BRETT BRADLEY

Dob-11/06/98

Mom, Dad and two-year-old sister, Lauren, all anticipated the arrival of their new family member, Brett. Jennifer (mom) was induced at 38 weeks and Brett Daniel Bradley was introduced to the world on November 6, 1998.

Unfortunately, after a 27-hour delivery, Brett was not breathing. The little guy was not in a hurry to leave his prior living arrangements, much to the doctors and mommy’s disappointment. After a couple minutes of intense pushing, Brett came out a little blue and at this moment our world started to swirl. Alarms were sounded and in what seemed like less than five seconds the room was filled with nurses and doctors and all kinds of equipment. It was totally amazing. Trained for emergencies such as this, the Neonatal Intensive Care Team rushed in to resuscitate our son, who was already fighting for his life.

While having only minutes to see him in the delivery room, we immediately knew there were problems. His head shape looked a little different than most newborns, and extra digits (fingers) on both hands and one foot immediately brought us to a place that we will never forget. A normal pregnancy was supposed to give us a normal baby. We did the classes, received great test results and sonograms, and followed all the rules. This happens to other people, not us. Our worst possible nightmare was coming true; something major was wrong with our little boy.

Mom and Dad will forever remember the quick second we had to look at our new son and had to quickly give him a welcome kiss before he was rushed to the NICU. We anxiously waited until the doctor returned about an hour later and informed us of Brett's status. Although, breathing on a respirator, they told us Brett had many problems. These included: an extra digit (pinky finger) on each hand and an extra pinky toe, two holes in his heart (ASD), wide set and “bugged” eyes, low set ears, sloped, tall forehead, club feet, contractures of the knees and elbows, small rib cage, short neck, flatness in back of the head, umbilical hernia, and a small jaw. We did not expect any health problems. A geneticist came in to see Brett in the NICU that evening and told us she was 99% sure Brett had Trisomy 13, a terminal syndrome, with most children not making it past their first birthday.

We were still in a state of shock when we heard the news. The neonatal doctors took Brett's blood and immediately sent out for a chromosome test to verify the syndrome. We had to wait four long days for the results. (Thankfully, Brett’s grandpa (Papa Jim) gave us a computer knowing we would find it a great tool for researching many of Brett’s potential problems). That evening, daddy (Blake) pulled up the syndrome on the Internet and after reviewing the symptoms, we were convinced he had Trisomy 13. Our prayers had been answered when test results came back negative and we were relieved to learn Brett did not have that syndrome. Still, this was only the beginning of our roller coaster in the hospital.

Next, an orthopedic doctor visited Brett and examined his extra digits and his tightness in his arms and legs. Brett kept his arms tucked into his chest and his knees tucked into his stomach as if he were still in the fetal position. It was physically impossible to straighten out his arms or legs, with the fear that they may break in two. The neonatal specialist said they doubted Brett would ever walk. Again, our hearts fell.

The next day, three of Brett’s ribs on the right side of his body were broke. At birth, he had a broken right clavicle, but his first day x-rays did not show any other broken bones. It was a mystery to why the bones suddenly broke, which led doctors to believe our son may have a possible bone disease. Luckily, test results came back negative. However, now because of his fractures, he had to remain lying on his left side and sadly, mom was still unable pick him up.

After the third day that Brett was in the NICU, mommy was finally able to hold, comfort, and breastfeed him. It was a joyous occasion. Also, individual family members, who were escorted by mom or dad, were able to visit the new addition. Brett’s sister, Lauren, was too young to go to the NICU and she did not understand why her mommy was always at the hospital and why the new baby was not coming to meet her. But thankfully, after ten days, Brett was able to come home (equipped with an apnea sleep monitor and reflux medication). Lauren (17 months) who was amazed by this new little person, immediately planted a big kiss on her baby brother’s forehead.

Not more than a few weeks later we were beginning to follow up with the many specialists referred to us by the hospital. Unaware of what syndrome Brett might have, every checkup was filled with nervousness and anticipation. We first saw a cardiologist, due to Brett’s Atrial Septal Defect (ASD), who informed us that one hole in his heart had already closed and the other hole should close within 12 – 18 months. Good news.

Next, we saw an opthamologist. Since Brett was under oxygen at birth, he had to have his eyes checked out. Of course, our concerns were that Brett wouldn’t be able to see. We did know that his eyes were spread a little further apart, but based on the uncertainty of everything else, our hearts had us preparing for the worst. He did have a premature retina, and it needed to be monitored. A month later, it healed on its own. According to the doctor, Brett’s eyesight should be normal. Again, more good news.

Then, we saw a pediatric orthopedic doctor who diagnosed Brett with Arthrogryposis and recommended that we begin physical therapy. So, at one month, Brett began weekly therapy treatments. We also went to see a neurologist, since Brett’s EEG at birth was abnormal and the results recorded his brain waves to correspond to a 34-week baby instead of a full-term baby. Fortunately, the neurologist found Brett to be within normal limits of a 1-month-old and stated the abnormal results could have been from the trauma associated with his birth.

Finally, we saw the geneticist who came to see Brett in the hospital. She examined Brett again and then went to work researching all of his anomalies. While, not certain, she told us Brett might have a rare syndrome called, “Carpenter’s Syndrome”. At this visit, we were advised that he may need skull surgery in the future based on the way his head was shaped. While she felt this could be years away, we still needed to monitor the size of his skull and get it measured monthly by our pediatrician. We were given a description of the syndrome from some case studies, and of course, we saw a picture of a severe case of the syndrome and that stuck in our minds. We constantly had to refer to other medical books to figure out what all of the words meant. While the Geneticist said not all of the symptoms applied to Brett, a great majority of them did. Some of the symptoms included having craniosynostosis, extra digits, heart defects, contractures of the legs, short stature, obesity, and mental retardation. These along with all of the things we knew he had, only had us fearing for the worst. Mommy did some more investigating on the internet and found a few families whose children also have the syndrome. Fortunately, their kids are doing very well, yet many parents doubted Brett had the same syndrome since his features were more different than with their children. At least, it was nice to receive the support and begin to learn information regarding what Brett’s future may entail.

A couple weeks after Brett was born we slowly got into a routine and doctor visits came to be the norm. At first, Brett was quite hands on, but things slowly got back to normal (or as normal as one would think). While searching the Internet once again, (in retrospect, the Internet paved the way for putting us in touch with resources and people that otherwise, would never have been available), Daddy came across two cranial facial doctors in Dallas (which was only 30 minutes from our house). Knowing what the geneticist said about Brett possibly needing intervention to correct his head shape, we decided to make an appointment with the cranial facial plastic surgeon, Dr. Jeffrey Fearon. We were sure he would tell us everything was fine. However, after only a few minutes into our conversation with Dr. Fearon, he mentioned that Brett, now 3 months, would need surgery for craniosynostosis.

Evidently, Brett’s head was growing taller and we were told that without the surgery his brain would either stop growing, or would grow up - instead of outwards - towards the forehead. The doctor examined Brett and determined that he had mild ridging on the coronal (on sides of forehead and face) sutures of his head and Brett’s lamdoid (back of head) sutures were closing prematurely. If the sutures closed, it would eventually cause pressure on the brain. Dr. Fearon wanted us to see his cranial facial team, which consisted of an Ear/Nose/Throat doctor, neuro-opthamologist, geneticist, neurosurgeon, anthropologist, and a developmental psychologist. After we saw the team and when Brett completed his CT scan and a MRI of the head, Dr. Fearon suggested surgery at around 8 to 9 months. We would need to monitor him with head measurements and CT scans until then.

At eleven weeks, Brett’s head circumference was very small, between the mean and the second percentile. As his head grew taller, his temples indented inward and his eyebrow bone became more prominent.

We decided to see another cranial facial doctor for a second opinion. He, Dr. Salyer, told us Brett should have the surgery at six months and wanted to begin the scheduling process. Due to both doctors having wonderful reputations, we searched the advice of two neurosurgeons as to the timing of the surgery. Since we would be doing this surgery only because it was medically necessary, and not for cosmetic reasons, we wanted the neurosurgeon’s opinion of just how much time Brett’s brain had until it would be running out of room.

Both neurosurgeon’s agreed Brett could wait until about 8 to 9 months. We then chose Dr. Fearon and his partner, Dr. Bruce, the neurosurgeon, to follow up and perform the surgery. Not only did Dr. Fearon have a reputation for a great bedside manner, but he also currently performs more cranial vault surgeries than any other plastic surgeon in the United States (between 50 & 60 a year, which is more than one a week). Dr. Fearon also mentioned the longer you can safely wait for the procedure, the less surgeries you’ll need as the child grows older.

Meanwhile, Brett was accepted to the orthopedic and neurology clinic at Texas Scottish Rite Hospital in Dallas, a Shriner’s hospital. The orthopedic doctor, Dr. Sucato, monitored Brett’s tightness in his arms and legs. Even though Brett’s whole body was affected; our focus was on Brett’s feet and legs. Brett kept his feet turned inward and it was still impossible to get his legs to straighten out. Therefore, our goal was to continue physical therapy to help relax and stretch his legs. Brett qualified for weekly physical and occupational therapy through the state-funded early childhood intervention program. Conveniently, we were able to receive the majority of his treatments in our own home.

In March 1999, our family moved to Las Vegas, Nevada, for daddy’s job. Fortunately, Blake’s parents live in Vegas and are great support. We were spoiled since we had mommy’s family in Texas. The struggle of the move was finding new and reputable doctors. We quickly learned that we were very fortunate with the amount of quality, pediatric specialists and hospitals in the Dallas/Fort Worth area. We enrolled Brett in Special Children’s Clinic (SSC); another state funded early childhood program. Brett continued to receive his physical and occupational therapy twice a week and one more session a week from an outside occupational therapist.

At four months of age, Brett usually resisted lying on his tummy, due to his arms, wrists, shoulders, and torso being so tight. It was a great triumph when Brett began reciprocal kicking to exercise his legs.

At the end of May, SSC’s pediatrician recommended we receive a CT Scan on Brett’s spine to rule out Spina Bifada and other abnormalities. Once again, we had a huge scare, but thankfully, everything was fine. Brett does have a sacral dimple above his bottom which, if open, would lead to his spine, but his neurologist confirmed that it is closed and will not cause Brett harm.

We flew back to Dallas in June when Brett was seven months old. Since mommy’s parents still live in Dallas, and we are happy with our former doctors, we decided to continue to see our specialists for follow-ups. After another head CT scan, it showed Brett’s head was beginning to fall off the curve, meaning his head circumference was not growing like it should be. He was also developing a metopic ridge and the back of his head was getting flatter. Dr. Fearon and the neurosurgeon, Dr. Bruce, also noticed he had less CSF fluid, larger ventricles, cerebellar tonsillar herniation and a mild chiari malformation. (We’re not sure what all that means, but we included it just in case those with similar cranialsynostosis can compare notes too!) They decided to schedule surgery and enlarge the front of Brett’s forehead.

We scheduled Brett’s surgery for August 6, 1999. The date was quickly approaching. Mom and Dad both donated blood for Brett just in case it was needed. Mommy flew out to Texas, once again, with the children, a few days before Daddy, so Brett could get his third MRI and CT scan on the head before the surgery. Luckily, Brett’s chiari malformation, which is located near the brain stem, was not severe, and they were able to go ahead as planned and extend the forehead. Had it been needed, they would have extended the back portion of his head, which is a more surgically involved. Due to a schedule change, the surgery was then moved up to August 4th and daddy quickly came to Texas.