How Is CCHS Typically Treated?
Any treatment for CCHS should come at the direction and under the care of a licensed physician. Currently, there is no cure for CCHS nor drug therapy for treatment. Patients may manage the condition in conjunction with their doctor through the support of a breathing device. Methods may include:
- Positive pressure ventilation (PPV) via tracheostomy, which pumps air into the lungs through a tube surgically placed into the windpipe
- Bi-level positive airway pressure ventilation via masks or prongs, a less invasive alternative to the tracheostomy technique that is typically utilized in milder forms of the disorder
- Negative pressure ventilation (NPV), a less popular technique that creates pressure in the chest and abdomen to help inflate the lungs by wearing a special jacket or vest
- Diaphragm pacing, a minimally invasive process that stimulates the phrenic nerve enabling more natural breathing for the patient and may provide many benefits to the patient (in-context link will be used to benefits below). The Avery Diaphragm Pacemaker is such a device.
The Avery Breathing Pacemaker
The Avery Breathing Pacemaker is an FDA-approved diaphragm pacing device that promotes natural breathing in patients with CCHS—as well as several other conditions—24 hours per day to patients of all ages. With this device, electrodes are placed on the phrenic nerve in the neck or chest and connect to an external device that creates radio waves. These waves are transferred to the nerve to stimulate it, causing the diaphragm to contract as it would naturally.
This device offers several benefits to patients over mechanical ventilation (e.g., PPV) systems including:
- Lower risk of upper airway infections due to reduction in suctioning, elimination of external humidifier and ventilator circuits, and potential removal of tracheostomy tube in some patients where appropriate
- More natural breathing function that is physiologically more accurate and comfortable
- Compact design without bulky tubing and batteries, which allows for greatly increased mobility (some of our patients still enjoy swimming and horseback riding!)
- Superior sensory and related motor functions, including improved sense of smell, ease of eating and drinking, and normal breathing and speech patterns.
- Significant long-term cost savings – Diaphragm Pacing Systems typically pay for themselves in roughly two years, with patients saving an average of $20,000 per year thereafter
The Avery Breathing Pacemaker has been featured in several studies and has been drastically improving the lives of patients for over 50 years. It is the only diaphragm-pacing device with full pre-market approval from the USFDA and CE marking privileges under the European Active Implantable Medical Device Directive for adult and pediatric use. In addition, Avery Biomedical is currently partnered with the CCHS Network to help improve the lives of patients with the disease. The CCHS Network is a nonprofit dedicated to research and providing support to families living with CCHS.
For More Information
For more information, please call us at 631-864-1600, or click the “request info” button below and fill out the contact form. We would be happy to get you more information about how the diaphragm pacing system may help, and connect you with a physician familiar with the device in your area if needed. If you’re looking for a physician, you can find a list of physicians and medical centers in the United States here, or find a list of international representatives here.