Connor Carlisle

Introduction

Just like our first son, Reid, our second son Connor came into this world bringing us tremendous joy. Connor’s medical condition, however was a frightening introduction to a whole new world. Connor has Cloverleaf Skull (also known as "Kleeblattschadel") which is a rare form of Cranio Synostosis. Cranio Synostosis occurs when one or more sutures of the skull are fused at birth. The skull bone is "programmed" to grow too quickly. Cloverleaf skull results in all or all but one of the skulls sutures being closed at birth.

Connor also has an Arnold Chiari Type I malformation. This is a brainstem abnormality where the cerebellar tonsils are being pushed down into the spinal column. Connor's Chiari has been quite problematic, requiring two "decompression" surgeries (it is unusual to have more than one decompression surgery and rare to have these surgeries so young). Decompression surgeries involve removing the top vertebrae (usu. C1&2) and surrounding bone that is pressing upon the brain stem. These are extremely dangerous surgeries. The brainstem area controls the respiratory center. As a result of compression to the brainstem, Connor has sleep apnea. This is why he has a trach and is on a ventilator when he sleeps. We remain hopeful that he will outgrow the need for a vent. However, it is likely that Connor will need to have another decompression sometime in the future. Thankfully, head growth begins to stabilize in the elementary school years. In addition to his breathing, the Chiari is affecting his gait and his swallowing ability.

Cloverleaf Skull & Arnold Chiari Type I are Connor's primary diagnosis. These have resulted in the following: placement of a VP Shunt (for fluid on the brain), arachnoid shunt (in spine), a Tracheostomy (Connor continues to breath through his trach), a gastronomy-tube (Connor receives his nourishment through a feeding tube directly to his stomach), P/E tubes in his ears to help with ear pressure and drainage due to the unusual shape of his ear canals, mild hearing loss, mild Cerebral Palsy, left & right inguanal hernia, and a mediport through which Connor can have blood drawn or IV meds given. Connor also had an Atrial Septal Defect (heart ASD), which is now resolved (closed on its own).

The last five years have truly been a blur. After Connor's birth our lives took on a surreal feel. Hospitals, ambulances and emergency rooms became an all too familiar part of our lives.

2001 (top)

Connor was born on Oct. 11, 2001. The week prior to his birth, my water broke (33 wks). An ultrasound showed Connor's unusual head shape in utero, and a Pediatric Neurosurgeon was called. The Neurosurgeon said there was a possibility Connor had something very rare called Cloverleaf Skull. I remained in the hospital and was given drugs to lengthen the pregnancy so that Connor could grow and develop as much as possible. My husband, Reid went home and did research on the internet and did not share the bleak results of the few outdated studies on infants with Cloverleaf Skull. Approximately 4 days later, Connor couldn't wait any longer, and was delivered by c-section. I remember feeling elated when I saw him, and then scared when I could not hold him as he was wheeled away to the NICU at the Children's Hospital adjacent by an underground tunnel. While his initial apgar scores were 8 & 9, it quickly became evident that he couldn't breath through his nasal passages. Within 4 days he had a Tracheostomy and began breathing more comfortably. I remember crying together when a nurse told us we wouldn't get to hear most sounds babies make (including cries), due to the trach. She taped his cries for us on a cassette tape before they put the trach in.

At 2 weeks he had severe breathing problems and a VP Shunt was placed to help drain excess fluid from his brain. The results looked promising, however a week later he began having problems again resulting in a blood transfusion and then a strip craniotomy. I'll never forget having him baptized in the NICU prior to the craniotomy. His prognosis was not good, but even just before surgery when he looked very ill, his eyes showed such awareness and encouragement. After the craniotomy, there was a huge difference in his appearance (his head rounded), and in his whole being. He totally connected with us and his surroundings and began making more active movements and even smiles!. Connor remained in the NICU for 8 weeks. Most of it is a blur of sound, most notably medical equipment alarms, dimmed light, and trying to get Connor first to breastfeed, then to drink from a bottle. Thankfully we had a great speech pathologist in the NICU who started Connor wearing a passy-muir valve over his trach at intervals throughout the day. This allowed him to make beautiful baby sounds and today allows him to speak more clearly in sentences!! Connor was sent home with monitors and we were terrified with every beep. Thankfully we had sporadic nursing help to give us reassurance.

Sixteen days after being discharged from the NICU a follow-up at the Neurosurgeons office revealed that Connor's VP shunt wasn't working properly. He had to go back into the hospital for several days to have it "revised". Thankfully we were home together in time for Connor’s first Christmas. Connor’s big brother Reid (just 2 ½ years old at the time) was particularly happy to have him home from the hospital. While the introduction of a new sibling is always somewhat traumatic, it is especially challenging for the sibling of a special needs child.

2002 (top)

2002 is a blur of doctor visits and hospitalizations. In January it became evident that Connor was aspirating the breast milk we were feeding him via bottle (we would suction it out of his trach). A "swallow study" at the hospital confirmed this and Connor had to have a gastrostomy tube (g-tube) placed and could no longer drink via mouth. This was heart breaking. A Pediatrician appt. soon revealed a left & right hernia, which led to another hospitalization that same month for repair. In February, the g-tube was replaced with a more permanent "MIC-KEY button" for feeding directly into the stomach.

In April, Connor began having trouble keeping his oxygen saturation level up, a sign that the brain stem was under pressure. This resulted in Connor's first Chiari decompression surgery and a lengthy hospital stay. When things did not improve as much as they had hoped, the Craniofacial & Neurosurgeons decided Connor's brain was in need of more space again. A second craniotomy was performed, this one expanded the back of Connor's skull. Connor did very well for several months after this. However, the Craniofacial surgeon indicated he did not think he could operate on Connor again because it was very difficult to close the scalp due to the scar tissue that already developed from the first craniotomy. When asked for other recommendations, we were given a list of 3 top Craniofacial Centers, with NYU being recommended most highly. In July I traveled to NYU to meet the renowned Dr. Joseph McCarthy. I made an appt. for Connor to be seen by their Craniofacial team, but Connor's medical status became very unstable and we had to repeatedly reschedule his appt. there.

In October things spiraled downward. Connor began having cyanotic episodes. He would suddenly stop breathing and need to be resuscitated. Reid and I became expert at using our oxygen tanks and ambu bag. He spent over a week at our local children's hospital. During this time he repeatedly stopped breathing. The Doctors in FL & NY worked with us and our insurance company (who I had been talking to for weeks about how to fly Connor up to NY). My husband flew with Connor to NJ in an air ambulance. He was transported to NYU in an ambulance and "little" Reid and I flew separately and met them at the hospital. This is how we came to meet most of the Craniofacial Team at NYU!

Shortly after arriving at NYU Medical Center, Connor got a severe blood infection which forced them to postpone surgery until December. After several weeks, they were able to do another posterior craniotomy. Several days post op, a complication arose from this requiring them to remove part of the resorbable plate that was placed in the back of Connor's head, however, he was released and we remained in a hospital apartment for roughly a week after surgery before we could head back home to Florida. We spent 6 weeks in NY during this trip and returned home Christmas Eve. We were truly exhausted, but relieved that Connor was doing well. He had learned to sit up during his stay in NY and was beginning to use sign language and saying a few words.

We also realized he had already now had 11 surgeries in just over 12 months of life.

2003 (top)

Connor had P/E tubes placed in his ears in February to relieve pressure.

Then in April, Connor began experiencing severe respiratory difficulties, leading to a 2 month hospital stay. When we arrived at the hospital, he was placed on a ventilator. MRI pictures showed Connor’s brainstem severely compressed again - bone had actually grown back from the last decompression and was pressing on the brainstem and there was a syrinx (cyst) now. Some of the risks of brainstem surgery include paralysis, death from blood loss, and blindness (a concern because the surgery is face down and places a great deal of pressure on the eyes, which are proptosed – or bulged out already in Connor’s case).

The first attemped decompression surgery had to be aborted after Connor’s blood pressure could not be maintained. This was terrifying. Connor was brought back to the ICU and we had to wait another 3 weeks before they could attempt another decompression. During the second attempt, they successfully removed much of the bone and hoped this would result in the syrinx shrinking. The 3 Neurosurgeons present also decided to trim Connor’s cerebellar tonsils. When the syrinx did not shrink, they went in for a third time several weeks later and placed a shunt into the spinal area to drain the syrinx (this shunt remains there today). After several days of recovery Connor came off the ventilator. However, a sleep study later revealed that due to central apnea, he would need to be back on a ventilator when he sleeps.

In August of this year (at 22 months old) Connor began to crawl! In 2003 & 2004, he received therapy 7 times a week at home for Speech, Physical, Occupational, & Play.

2004 (top)

In January, Connor began to walk (27 months)! It took a great deal of effort and he was very wobbly, but he was determined! In February Connor came down with a nasty virus called Adeno that required a week-long hospital stay.

In March a swallow study showed he was swallowing properly and could begin a Feeding Program to teach him to eat by mouth. So in April, he began an intensive all-day program that lasted 4 weeks to introduce him to pureed foods. He did very well and left the program eating pureed and some minimally textured foods, with only 1 tube feeding per day. We also learned from this program that Connor is allergic to eggs and peanuts after he had to be taken to the for severe allergic reactions.

At the end of the following month, we noticed that Connor seemed to be again aspirating his food. In addition to this, he suddenly needed more ventilator support and began stumbling more when he was walking. Sadly, we had to go back to all g-tube feedings.

After consulting with local and NY specialists, it was agreed that his brain was again in need of more space.

In August Connor began preschool at a normal preschool! Connor’s local Cardiologist, Dr. Martinez also found that Connor’s Atrial Septal Defect (ASD) appeared to have closed on its own. Yeah!

At the end of September, we flew back up to NYU. Because of the past difficulties closing Connor’s scalp due to scar tissue, craniotomies could no longer be performed. A method called distraction would have to be used. At 2 years of age, the surgeons felt he was too young to have a complete frontal distraction, although one would be needed in the future. They decided it was time to pull forward the front of Connor’s head using a Rigid External Device (RED, we called it a halo). The surgeons separated the front of the skull from the back and placed hardware both inside and out to anchor the RED. After a week, they taught us how to slowly advance the front of Connor’s skull using a special tool (that resembles a screw driver).

We remained in NYC a total of 5 weeks before completing the advancement, which resulted in 16 mm being added to Connor’s forehead. He immediately began walking better and needed less ventilator support while he slept, even with the large halo on his head. After celebrating his 3rd birthday in NYC, we returned home to Florida with the halo on. Ten days later we had to return when the halo became unstable and had to be adjusted on his head. We flew back home again and the halo lasted another 3 ½ weeks before a severe staph infection formed around the screws on the sides of Connor’s head, leading to more instability in the device and its removal by a local surgeon. We returned to NY once again to have the internal plates and screws removed just before New Years Eve.

My husband and I agreed for Connor’s safety, that when a future halo was placed, we would remain in NYC until it was surgically removed.

2005 (top)

Connor returned to preschool and we followed-up frequently with a local surgeon who assisted us with healing the surgical wounds on Connor’s head. Connor receives therapy 12x/week (6x in the public school system and 6x privately). He is bright, energetic and mischievous. Although he is delayed with some fine and gross motor skills, and sometimes can’t get out the whole song or sentence loudly due to the trach, he is cognitively appropriate for his age!

In 2005, Connor experienced many trach infections and a 10-day hospitalization for pneumonia & RSV. He also had his first seizure in August and is now on medication to prevent them. Thankfully, his only surgery was the replacement of his ear P/E tubes!

In November we returned to NYU to meet with the Craniofacial team and discuss his next surgery. They determined it would be a monoblock (mid-face) distraction. They will this time seek to advance his forehead, orbits (to protect the eyes), nasal passages (to finally open them), and upper jaw (this will help with his tongue placement – and hopefully someday chewing). Moving the front of the skull forward will also give the brain more space. They have scheduled this surgery for May 24, 2006.

2006 (top)

In April we went to NYU Medical Center to meet with the team Orthodontists, Doctors Grayson and Garfinkel to complete x-rays and make moldings of Connor’s teeth. We also met with the team Opthalmologist, Dr. Steele who prescribed eye glasses for Connor’s vision (poor in the left eye).

It was also in April that Connor began experiencing more frequent, sudden severe pain in the back of his head. We soon learned that these were “Chiari headaches”. They were so severe that he would cry, break out into a sweat and sometimes release his bowels. However they lasted no more than 2 – 10minutes. Connor seeks relief from these by throwing his head back which we were told by his local Neurosurgeon, can help with CSF flow. We also give him Tylenol via g-tube, but we are not sure how helpful this is because of the time it takes the body to absorb the medicine.

After much difficulty finding an apt. to sublet, getting our flights and Connor’s airplane oxygen coordinated, as well as oxygen coordinated with companies in NJ and NY, and medical supplies flown from our Pediatric Medical Supply company in FL, we arrived in NYC several days before the scheduled surgery in May. While we went through most of our savings during this trip, things would have been unimaginably worse without the help of prayer, care packages, food and gift cards from friends and family. A local foundation also allowed individuals to make donations in Connor’s name which we could use toward rent on the apartment. This was a huge help and we remain grateful to all those who helped us so much, including several family members and friends who stayed with us when my husband had to go on business trips (he worked from the apt. most of the time we were in NYC!).

Prior to surgery, Connor met with Dr. Miles at the NYU Epilepsy Center where an EEG was performed to see if the severe head pain was actually seizure activity. This was ruled out, however his EEG shows unusual/abnormal brain activity as it has in the past. They indicated this may be a result of scar tissue.

After meeting with team Neurosurgeon, Dr. Weiner and lead Craniofacial Surgeon, Dr. McCarthy, we all waited with Connor (including his big brother, who adores him). At NYU, they allow a parent to don surgical garb to carry the child into the actual Operating Room. Holding your child while he is being put under anesthesia (and who is NOT happy about it) is one of the my most heart wrenching experiences. I can honestly say it doesn’t get any easier, even though this is Connor’s 23rd surgery.

Connor’s surgery to place the Rigid External Device (R.E.D.) took over 10 hours. Connor required 3 blood transfusions, and we learned from the anesthesiologist that they had such a hard time finding veins/arteries that they had to place IV lines in both his neck and his foot. He also had a pressure bandage on his leg where they accidentally cut into an artery there. As with the previous placement of the R.E.D., Connor’s skull was separated from side to side and they indicated they also removed and reshaped most of the bone on his forehead before they attached the upper part of the R.E.D.. There were also wires under the front of Connor’s skull which attached to the special mouth piece made by the Orthodontist.

When Connor came out of surgery he was very swollen, but he was awake and alert. When he saw us, he immediately sat up! The swelling and bleeding got worse over the next 48 hours, and we stayed at his side around the clock as we always do when he is in the hospital. Even after this major cranial surgery, he insisted on sitting up to urinate in the bedpan, since potty training was a skill he had mastered back at home! Just 3 days post surgery, he was sent “home” with us to the apartment we rented across the street from the hospital. After a week we were shown how to advance the R.E.D. in two places using two special tools to advance Connor’s face in two places at 1 mm a day. Thus began what turned into an 11 ½ week stay in New York City.

While living in an apt. in NYC lacks many of the conveniences of home (ease of grocery shopping and laundry, and high expense for everything in between), the children loved the adventure of exploring someplace new and even watching the traffic, from our 9th floor apt. and gazing at the Empire State Building from the 23rd floor roof at night.

Because of the large R.E.D. attached to Connor’s head, we had to be very careful. In the city, he was pushed around in a stroller with his medical equipment (and if we had to go somewhere requiring a taxi, he was strapped in for the frightening trip!). Ofcourse Connor and his brother’s favorite mode of transportation was the subway, which was truly challenging with a double stroller full of heavy equipment! I couldn’t begin to recount how many wonderful New Yorkers helped us up and down stairs and with directions!

While we met with the Surgeons twice a week to make adjustments to the R.E.D., we also took the opportunity visit some child-friendly sites. The boys particularly enjoyed the USS Intrepid, ferries to the Statue of Liberty and Staten Island Children’s Museum, crossing the Brooklyn Bridge and going to the NY Transit Museum, Grand Central and the Museum of Natural History.

During the stay in NY, Connor also required two R.E.D. adjustments under anesthesia at the hospital. After 10 weeks, the R.E.D. was removed with no anesthesia when Connor developed an eye infection. This was followed by surgery on August 3rd to remove the internal wires and screws from Connor’s skull. A Canthropexy was also performed on each eye to lessen the downward turn of the eyes that is caused by using a R.E.D. to advance the mid-face.

Connor was so happy to have the halo off, that he repeatedly said “My halo is off” with a BIG GRIN on his face every day for the next 2 months! The surgeons were pleased with the amount of extra “brain room” the mid-face advancement gave Connor, estimating almost 20% more intracranial volume. It took us a while to get used to Connor’s “new look”. While the past craniotomies made slight changes in his appearance, the mid-face advancement made a dramatic impact. Dr. McCarthy described the result as giving the patient a less dysmorphic, more natural look. It also achieved the goals of providing protection to Connor’s eyes, opening his nasal passages and advancing his upper jaw which gave his tongue better placement. We are grateful to Dr. McCarthy and his team for what they’ve done for Connor.

We met with the NYU Craniofacial team just before returning home to FL. They indicated that Connor will likely need another mid-face advancement in the future, and most definitely more advancement of the upper jaw and much Orthodontia work. They were hopeful that Sleep and Swallow studies in Florida would show improvement in those areas.

When we returned home, Connor began Pre-Kindergarten (accompanied by wonderful nurse Pat 4 days a week and me one day a week) and a Computer Tots class. He did beautifully. We also restarted his Speech, Physical and Occupational Therapy, but cut them down to 5 times a week as he was pretty tired out! He also had a brain and 3-d nasal CT scan. Connor had repeated colds during the fall and into winter, so his MRI, Sleep and Swallow studies were repeatedly postponed. In November, Connor had surgery performed by his ENT to remove his Adenoids.

2007 (top)

In January, the several weeks that Connor didn’t have a cold finally coincided with an MRI appointment. The MRI revealed the added Cranial volume and showed that the Chiari remained unchanged and was stable. We also learned at this time that the Mediport in Connor’s chest was not working properly, and the General Surgeon, Neurosurgeon and Pulmonologist concurred that Connor may not need it anymore. It was surgically removed on January 5.

In February, Connor had the Sleep Study at our local hospital, All Children’s Hospital (Connor’s 7th). During the Sleep Study they apply probes to Connor’s head and chest and see how he does off the ventilator while he sleeps. We were sad that after only 1 hour Connor had to be placed on the ventilator because his carbon dioxide level reached 60 (35 – 45 is good). This indicated that his breaths while sleeping remain shallow and that he still has central apnea. They also recommended Connor get cuffed trachs. This type of trach has a cuff hanging from it that we now fill with water when Connor falls asleep. This allows a balloon to form around the trach which keeps air from the ventilator from leaking out his mouth and nose when he sleeps (which we learned was the reason his ventilator low pressure alarms were going off so frequently). The cuffed trach doesn’t allow Connor to speak (air doesn’t move over the vocal chords now), so he has learned how to get our attention when he needs us at night by making the ventilator high pressure alarms go off!). We deflate the cuff when he wakes up in the morning so he can speak. Connor’s next Sleep Study is scheduled for August.

In March, April & May Connor played on a Miracle League baseball team with his best pal from preschool, several friends from All Children’s Hospital and many new friends. He also learned how to ride a bike with training wheels!

In May Connor had a Swallow Study which revealed that Connor continues to micro aspirate everything he was given during the study (formula with various amounts of Thick-it), so would need to continue with 100% g-tube feedings. Connor is now scheduled to have a Bronchoscopy in August to examine his vocal chords, trachea, epiglottis, etc… to see if there is an anatomical reason for his difficulty in swallowing. If this is not the case, Speech Therapy has approached us regarding a program using electrical stimulation therapy on the swallowing muscles.

Later in May, Connor developed pneumonia and was hospitalized for a week because he needed intravenous antibiotics to treat pseudomonis. After several days of IV meds and great difficulty finding good veins, Connor had to have shots to complete the course of antibiotics. He was such a trooper as he was so ready to do to anything to get out of the hospital!

Connor’s Audiology exam showed that he is experiencing mild conductive hearing loss again, and that he has a perforation in his right ear drum. Connor will have new P/E tubes placed in his ears in August when he has the Bronchoscopy.

While the many tests so far this year have revealed that Connor still needs to have a trach due to his need for ventilator support at night and that he still needs all his nourishment through his feeding tube, we are so thankful for how well he is doing overall and how far he has come after 28 surgeries. His fun-loving spirit, determination and ability have inspired not only his doctors and therapists, but also his teachers, classmates, and our family and friends.

We know there will be future trips to NYU Medical Center and possibly other facilities which specialize in Chiari malformations, sleep and swallow disorders. Our goal has always been to provide Connor with the best possible quality of life and do our best to decrease his need for medical equipment, supplies and constant intervention. In the meantime, a new chapter begins in our adventure!

After the medical procedures in August, Connor will begin Kindergarten where he will be fully included in a regular classroom (and accompanied by a nurse).