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.
Yesterday and Shiva for the family
10-10-08 – 8-2-10
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.
Not Good
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.
Four Months
4th of July was the four month anniversary of Evan’s accident and his life at the hospital. Four months…how did that happen? The baclofen pump has been in place for nearly a month and the quantity of the drug has been increasing steadily. Evan is now at 900 micrograms (equals 0.8milligrams – a far cry from the 60 milligrams he was on orally). Evan’s body is much more relaxed, though he still has storming episodes where his whole body stiffens up and gets agitated. He continues to stay in the PICU to ensure his heart rate and respiration are monitored closely, since the baclofen can relax the body too much and make a child stop breathing all together. Some of the nurses and doctors also think that Evan’s agitation is a result of his feeds, so they have turned off the formula he receives through his PEG about a week-and-a-half ago. Instead, Evan receives TPN, which stands for total parenteral nutrition and includes all the essential salts, glucose, amino acids, lipids and vitamins via IV making it nutritionally complete . We are not convinced it is a feeding issue, but his neurosurgeon wants to hold off on food as a variable until we capture the storming episodes and bring them under control. In the next few days they GI Doctor is going to go in with a video scope to study Evan’s whole GI Tract. Everyone is out of fresh ideas and this test may reveal the source of his extreme discomfort. In the mean time we will remain in the PICU.
Surgery went well, details to follow.
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.
The Pump and our A-HA moment!
We said our goodbyes to Evan early this morning as he was wheeled into the operating room to get his baclofen pump installed. That procedure was successfully completed in less than an hour. Soon afterward out came Evan accompanied by his neurosurgeon who was sporting a knowing grin on his face. The grin was as a result of our “A-HA” moment. You see, after last weeks’ surgery (when the catheter was inserted in his brain) we had 2-3 good days with Evan finally feeling relaxed. The next few days he reverted back to the agitation and distress similar to before the surgery. The doctors, nurses and all of us where perplexed at why his spasticity returned after the days of calm. Theories were thrown around the pediatric ICU, but no one really understood what happened…until today.
A post-op catscan revealed that the cathater that was inserted into the third ventircle of Evan’s brain had slipped out of its position. It probably slowly worked its way out of position explaining the gradual regression during the week. So now what…
Evan is going back to the operating room on Monday morning to re-establish the catheter into the correct position, this time using a more robust tie-down. In order to limit the size of the lump under Evan’s scalp, during the original procedure the surgeon had opted for the method which failed (we still love him). We are disappointed that Evan has to go back through another surgery, but happy to know that we have the plan in place to get Evan back to comfortable.
The hours following today’s surgery were quite difficult for Evan. Here is a picture of a sample pump I placed on Evan to give you an idea of how big it is in proportion to the little guy. The actual pump is installed under the gauze in the same location on the other side of his tummy.
Baclofen Pump
After finally putting him on a steady IV of pain meds he was able to fall asleep for the better part of this afternoon. The next couple days are going to be difficult. Unless something remarkable happens we’ll wait until Monday afternoon for the next update.
3 months and a pump
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…
48 Hours Post-Op
It has now been just over 48 hours since Evan came out of surgery. The docs have ratcheted up his dosage of baclofen every 12 hours and it currently stands at 240 micrograms per day. This is 0.24 milligrams versus the 60 milligrams he was receiving orally before Friday. At the current dose, Evan finally seems very relaxed with eyes open and a heart rate that shows the calm on the monitor. His body is more loose with increasing range of motion. It is really fantastic to see Evan in such a mellow state.
OK, comic relief for you…Daddy just snapped this photo. Enjoy!
Just Chillin with my pals
Surgery Day Updates
12:35 Last update for today. Finally fell asleep at 8:00. The 5-8 stretch wasn’t too bad but he sure wasn’t happy even with the morphine. I made Mom go home at about 9:00 and went to sleep on the couch in his room. I just woke up (had a bad dream that I was in the hospital because my kid just had brain surgery) he is still in a very deep sleep and looks quite comfortable. I am going back to sleep and feeling hopeful for the first time in quite a while. 🙂
5:20 We have been back in the room for an hour and a half. Evan just woke up. He’s not happy so the nurse gave some morphine to keep him comfortable. The new tube just got hooked up and the baclofen is flowing to his brain. The starting dose is incredibly small at less than 100 micrograms per day vs current 60 milligrams orally. They really shaved quite a bit of his hair, the OR nurse was kind enough to save some locks for us.
3:45 Done Dr. just came out and gave us the all clear.
3:15 Anesthesiologist just called us in the waiting room. All went well. Doing final stitches and cleanup. Will be doing a post-op CAT Scan and then straight up to Pediatric Intensive Care unit where he will stay for the next few days.
2:55 Brief chat with an OR Tech who went into the room to get a status update. Everything appears to be going well. Expected to be another 45 minutes.
2:38 Still waiting…..
1:23 Surgeon just came to see us. Let us know the GI Dr. Already replaced his G-Tube with the Mic-Key button. Now the brain work will begin.
12:55 Mom and I went with Evan to CAT Scan room. Still sleeping. Dr. Started anesthesia. Lots of kisses goodbye. Now in the waiting room.
12:20 Rolled down to pre-op. Evan was sound asleep. Final meetup with the surgeon. Hooked him up to IV and monitors. Still sleeping on his own.
Zen Garden & Surgery
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.
Posing in the hallway
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.
Fresh Air
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.