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

Yesterday was a hard day for the whole family and everyone who knew Evan.  Over 200 friends and family comforted Brian and Sharon and Alex both at the funeral and afterwards at their home.
Brian and Sharon and Alex are now sitting shiva at thier home in Laguna Niguel until Sunday evening.
Anyone who would like to join the family please call Evan’s uncle Adam at (949) 697-6726.
The family is requesting donations to CHOC Foundation in lieu of flowers.

Our Baby Evan died very peacefully at 9:20am today. The nurses called at 6:00 to let us know we should come in. Mom, Dad, Grandparents, Aunts and Uncles were all here holding and comforting Evan. He wasy very calm and slowly stopped breathing while in my arms. In the last hours Evan actually smiled a few times so we know he is at peace.

We continue to take shifts at the hospital in an effort to “be there” with Evan.  But in truth, we don’t really think that Evan knows we are here.  No amount of talking, singing, rocking or rubbing has much of an effect on him.  Perhaps putting in our time at the hospital allows us to grieve for the life we had hoped he would live.

Evan had some tests to determine his intolerability to feeds.  The GI doc sent a scope down his GI tract to see if we were just missing the obvious.  The results showed that the biggest problem was neurological in that Evan’s brain is not telling his body to move the nutrition from his stomach into the intestines and then out.  This is called poor motility.  Over time Evan’s stomach has started to shut down, as are his intestines, and the only way he can have a bowl movement is with the use of a sepository.  The G-tube is now going unused as Evan can no longer tolerate any feeds.  He receives nutrition via IV with TPN (mentioned in post below).  There are all kinds of long term issues associated with this process though.

The baclofen pump is doing its job.  Evan’s neurosurgeon believes that the dosing is at its peak, but modified the timing so that instead of a continuous drip the baclofen, the pump is programmed to deliver a dose six times a day (every four hours).  Other pain meds are also being used to help with Evan’s discomfort, so much so that docs put him on a fetanyl (the synthetic form of morphine) and versed continuous drip.  The combination of these drugs plus the baclofen really keep Evan calm, although he does have some daily rough patches.

Other than the obvious agitation/pain Evan has, he has also had some changes to his coloring, skin texture and respiration.  His little body is getting tired and hope wearing thin.

Too tired to write much now. Everything went well today. Other than waking up briefly when turned Evan has been in a deep sleep all day.

June 4th marks the three month mark since Evan’s accident.  Three months of a nightmare we have all lived.  It has also been one week since the brain surgery and time for the actual baclofen pump to be installed.  Surgery is scheduled for 7am, so by the time you read this, Evan will have new hardware in his body.  The dosage of the baclofen has been raised daily to now at 500 micrograms (a far cry from the 60 milligrams he was having orally).  It has not relieved all of his spasticity as he does still arch and get aggitated, but we are hopefull it will continue to get better for Evan.  The dosage can continue to be adjusted even once the pump is inserted.  More on this after sugery…

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.