Zoe Colquitt was born with congenital central hypoventilation syndrome (CCHS), also known as “Ondine’s Curse.”
CCHS is a neurological disorder that can affect multiple systems, but primarily impacts the respiration and causes patients to stop breathing during sleep, which is why Zoe, now 12, must use a ventilator 12 hours per day. But that doesn’t slow her down much. Zoe has a nickname – Mighty Z.
When Zoe was born, doctors suspected she had CCHS, but at the time, there wasn’t even a test to prove it. In 2003, French researchers found the genetic marker and developed a definitive test for the disease.
Because of her low respiratory drive, Zoe underwent a tracheotomy at 4 months and was finally able to come home from the hospital, though a ventilator was always nearby.
When she was 3 years old, Zoe had a surgical procedure to implant receivers and electrodes for a breathing pacemaker system that eventually allowed her to have the trach tube removed. The “pacers” stimulate the phrenic nerve that controls the diaphragm and prompts breathing. This gives her greater freedom and means she only needs the ventilator at night or when she’s sick.
She’s been treated at Children’s for the past four and a half years by teams in Pulmonology, Cardiology, Gastroenterology and Endocrinology, just to name a few. While her mom Shelley Colquitt says all provide excellent coordinated care, Zoe plays favorites. She likes Andrew Gelfand, M.D., in Pulmonology best.
More on CCHS
About 1 in 200,000 children are born with CCHS, making it a statistically rare or “orphan disease.” CCHS causes a gene mutation that affects the regulation of certain involuntary body functions including heart, rate, blood pressure, temperature regulation, bowel function and breathing.
But Zoe’s disease doesn’t slow her down much. Zoe lives up to her nickname, Mighty Z. She attends a mainstream private school, actually enjoys homework and loves music. She really loves music.
Zoe’s Love of Music
She started playing piano at age 9, and when her third grade class was asked to research a music composer, she was thrilled. But while her classmates chose Bach and Beethoven, Zoe chose Timothy Brown.
After all, he wrote her favorite song, at the time, Teddy Bear, Teddy Bear, a piece she was learning to play on the piano.
With a little internet research assistance from her mom, Zoe learned Brown lived near Dallas, and the family began communicating with him. A few emails and phone calls later, Brown found himself at Zoe’s school entertaining her class with a live performance of Teddy Bear, Teddy Bear. Brown has enjoyed a strong bond with the family ever since.
Composer Inspired by Children’s Patient: Part 2 shares more of Zoe’s journey, the song Timothy Brown wrote for Zoe and the legend behind “Ondine’s Curse.”