For the first time in years, 14-year-old Allison can breathe safely without a cumbersome mechanical ventilator by her side – thanks to a diaphragm pacemaker she received in a four-hour minimally invasive surgery at Children's. It was the first time the team at Children's implanted the “breathing” pacemaker without having to open the chest cavity.
The surgery was performed by Marleta Reynolds, MD, head of Department of Surgery and Don Shaul, MD, a colleague from Children’s Hospital Los Angeles (CHLA) who pioneered this minimally invasive technique in children.
Children’s and CHLA are now the only programs in the world that implant breathing pacemakers using only a few tiny incisions, resulting in less pain, minimal scarring and faster recovery time for patients like Allison. She was able to return home in three days.
Congenital Central Hypoventilation Syndrome
Allison has a rare disorder called congenital central hypoventilation syndrome (CCHS), a genetic condition in which the brain fails to properly control breathing during sleep and in most cases, during wakefulness. Since birth, Allison has been symptomatic, but she did not consistently receive mechanical ventilation until 7 months of age, and even then only with mask ventilation. Her condition worsened at age 10 when it became apparent that she needed the ventilator during the day as well as while sleeping.
Now, the implanted pacemaker allows her the freedom to participate in regular activities without being attached to a mechanical ventilator.
Center for Autonomic Medicine in Pediatrics
“Although we have long used diaphragm pacing in children with CCHS, the implantation technique required open chest surgery with four reasonably large incisions,” says Debra Weese-Mayer, MD, head of the Center for Autonomic Medicine in Pediatrics (CAMP) at Children's, who has treated Allison the past four years. “The thoracoscopic implantation with small incisions and expedited recovery makes it much easier on the patient. We plan to use it for ambulatory children with CCHS who require continuous ventilatory support.”
Weese-Mayer has gained international recognition for the clinical care and research of children with CCHS and other abnormalities in systems that regulate breathing, heart rate and other functions that the body does on its own.
“In addition to children with CCHS, some patients with high spinal cord injuries could benefit from getting a breathing pacemaker implanted through the minimally invasive technique,” says Reynolds. “This is a valuable expansion of our program in advanced minimally invasive surgery.”
Allison’s New Life
“Before diaphragm pacing Allison had to be on the mechanical ventilator 19 to 24 hours a day,” says Allison’s mother, Kim Nichols. “Now she only needs to wear a small, fashionable transmitter in a handbag and can do what she loves more easily, like dancing and playing the violin in the orchestra. This has changed my daughter’s life.”
For Allison, she is excited to have the chance to live more like other people. “I really hope this surgery can help other people with CCHS. It has made a huge difference in my life and I would like to thank all my doctors and the CAMP team,” she says.