like, “parenting doesn’t come with a list of instructions“ or “you will make mistakes. It’s a given.”  But I thought the types of mistakes we would make would be closer to which grade school June attended or if we challenged her enough. I wasn’t expecting to make major life or death medical treatment decisions. I thought if a bad situation arose, the doctors would tell us what to do because I thought doctors knew everything when it came to medicine, at least any of the medical conditions I thought June would face. I can’t tell you how frustrating it is to sit in a room with really smart doctors, some of the best in the country – asking them which treatment we should use on June and they tell me they don’t know. I might as well live in an era before penicillin because that is how crazy it feels. They tell me what they know, which with infantile spasms is very little, and then Matt and I are supposed to make a decision.  I guess this is what everyone meant when they said there is no instruction book for parents. Gosh, I wish there was. Not just because I love following directions – it brings me comfort to have step-by-step lists of what to do in all new situations, but because I would feel less guilty about making the wrong choices.

As I mentioned, before we start ACTH we must wean June off the diet. It will take 3 weeks to wean, and then we will start ACTH as soon as the doctors say it is safe. I’m scared about the wean because I don’t want to lose all the progress we’ve made with the diet.  But the doctors say if ACTH works, it will give June a much better chance at developing. Once June is on ACTH, we need to be very careful since her immune system is suppressed. We won't take her anywhere and we will have to limit her visitors. I'm told we should be able to tell if ACTH is working about 2-3 weeks after we start, but then it takes about a month to wean her off the steroid. The doctors say June's immune system will be compromised about 2-3 months after she is off the drug, so we will keep June isolated for about 3-4 months at minimum. If it is working it will be even more extensive because she will be on the drug for a longer period.

This was a difficult decision for Matt and me to make, but we decided we had to try it.  If June had cancer, we wouldn't hesitate about doing chemotherapy even with all the nasty side effects. Besides having a suppressed immune system, June will likely be very irritable and uncomfortable on ACTH.  Another mother told me that the hardest thing was not seeing her baby smile while on it.  If that is true, that is going to be so difficult for us, but I know she would want us to do this. She would want us to do everything we can to help her. I can see that in her eyes as her big personality is fighting to come out of her little body. I am sure it will be harder on me to watch her uncomfortable than it will be on her.  As I explained this decision to my friend Emily she said, “sometimes we have to be bold to make any real change.” What she said made me think of the quote, “Be bold and mighty forces will come to your aid.” I have to trust that we are doing the right thing for June even though this is going to be a hard and difficult few months and God will send us assistance to help get through this time. 

Thanks you all for your support. We really feel it more than you know.