The University of Illinois Hospital & Health Sciences System is one of only a dozen hospitals nationwide to implant the NeuRx Diaphragm Pacing System in a patient with amyotrophic lateral sclerosis (ALS).
Angela Thompson, 41, of Chicago, had surgery to implant the device on July 27.
The device was recently approved by the U.S. Food and Drug Administration and may help some patients with ALS (also known as Lou Gehrig's disease) to live longer without a mechanical ventilator.
Most ALS patients over the course of their disease develop reduced lung function or too-shallow breathing that requires them to have a tracheostomy or mechanical ventilator support, says Dr. Malek Massad, professor and head of cardiothoracic surgery at UIC.
Clinical trials of the device show that it may help people with ALS and chronic hypoventilation breathe and sleep better and delay the need for a ventilator for up to 18 months.
"It works similar to a heart pacemaker," said Dr. Khaled Abdelhady, assistant professor of cardiothoracic surgery at UIC, who participated in early development of the device and led the surgical team that operated on Thompson. "The device helps patients breathe easier by conditioning the diaphragm muscle through electrical stimulation."
ALS is a rapidly progressing, incurable and fatal neuromuscular disease characterized by progressive muscle weakness that results in paralysis. As the phrenic nerve to the diaphragm muscle fails, patients lose the ability to breathe without ventilator support.
Approximately 30,000 people in the U.S. live with ALS.
"I will never give up," said Thompson, who was diagnosed in 2010 and is cared for by Dr. Julie Rowin, associate professor of neurology and rehabilitation at UIC and director of the ALS Association Clinic and MDA/ALS Center at UI Hospital. Thompson said she hopes other ALS patients will also benefit from the device.
The device, which is implanted using minimally invasive laparoscopic techniques, provides electric stimulation to the muscle and nerves in the diaphragm. When stimulated, the diaphragm contracts, conditioning the muscle and improving resistance to fatigue under normal exertion.
Surgeons make four small incisions in the abdomen and insert a laparoscope to view the diaphragm and emplace small electrodes. The electrodes are connected by wires under the skin to a small, external, battery-powered pulse generator that stimulates the diaphragm.
When the patient awakes after surgery, the device is programmed to allow an effective yet comfortable breath. Patients start with three 30-minute sessions daily. As the disease progresses, the conditioning time is increased.
The NeuRx device was approved by the FDA last year under a Humanitarian Device Exemption (HDE), a designation given to medical devices that demonstrate safety and probable benefit for rare diseases. The device received the designation following a multi-center clinical trial that enrolled 106 patients and treated 86 for chronic hypoventilation.
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