How is Congenital Central Hypoventilation Syndrome Treated?
There is no cure for CCHS, but there are effective ways to manage it.
Treatment often involves helping the child breathe with a ventilator (a breathing machine), especially during sleep. For children who need breathing support all the time, a tracheostomy may be placed. A tracheostomy is a small opening made in the neck into the windpipe where a tube can be placed to help with breathing. Children can use portable ventilators to move around and live as normal a life as possible.
Some people may also be helped by a diaphragm pacemaker. This is a device that is surgically implanted to stimulate the diaphragm, the muscle that helps us breathe. It works by sending electrical signals to the diaphragm to help the diaphragm to contract and pull air into the lungs. Diaphragm pacemakers can allow some people with CCHS to breathe without a traditional ventilator during certain times, like when they are awake.
Children with heart rhythm problems may need a pacemaker, a small device implanted in the chest that helps keep the heartbeat steady. It works by sending electrical signals to the heart if it starts beating too slowly or irregularly. If your child needs a pacemaker, their cardiologist will manage the device and monitor it during follow-up visits.
Because CCHS can affect many systems of the body, children often receive care from a team of specialists. Depending on your child’s needs, regular checkups with this team will help monitor breathing, heart function, digestion, growth and development over time.
Doctors recommend avoiding activities that could make breathing more difficult or hazardous. These activities can lead to dangerously low oxygen levels (hypoxia). This means the body and brain aren’t getting enough oxygen to work properly. This can lead to confusion, fainting, seizures or damage to the brain and other organs.
These activities include:
- Swimming, since children with CCHS may not recognize when they are holding their breath too long underwater, which can increase the risk of drowning
- Playing breath-holding games or contests, because children with CCHS may not feel the natural urge to breathe and could pass out without warning
- Using alcohol, recreational drugs or certain prescription medicines that slow down breathing
Your child’s doctor will also provide specific safety tips based on your child’s needs. Following these recommendations can help your child stay active, healthy and doing the things they love.