How is Evan?
The place for regular updates about Baby Evan

It’s been quite a while since our last posting.  Recently each day seems very similar to the one before.  Evan has been making very small improvements that we wanted to update you on.

Evan’s eyes have opened up more often and for longer periods of time throughout the day.  We brought in his mobile from home and attached it to the hospital crib. We can now clearly see him focusing on the mobile and other points of interest such as the TV, blowing window blinds and ceiling lights when riding in the wagon. Although he will truly keep his focus he rarely turns his head on his own to follow anything and he is rarely tracking moving objects, as much as we keep hoping he does!  Evan’s arching has gotten slightly better and he is not as agitated as he once was.  Dena still comes by a couple times a week to stretch and soothe Evan, which usually leaves him loose as a wet noodle when she leaves (thanks Dena!)
Evan has gotten into a routine at night of going to sleep for his “long” stretch at around 7pm. By “long” we mean 5 – 7 hours, so Evan is usually awake at 1 – 2am.  We have not been staying with Evan at the hospital after he falls asleep, which means that he is on his own (w/nurses) in the early morning hours.  Our house phone has the nurses station on speed dial so we can call before we turn in for the night and first thing when we wake up in the morning to check in.
A few weeks ago we got the doctor approval to take Evan outside. After months of being cooped up, Evan really enjoys the daily field trip downstairs to the third floor Zen Garden.  It is a calm place to get some fresh air and sunlight, has a soothing fountain, potted trees (and is non-smoking!).  We put Evan in his wagon and take the elevator down to the patio which is often completely empty. The 3rd floor is home to the cardiac intensive care unit which we need to pass through to reach the patio. Ironically Evan in his little red wagon actually brings a smile to the usually grim faces hanging around the CICU.

In our last post we mentioned the possibility of a surgical solution to ease some of Evan’s discomfort. Team Evan has been tirelessly working on this ever since. So here’s the deal… it turns out that baclofen, the drug which holds the most promise for counteracting the mixed brain signals causing the contractions and dystonia, is not easily absorbed via the bloodstream. The current baclofen dose is administered via Evan’s feeding tube which he then absorbs through the digestive tract into his blood stream. Problem is, most of the drug never reaches the intended destination in the brain. The other downside is the nasty side effects (which we have no idea if he is experiencing) including nausea, dizziness, confusion, headaches and sleepiness.  Couple those side effects with the constant physical pain and discomfort he feels from the body and joint issues, and it’s not a pretty picture.
Now in bigger kids (and adults) with severe spasticity they would implant a baclofen pump with a three month supply inside the abdomen and run an angle hair pasta sized catheter straight to the spinal column. Then with a constant tiny drip into the spinal fluid (which circulates into the brain) many kids experience positive results. The other plus is with this direct method they can dramatically cut the dose which also reduces or eliminates the side effects. Over the past few years doctors have been experimenting with the off label use of the these pumps.  In Evan’s case, his spasticity and dystonia are a top down issue. Based on recent success at Children’s Hospital of Pittsburgh Evan’s neurosurgeon proposed to put in a pump, however, instead of to the spine, he will run the catheter straight to the third ventricle in Evan’s brain.
Over the past month we have been working with the insurance company to gain approval for the intraventricular backlofen pump.  After many (many!) “conversations” between us, his doctors and the insurance company, they finally approved the procedure. With our luck the approval came about a week ago and two days before the neurosurgeon left for a medical conference in Greece.  (I know, go figure!)  The surgeon is back in town and we are scheduled to have the surgery tomorrow 5/28 at 1PM. We’ll make sure to post a brief update when he comes out of recovery tomorrow evening.