About ALS and Diaphragm Pacing Stimulation

• An estimated 5,600 Americans are diagnosed with ALS each year.
• ALS, amyotrophic lateral sclerosis also known as Lou Gehrig’s Disease is a debilitating motor neuron disease that weakens muscles and leads to eventual paralysis and death.
• There is no cure for the disease and most ALS patients die from respiratory failure.
• It is known as an orphan disease because it involves so few patients in a given year.
• ALS patients live an average of five years after onset. With progressive damage to motor neurons, they become unable to breathe without a ventilator which increases risk of infection, restricts patient’s independence and is expensive to maintain.
• ALS patients usually lose about one to three percent of their breathing ability each month.
• Diaphragm Pacing is a minimally invasive way to help preserve the diaphragm and allow patients to continue breathing using their own muscle as opposed to the positive pressure of mechanical ventilators. The NeuRx Pacer is being developed by Synapse Biomedical, Inc. (Oberlin, OH).
• The diaphragm pacing surgery implants electrodes to stimulate and strengthen the diaphragm muscles. The electrodes are connected through the skin to an external device that is programmed to allow for the best diaphragm movement for the patient. Patients who undergo the procedure are asked to use the device five times a day for 30 minutes at a time.
• The diaphragm pacing system has been able to allow over 95% of the spinal cord injured patients implanted to breathe without the use of a mechanical ventilator. Statistically the system appears to delay the need for a ventilator in ALS patients by 15 months.
• Positive pressure breathing assistance (also know as BiPap), is also used to help ALS patients breath but less than 20 percent of patients use it. During REM (rapid eye movement) sleep also known as dream sleep the only muscles working are the eyes and the diaphragm. When the diaphragm is weakened or is no longer under good natural control, patients cannot reach this cycle of sleep, leaving them without a truly restful good night of sleep.
• The only federally approved drug for ALS is Rilutek, which can increase life by about three to six months.
• Christopher Reeve underwent the diaphragm pacing procedure and was able to breathe on his own for many hours of the day. Of the 37 paralyzed patients that underwent this procedure, 97 percent are no longer dependant on their ventilators.
• Ventilators can help patients breathe and live longer but fewer than 10 percent of ALS patients elect to go on one.

Source: Dr. Ray Onders, Director of Minimally Invasive Surgery at University Hospital Case Medical Center in Cleveland, Ohio and Associate Professor of Surgery at Case Western Reserve University School of Medicine