Bryson, age 12, 6th grade, September 2004

My pregnancy was not exactly normal...I went into pre-term labor twice at 7 months and was put on bed rest and pills for the remaining two months, plus had placenta previa. The bed rest must have worked, because Bryson was born later than we expected. The doctors had originally estimated that he was due July 12th, then an ultrasound made them adjust that to August 25th. He was actually born September 2nd, weighing in at 7 lbs. 9 oz.! The one thing I question about all of that is this: ultrasound due date is determined based on fetal head circumference...in Pfeiffer's, the skull sutures are fused which does not allow the skull to grow properly...my original due date was mid-July which coincidentally (or not?) is when I went into pre-term labor twice. So, was it really pre-term labor, or was he really supposed to have been born in July and we delayed his arrival? His dad was only 6 lbs at birth, and I was 7 lbs 3 oz, so a small baby born in July would not have been out of the realm of possibility. All of that aside, though, I honestly don't think he would have lived if he had been born in July, so it's a good think we kept him in the oven a little longer.

Delivery was difficult, also, and Bryson was delivered by vacuum extraction at 12:38 AM. He was not breathing, and was blue. It took the doctor's a little over 2 minutes to get him breathing. His first APGAR score was only 3, and his five-minute APGAR was 7. After getting to hold him only long enough to have a quick picture taken, he was whisked off to the nursery. His dad went with him, and I was left not knowing quite what was going on. My husband came back a little later and said that he was having his first bath and was breathing fine at that point. The nurses told us that he had a cone-shaped head, but that that was normal for babies born by vacuum extraction. At that point we thought all was well, even though they kept him in the nursery for several hours. Later that day, after we'd moved to a regular post-delivery room, a doctor with a heavy foreign accent came in and started rattling off a bunch of stuff. He had two student doctors with him, who didn't speak, and we only understood a couple of words that he said because of his accent. We heard: baby, syndrome, & surgery. Needless to say, we were terrified because we didn't understand what he was trying to tell us. The nurse came in after he left and explained that our baby appeared to have a syndrome that involved his skull and facial bones, and that they had called in a geneticist from another nearby hospital and he would be able to tell us more when he got there. The next day this funny little doctor (Dr. Tal Thomas) with a wheeled cart loaded down with computer equipment came in to see us. He had an accent, too, but it was Welsh and much easier to understand. He was so nice, and put us so much at ease. He examined Bryson to find out if he had Pfeiffer's Syndrome with Kleeblatschadel (clover-leaf skull) formation, or just the Kleeblatschadel itself. The most physically notable difference is that in Pfeiffer's Syndrome, the big toes and thumbs are affected, where Kleeblatschadel only affects the skull. Bryson's big toes were broad, though his thumbs were not, and from that he said that it was probably Pfeiffer's Syndrome with the clover-leaf formation. The diagnosis remained at question for a couple of months while he watched Bryson grow, and when the big toes remained really broad over a length of time, he confirmed the diagnosis as Pfeiffer's Syndrome. This doctor told us that Bryson would have to have operations, but that he was going to send us to the best doctor in the world, the 'King of China' as he called him, who just happened to work at a nearby hospital. This was Dr. Argenta, a world-renowned cranio-facial reconstruction surgeon. He has been a Godsend to our family!

We were released from the hospital on the evening of September 5th, with the instructions that we should bring Bryson to see Dr. Argenta a few days later, and that until then we should not take him out in public for fear of him getting sick. We took him home and started life as brand-new parents. Everything seemed to be fine, except that he couldn't keep food down very well, and he didn't seem to breathe quite right at night. He seemed to be holding his breath. I was soon totally exhausted because I stayed up all night watching him breathe. The pediatrician that we had at the time told us that we were just new parents and that he would be just fine...I didn't need to watch him constantly like that. I'm glad I did!

We saw Dr. Argenta, and scheduled Bryson's first operation for when he was 3 weeks old. This operation was a big one, a frontal craniectomy. He came through the operation just fine, and went straight to the Pediatric Intensive Care Unit (PICU). It was there that the nurses discovered that he had APNEA...he held his breath when he was asleep and didn't breathe correctly, lowering his oxygenation levels. When he was released from the hospital a week and a half later, they sent us home with an APNEA monitor, which would emit a shrill siren anytime that Bryson stopped breathing correctly. Thank God they did! He did not outgrow the APNEA until he was 3 1/2 years old, and there were many times when the monitor went off all night long. Several sleep studies performed at the hospital showed up to 60 episodes a night!

Over the next year, Bryson had surgery every couple of months. He had operations to reconstruct his skull, implant ear tubes, correct a hernia, and implant a VP Shunt. That one was scary! When he was 4 months old, he had just had another operation on his skull during which the doctors opened up two man-made fontanels (soft-spots) to allow room for brain growth. He was left with two hollow spots in his skull that were not very noticeable. Two months later, at age 6 months, we were sitting on the couch and literally watching those spots swell and bulge, becoming very noticeable. It scared me half to death. I called the pediatrician's office and was told that I was, again, imagining things. That brain swelling didn't happen like that, that it took months, not hours. (This was the same pediatrician's office that told me that I shouldn't be concerned about lots of crying and projectile vomiting of formula...which I later learned, after he outgrew it, was from bad reflux.) Anyway, I didn't quite believe them, so the very next morning we were at Dr. Argenta's office when they opened. He took us right on back, took one look at Bryson, and sent him for a CT scan. He told us that Bryson had Hydrocephalus and needed surgery right away. He also told us that if we had waited he would have suffered irreversible brain damage! (Needless to say, I changed pediatricians immediately after that!!!) Bryson had the shunt implanted 2 days later, and has never had a problem with it since. In fact, we are not even sure if it's still needed at this point, or not. The doctors said that one of two things has occurred...either he still needs it and it's still working fine, or he's outgrown the need for it. Either way, they plan to just leave it in place, which is normal procedure.

When Bryson was two years old, he developed an intense allergy to Latex. We were told that this happens often to medical staff who have had constant contact with latex gloves and such, and that it wasn't unheard of in patients who have had a lot of operations. This was another very scary thing, as it was so violent. Bryson had been playing with a latex balloon, which was normal for him, and all of a sudden he started turning quite red and his face began swelling. We were actually at a store at the time, and rushed him straight to a doctor's office (not ours, but the closest one to that store). In the few moments that it took us to get there, his face had swollen to the point that even the tissues in his eyes were swollen! They rushed him back and gave him medicines, which calmed everything down. The doctor then explained to us that Bryson was suffering an anaphylactic reaction to the latex, and that he was just moments from having his airway swell completely shut! After that, we have been extremely careful to avoid latex items at all costs. Recently, at a church function, Bryson accidentally came in contact with a latex balloon, and it was proven that he is still highly allergic to latex. In a way it was a good thing that it happened, because he had gotten to the point where he no longer believed that he was allergic to it...he thought he'd outgrown it, even though I told him that you don't just 'outgrow' such an allergy. In this case, a child who didn't know Bryson was allergic to latex got out a balloon and started playing with it. Bryson didn't see it until it accidentally bounced off of his face. His youth pastor brought him home immediately and I gave him a dose of Benadryl, and he still developed a horrific rash that lasted several hours. Luckily the Benadryl kept the swelling down and his airway didn't become involved, though. Now he is very cautious, because he knows that he IS still allergic to latex and that if one brief touch can give him that type of rash anything more would cause much worse!

When Bryson was little, he developed a condition called "Chronic Croup". Most babies get regular croup, which gives them a barky cough and makes them feel bad. They also normally outgrow it by the time they turn 3 or 4 years old. It's much, much worse for Bryson. For one thing, it's something that he's never outgrown, and possibly never will. For another thing, he has a narrow airway, so it tends to completely swell his airway closed. In the past he would stop breathing and turn blue. We called 9-1-1 many times, and took ambulance rides often, with hospital stays and lots of medicines, breathing treatments, and tubes. We almost lost him on several occasions. Several years ago, when we switched to our current (wonderful!) pediatrician, he set us up with an at-home nebulizer and medicines. This has made life so much easier! Now when the first signs appear, we give Bryson a dose of Prednisolone (steroids). Since that can take 6-8 hours to work, we also give him breathing treatments with both saline and Pulmicort. That works wonders…we've only gone to the hospital once for a croup episode in the past two years! Plus, as the years have gone by, the episodes have lessened in frequency…when he was a baby it would happen once or twice a month, and now it's generally two to three times a year.

Bryson is now (May, 2005) 12 1/2 years old. He has had 26 operations, including several LeFort III procedures. He is, for the most part, a healthy kid, though he does have moderate hearing loss and suffers from ADD. Doctors have offered to perform exploratory surgery to see if they can correct his hearing loss (multiple sets of ear tubes have not been able to fix it), but Bryson has decided that he does not want to have that done because there is a possibility of doing even more damage and losing the level of hearing that he currently has...I can't argue with him. Due to his ADD and some mild learning disabilities, he is in inclusion classes at his Middle School. With medication he did well for the first half of 6th grade. Half way through the school year, puberty kicked in and the medication he was on for his ADD made him unable to eat or sleep at all, so we had to take him off of it. We have now struggled with different meds for the last half of the school year, and are in search of a different (and better) medication for him before he starts 7th grade next August. We have been blessed with the fact that Bryson has quite a few friends in school and in our neighborhood. I always worried that he would be picked on by the other kids, but that really hasn't been the case. For the most part, he is just a normal soon-to-be teenager. He loves playing basketball, soccer, and football, hanging out with his friends, singing, playing video games, riding his bike, and is a big UNC Tarheels fan. He is growing into a strong young man...not just physically, but in character as well. Bryson will have more surgeries in the future, though at this point nothing is actively scheduled other than getting braces over the summer. We are so proud of him for all that he has gone through, all that he has become, and all that he will become in the future. He is a great kid, and we love him very much!

I should also note that Bryson does have a younger brother, Zack, who was born without Pfeiffer's Syndrome. When I became pregnant with Zack, we didn't know if he would have it, or not, but he does not.

I know that this has been an epic, but I didn't want to leave anything out. Every bit was important in making Bryson who he is today.

Thanks,
Melissa Hooper

Update:  February 28, 2006