Diaphragm pacing is a unique treatment option to help care for children with congenital central hypoventilation syndrome (CCHS), a rare genetic disorder of the autonomic nervous system. Children’s Healthcare of Atlanta is one of only a few pediatric hospitals in the country to have a program and team dedicated to caring for children with CCHS.
What is congenital central hypoventilation syndrome (CCHS)?
Children with CCHS require lifelong ventilator support because they are unable to control their breathing. The severity of CCHS can vary in children. Some kids require continuous ventilator support, while others only need ventilator support while they sleep.
Home assisted ventilation options include:
- Positive pressure ventilation through a tracheostomy
- Noninvasive positive pressure ventilation
- Diaphragm pacing using phrenic nerve stimulation
What is a diaphragm pacing system?
A diaphragm pacing system is a lightweight, battery-powered electronic device that supports breathing, possibly eliminating the need for a traditional ventilator either part time or full time. It stimulates diaphragm contraction through electrodes that are surgically placed on the phrenic nerves in the neck. The phrenic nerve electrodes are stimulated by a small battery-operated control box (transmitter) that is worn outside the body. When the phrenic nerve electrodes are stimulated by the transmitter, air flows into the lungs through the contraction of the diaphragm.
What are the benefits of diaphragm pacing?
Diaphragm pacing allows children who are dependent on a traditional ventilator full time to be free of positive-pressure ventilation during the day, allowing them mobility. Since the diaphragm pacer device is small, portable and does not require any tubes, it allows children to participate in school and other activities without being attached to the ventilator circuit.
For children who are ventilator dependent only while they sleep, diaphragm pacing may allow removal of their tracheostomy tube.
Children's Diaphragm Pacing Program requires the collaboration of many pediatric specialists. Our team includes the following providers:
- Anesthesiologist: Humphrey Lam, MD
- Cardiologist: Chad Mao, MD
- Critical Care Medicine Doctor: Pradip Kamat, MD
- General and Thoracic Surgeon: Matthew Clifton, MD
- Geneticist: Rossana Sanchez Russo, MD
- Otolaryngologist: April Landry, MD
- Pulmonologist: Ajay Kasi, MD
- Respiratory Therapist: Tracy Edwards, RRT; Walter Reeder, BS, RRT
- Sleep Medicine Specialist: Roberta M. Leu, MD