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.
