Diaphragm Pacing System for CCHS

The Diaphragm Pacing System manufactured by Avery Biomedical Devices is a device used to provide respiratory support for patients with chronic respiratory insufficiency whose diaphragm, lungs, and phrenic nerves have residual function. The device delivers electrical impulses to the hemidiaphragms in order to restore breathing function. It is used in patients with Congenital Central Hypoventilation Syndrome (CCHS) in order to restore regular breathing function.

About CCHS and the Diaphragm Pacing System

Congenital Central Hypoventilation Syndrome (CCHS, also historically known as Ondine’s Syndrome) is a congenital form of Central Sleep Apnea, which studies have shown to be caused by a mutation of the PHOX2b gene.

CCHS is characterized by adequate ventilation while the patient is awake and by hypoventilation with shallow breathing during sleep. More severely affected patients hypoventilate when both awake and asleep. With CCHS, this is caused by the brain not utilizing the phrenic nerves (the neurological pathways between the brain and each hemidiaphragm, originating at the C3-C5 vertebrae) in a consistent manner. While this may cause a major difficulty in patients with CCHS, there may be an option to facilitate regular breathing with a diaphragm pacing system.

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How a Diaphragm Pacing System Works

A diaphragm pacing system consists of surgically implanted receivers and electrodes and an external transmitter with antennas which are worn directly over the implanted receivers.

The external transmitter and antennas send radiofrequency energy to the implanted receivers just under the skin. The receivers then convert the radio waves into stimulating pulses.

These pulses are then sent down the electrodes to the phrenic nerves, causing the diaphragms to contract. This contraction causes inhalation of air. When the pulses stop, the diaphragms relax and exhalation occurs. Repetition of this series of pulses produces a normal breathing pattern.

Who Is a Candidate for the Diaphragm Pacing System?

A diaphragm pacing system can provide ventilatory support for patients with chronic respiratory insufficiency whose diaphragm, lungs, and phrenic nerves have residual function. Typically, these patients have high spinal cord injuries, central sleep apnea or other central neurological disorders or a paralyzed diaphragm.

Ondine, a water nymph from German mythology, cursed her unfaithful husband “As long as you are awake, you shall have your breath, but should you ever fall asleep, then that breath will be taken from you and you will die!” From this ancient myth comes the name “Ondine’s Syndrome,” still used in certain parts of the world as a name for CCHS due to the difficulty of breathing during sleep.

Advantages over Mechanical Ventilation

Diaphragm Pacing Systems provide many advantages for CCHS patients over mechanical ventilation (Positive Pressure Ventilation, or PPV). Some advantages include:

  • 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

For More Information

The Diaphragm Pacing System may be an excellent option for patients with CCHS to improve their quality of life and decrease difficulties caused as a result of care for CCHS. 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.

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