A Delicate Balance

Will at the Infinity Fountain outside the Clinic

We spent most of this week in the hospital with Will...again.  Initially, the reason for the admission was to evaluate the effects of the ACTH.  It seemed to be working, although there was still some seizure activity, so the plan upon admission was to monitor Will's brain wave activity and to look at increasing the dose of ACTH in an effort to achieve a normal EEG.  However, once test results came back, Will's treatment plan took a detour.

Will was admitted on Monday (4/11) for additional testing and video EEG monitoring following his neuropsychological evaluation.  During rounds the next morning, the doctors discussed the plan to increase the ACTH back to his previous dose, and in anticipation of this, Will was scheduled to have a renal ultrasound, another EKG and echocardiogram on Wednesday.  We added another specialist to our team, a nephrologist which is a specialist in kidney function, in addition to our neurologist and cardiologist.  Will's blood pressure ran dangerously high at the previous dose of ACTH, and deciding to go back to that dose was a scary proposition.  We needed to be sure that his kidneys and heart looked good, and as long as we could get everyone on board the plan was to increase the dose of ACTH with extremely careful monitoring and with the addition of medicine to control his blood pressure.  This meant that we would likely be inpatient for several more days.  Another delicate balance of risk and reward.

The renal ultrasound went well, and we got word that Will's kidney function looked great.  Next, we needed to get the tests run on his heart.  We were waiting for transport to take us down to the where the scans would take place, when we got word that those tests had been cancelled, and we were given a brochure on the next drug option, Vigabatrin.  Our hearts absolutely sunk. We felt physically ill to realize that he had failed yet another drug, especially one that we thought was working.

We were told that the data collected from the video EEG revealed a few things.  First, ACTH was not actually working, but was basically just masking his symptoms.  The activity we have been seeing was definitely seizures, and not just any type of seizures, but the dreaded infantile spasms.  Second, the focus of Will's seizures seems to be slowing.  Essentially, what this means is that a portion of the seizures seem to be originating from multiple areas of Will's brain, and not just from the left rear quadrant.  This also means that brain surgery to remove a portion of Will's brain, while it may improve the spasms, is less likely to eliminate the seizures than we had originally thought.  With infantile spasms, unless the seizures are completely eliminated, the prognosis for mental development is not very good, so the goal is total elimination and not just improvement.  Will's surgical review board is scheduled for next month, so we will know more as to the Clinic's recommendation on surgery at that time.  We are also hoping that getting the 2nd opinion in Detroit (and if necessary, a third or even fourth opinion elsewhere...) will help us to make the right decision.

We are, as always, happy to be home again.  Jack, at age 4, is struggling to deal with all of this, and we miss him terribly when we are away.  As we tucked him in to bed last week, he asked, "Mommy...what is ep-i-lep-sy?"  No matter how hard we try to protect him from all that is happening, he is more aware of everything that is going on than we think.

Tomorrow we are starting Will's first dose of Vigabatrin.  We have been uncomfortable with the idea of trying this drug because it carries with it a rather significant risk of permanent loss of peripheral vision.  The drug facts list the risk as 1 in 3, although anecdotal evidence shows the risk to be less in children.  We are running low on options, and we don't really have a choice.  At this point, Vigabatrin is less of a risk for him than ACTH, and the doctors feel that the chances of Vigabatrin working is almost equal to ACTH, and we can go to a higher dose with less risk.  For a few weeks, he will be on 4 very strong medications - ACTH, Vigabatrin, Keppra and Topamax, all of which have significant side effects.  When you combine this with the effects of the infantile spasms, Will's brain development has basically been on pause for months.  Although, we can be thankful that his brain development has not regressed very much either. 

Thankfully, we will be weaning ACTH and Topamax in about a month.  Even though we have only been dealing with immune suppression for about a month, living in isolation and not being able to go anywhere, the constant blood pressure checks, and the needle in his little thigh every morning is definitely wearing on us all physically and emotionally.   In this respect we will be glad when Will is done with ACTH.  We are also hoping that weaning some of the medications will help to get our little boy's personality back, although Will has amazed us with the way his spirit still shines through despite all of his medications and his disorder.