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Children born with congenital central hypoventilation syndrome (CCHS) take shallow or slow breaths, especially when sleeping. As a result, oxygen levels drop too low and carbon monoxide levels become dangerously high. Diaphragm pacing is a specialized treatment that regulates your child’s breathing without the need for mechanical ventilation.
The CCHS and Diaphragm Pacing Program at Children’s Hospital Los Angeles is one of the oldest and largest programs in the U.S. We care for a high volume of children with CCHS every year. Our team pioneered a surgical approach for diaphragm pacing that reduces or eliminates the need for mechanical ventilation. This technique is in use worldwide today.
Diaphragm pacing and CCHS services are specialized pulmonology offerings at CHLA. U.S. News & World Report ranks our pediatric pulmonology and sleep medicine services among the country’s best.
Our CCHS and Diaphragm Pacing Program offers:
An estimated 1,200 people worldwide have CCHS. This rare disorder affects a child’s autonomic nervous system. This network of nerves controls involuntary (automatic) body functions like breathing, digestion and body temperature regulation.
Children with CCHS have a changed (mutated) PHOX2B gene. For most children, this gene mutation occurs for no known reason. In rare instances, a child inherits the changed gene from a parent.
The genetic change affects nerve signals to the brain that control how quickly and deeply your child breathes. Your child’s lungs and heart are healthy, but their brain doesn’t get the signals it needs to regulate breathing. There isn’t a cure for CCHS. However, expert medical care enables most children to grow and thrive into adulthood.
Children with CCHS may have other health issues, such as:
Most children show signs of low oxygen from CCHS—such as blue-tinted skin, fingernails or lips—soon after birth. Experts at our Division of Medical Genetics perform genetic testing (a specialized blood test) to check for the PHOX2B gene change that causes CCHS. You and other members of your family may also take this test. Our medical genetics team offers compassionate genetic testing, counseling and more.
After a CCHS diagnosis, your child may get additional tests to assess their overall health:
Diaphragm pacing is a type of breathing support (ventilation) that regulates your child’s breathing. It can replace or complement mechanical ventilation. Mechanical ventilation requires a ventilator system and tracheostomy (surgically created hole in the windpipe that connects to a ventilator via a tube).
Diaphragm pacing uses surgically implanted electrodes (tiny transmitters) and a small battery-powered diaphragm pacing system. The portable pacemaker sends electrical impulses to electrodes attached to the phrenic nerves, which are essential to breathing. The impulses cause the diaphragm muscles under each lung to expand during inhalation and contract more fully during exhalation.
We evaluate children 2 and older to determine if they’re good candidates for diaphragm pacing. Our general pediatric surgeons and neurosurgeons work together to perform this procedure, which can take up to five hours.
Our team pioneered a thoracoscopic approach, using a scope with a light and video camera to place electrodes. Unlike earlier methods, thoracoscopic surgery takes place through several small chest incisions instead of two large ones. It also doesn’t require spreading the ribs to reach the lungs. This approach causes less pain and scarring, allowing your child to heal faster.
During diaphragm pacing surgery, your child’s surgical team:
After diaphragm pacing surgery:
The diaphragm pacing device is small enough to fit into a backpack or fanny pack, making it easy for your child to be active at school, home and with friends.
Diaphragm pacing allows children with CCHS to:
Children with CCHS receive coordinated care from a team of dedicated medical specialists. In addition to pulmonologists and respiratory care practitioners, your child’s care team may include:
Other team members may include:
Our expert team of pulmonology and sleep medicine specialists diagnoses and manages all types of breathing, lung and sleep issues in children. Learn more about our leading-edge Pulmonology and Sleep Medicine services.
Pulmonology and sleep medicine experts at CHLA welcome new patients, referrals and second opinions. Please contact us: