Every Generation Has A Legend. Every Journey Has A First Step. Every Saga Has A Beginning.

Friday, March 25, 2011

Make A Wish!!!

In November of 2010, I submitted Perrin's name to Make A Wish after we got the diagnosis of blepharospasm and Meige's syndrome. Upon research done by my husband and I, we found out that Perrin has the degenerative version of blepharospasm. It is a fairly rare disease, affecting only one in every 20,000 people in the United States.

Some of our research turned up these facts:
-Blepharospasm usually begins gradually with excessive blinking and/or eye irritation. In the early stages it may only occur with specific precipitating stressors, such as bright lights, fatigue, and emotional tension. As the condition progresses, it occurs frequently during the day. The spasms disappear in sleep, and some people find that after a good night's sleep, the spasms don't appear for several hours after waking. Concentrating on a specific task may reduce the frequency of the spasms. As the condition progresses, the spasms may intensify so that when they occur, the patient is functionally blind; and the eyelids may remain forcefully closed for several hours at a time.
-Blepharospasm is thought to be due to abnormal functioning of the basal ganglia which are situated at the base of the brain.
-Blepharospasm can occur with dystonia affecting the mouth and/or jaw (oromandibular dystonia, Meige syndrome). In such cases, spasms of the eyelids are accompanied by jaw clenching or mouth opening, grimacing, and tongue protrusion.
-There is, unfortunately, no cure.

So it is a fairly hard disease for him to have at 8 years old. It affects him when he's tired, stressed or nervous. On March 10, 2011, I decided to call the local chapter of Make A Wish back to check on the progress of everything. Great News!! Perrin had been deemed medically eligible for his wish almost immediately back in November. They sent a letter, which I guess was lost in the mail. We also had been already paired with volunteers, or "Wish Granters", They also asked if I happened to know what his wish was. Anyone who knows Perrin knew what it was right away. He is a huge star wars nerd. His wish is to go to Disney World for Star Wars Weekend to meet Darth Vader, the Stormtroopers and the bounty hunters. We met with our Wish Granters on March 15, 2011 for 2 hours!! We signed a bunch of paperwork and they were talking about possibly sending him in May 2011. Since Star Wars Weekend is only May 20-22 and May 27-29, we chose the beginning of June since he will be out of school by then. So we set our dates for June 2-8.

The past is the past.

Since the shunt malfuntion/surgery mistake, we have been slowly trying to rebuild what was broken down. We changed neurosurgeons. We now have a FABULOUS neurosurgeon. At the advice of our neurologist, we went to see a neuropsychologist. We learned a lot of new things. He has been diagnosed with ADHD, dysgraphia and depression. We were advised for him to start seeing a counselor because he is very aware of his medical conditions and disabilities. They said he knows that his life will be very hard. I have sat him down and explained to him, while his life will most certainly be hard, it will be wonderful because he is our baby boy and we love him and will help him in any way possible. He is a happy boy most of the time and he now has an emotional support animal named Stella.

Every Saga Has A Beginning.

So, from 2003 to 2005 it was difficult but par for the course for special needs children. When he was 9 months old my husband had to beg his PCP to do a CT scan because he was crying constantly, holding his head or sleeping for 15 hrs out of the day. Finally she came back with a referral to a neurosurgeon. Said he had a severe case of hydrocephalus. Our neurologist argued with us and said she was the dr and we didn't know anything. We decided to go behind her back and go to the neurosurgeon. He told us we had 1 day to get the surgery to put a VP shunt in. The hydrocephalus was preventing Perrin from sitting or even crawling on his own. We had the surgery done in Oct of 2003. The day after surgery, he was sitting up and playing. It was the happiest I had seen him in A LONG TIME! After that, he had a circumcision, because the skin was growing around the urethra, therapies, got weaned off oxygen and phenobarbital at a year and a half old. At 2, he was diagnosed with Cerebral Palsy. The neurologist at Children's Hospital in New Orleans attributed it to the Grade IV brain bleed and the hypoxia at 2 days old. In August 26, 2005, we were set to have a nissen re-do and a hiatal hernia stitch up. The day of surgery he woke up with a 104.5 degree fever, therefore canceling surgery. Later we would see this as divine intervention. On August 28, 2005 we were ordered to evacuate New Orleans due to Hurricane Katrina. Had he had the surgery, he would have been in PICU during Katrina and had to have been air evac'ed out. WHEW! We ended up in Branson, MO for 6 months. In Dec 2005 He had the Nissen Redo and Hiatal Hernia surgery in Springfield, MO. We then went down to San Antonio for a titanium rib evaluation. His rib are malformed but luckily it is seen as "just a cosmetic issue" Since they wanted to re-eval him in a year, we decided to move to TX. My inlaws lived in Austin so we figured it would be nice for him to be near his grandparents. We settled in Round Rock and he started PPCD (preschool program for children w disablities) thru the public school. As he went thru the years, he continued to progress and by Kindergarten, he was mainstreamed with aides, resource and therapies. By 2nd grade, he had a great group of friends. A couple of the parents in class have likened him to Norm from Cheers. When he walks into class everyone screams out "PERRIN!!!" It is great to hear how much his classmates love him and look past his differences. By Thanksgiving of 08, he was starting to regress and have mood changes. We did a CT scan and it showed that his shunt was clogged up. He needed yet another surgery. We were unsure as to if it was just the tubing in his belly that needed replacing or if it was the tubing in his brain as well. Dec 17 he had his shunt revision. The neurosurgeon said that everything was fine. She had to replace the whole system because it was completely clogged up with protein. The next day he was MISERABLE! He could barely sit up, when he did he gagged and he was refusing to eat. 2 day later, he was eating (not much) and the neurosurgeon said we were to be discharged. I objected because he wasn't drinking or eating and he was still gagged a lot. He couldn't handle the pressure of his brain. She reprogrammed his shunt and let him stay one more day. Next day she said he needed to go home. Once again, I protested. He was still refusing to eat and was only drinking enough to stay hydrated. She said he had to get used to the pressure (said that was why he was gagging every time he sat up) and the only way to get him to eat was to force him at home where there were no IVs for hydration. So we went home. The entire trip hm, 45 mins, he gagged. We went back to the dr 3 days later. He had stopped gagging and had slowly starting eating and drinking again. She said he could return to school the following week. He went back and on Monday, Tuesday and Wednesday he was going to the nurse's office. He was having bad headaches and was nauseous. I brought him into the ER and the neurosurgeon came in and tapped his shunt. It wasn't draining, which meant it was malfunctioning and needed to have emergency surgery to pull the catheter back through his brain as there were not enough holes in the ventricles to drain the fluid. Also, there was a leak in the cap of the reservoir, so that would need to be replaced. He was in surgery for about and hr and a half then wheeled up to his room, looking better than the first time!!! This time the surgery was perfect. He went home and was functioning great.

Every Journey Has A First Step.

I want to share Perrin's whole story. I was 20 years old when I got pregnant. It was a big surprise to my husband and I, we had only been married for 2 months. We were very excited to start our family. The pregnancy was normal. No problems until December 24, 2002. It started as a normal day and I was 25 weeks pg. I headed out to work and was there for about 15 mins and this excruciating pain started in my back. It got so bad I could hardly stand up. I called my mother, who lived close by, and she came there and picked me up. She rushed me to the hospital and the ran me up to L&D. They did an ultrasound and saw that I was already 3 cm dilated and my bag of waters was bulging. They started me on magnesium sulfate (the worst stuff on the face of the planet!!! Makes your veins burn, your head spin and you have an uncontrollable urge to vomit) and gave me steroids for his lungs and demerol to make me sleep and as a painkiller. The rest of the night is a blur. What I remember is at 12:30 pm I was admitted, 2 pm the doctor came in to talk to me and he pulled my husband out into the hall, 6 pm my water broke, 9:35 pm I feel him drop down and a strong need to push. I push the nurse call button and she comes running in and while trying to call the NICU staff, she is holding his tiny head in and telling me not to push. At 9:38 pm on 12/24/2002 Perrin was born, 1 pound 13.8 ounces, 14 inches long. All I saw was his limp body being picked up and put under the warmer surrounded by nurses and doctors. I hear them repeatedly shout, "he's not breathing" "we need to intubate NOW!" I start FREAKING out! My husband finally breaks through the throng of drs and nurses to see that they have him intubated, bagged and started to pump air into his little body. Then they take off running with him to the NICU. I still have not seen him at this point. Around 11, the perinatologist comes into talk to me. He warns me that Perrin is in very bad shape. Most preemies born at this age will be severely disabled. There is a large chance he may not survive, but he was bigger than they expected and longer than they expected. At 12:30, I get to see him. My beautiful baby boy was so tiny and I couldn't even touch him. I could barely talk to him because they didn't want him stimulated. He was pretty much in a drug induced coma. The next day, Christmas Day, I was discharged from the hospital and went home after spending a large part of the day sitting beside him in the NICU. On 12/26, We tried to go up to see him and kept getting turned away. No explanation. Just "No, you can't come into the NICU. Call again later" So we finally just go up to the hospital and sit in the waiting rm. The Neonatologist comes out to talk to us and explains why we couldn't go in. Perrin was put on a ventilator when he was intubated. The vent was too strong for his little lungs and it blew his left lung and the air that leaked into his chest cavity collapsed his right lung. He was hypoxic for 45 mins. They finally got him stabilized and allowed us to go in to see him. He had a chest tube in and looked to be in pain. I couldn't stop crying. As we sat bedside, the dr asked "If he destats would you like to hold him as he dies?" and "How long do you want us to fight to keep him alive?" I was stunned. I said you fight until you can't anymore. There is not stopping. He gradually got strong enough to be transferred to Tulane University Hospital in downtown New Orleans on New Years Eve. He had to have a PDA ligation that they did not have a surgeon at the local hospital. He was at Tulane for 3 months. While there, he had 3 surgeries, 2 PDA ligations and a nissen fudoplication/ Gtube placement and a bout of seizures. Turns out, the seizures were a type of meningitis. They treated him with phenobarbital and antibiotics. On April 5, 2003, Perrin was finally "healthy" enough to go home. He came home on 3 medication and oxygen but it was great to have my baby home.

Every Generation Has A Legend.

So, where to start? My name is Susan, mom to 3 beautiful children; Perrin, Julianna, and Aleksander. My oldest was born at 25 weeks at 1 lb 13.8 oz. As a result, he has CP, Hydrocephalus controlled by a VP shunt, blepharospasm, Meige's Syndrome, dystonia, ADHD, and dysgraphia. He just had his 11th surgery in Dec of '09 for a shunt revision. Unfortunately, the neurosurgeon made a mistake and pushed the catheter too far into his brain and nicked the corpus colloseum. So, with that, he regressed horribly. Complete 180 in attitude, school work, attention, etc. We have been slowly progressing back to where he once was but it is still an uphill climb. He has been quick to anger, depressed, and just overall more moody than he ever was.