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March 14, 2005 – ALS Patient is First to Have “Reeve” Surgery Researchers Investigate Breathing Device for Lou Gehrig’s Disease

By: Eric Sandstrom

CLEVELAND: Doctors at University Hospitals of Cleveland performed minimally invasive surgery today (March 14) on a 40-year-old Lou Gehrig’s disease patient with the hope that early training of her diaphragm with the pacing system can delay her need for a respirator and improve her quality of life.

The Arkansas woman is the first patient with Lou Gehrig’s disease (also known as ALS) to undergo the procedure that actor Christopher Reeve—a quadriplegic—had two years ago when an electronic breathing device was implanted in his diaphragm.

This operation marks the beginning of a study by UHC surgeon Raymond Onders, MD; pulmonologist Robert Schilz Phd, DO, and neurologist Bashar Katirji, M.D.to determine whether electrically stimulating the diaphragm muscle with electrodes will maintain the muscle strength and decrease the rate of respiratory decline that ALS causes. The procedure has been performed successfully on 15 patients with spinal cord injuries, but never upon an ALS patient until now. This new study is designed to enroll ten ALS patients for the procedure.

An estimated 5,600 Americans are diagnosed every year with amyotrophic lateral sclerosis (ALS), a muscle disorder caused by a nerve dysfunction. Most ALS patients will eventually require a respirator when their diaphragm muscles weaken to the point of causing breathing problems. But respirators are expensive to maintain, increase the risk of infection, and restrict the patient’s independence—all issues that led Dr. Onders and a team of researchers to develop a diaphragmatic pacing device for patients who wished to breathe without respirators.

“The challenges ALS patients and spinal cord injury patients face are quite different but they share the need for a respirator in the end,” Dr. Onders says. “If our diaphragmatic pacing device performs for ALS patients as well as it does for those with spinal cord injuries, then the potential to expand their horizons has been realized,” Dr. Onders says. “We certainly hope ALS patients can achieve that benefit.”

Dr. Onders surgically implanted the device in Mr. Reeve in February 2003, eight years after the actor fell from a horse and was left paralyzed and dependent on a respirator. The breathing device developed in partnership with biomedical engineers at Case Western Reserve University, functioned successfully until Mr. Reeve died last year due to unrelated health problems.

Working through a small laparoscope in what is essentially an outpatient procedure, surgeons placed electrodes in Reeve’s diaphragm muscle. The electrodes are attached through wires under the skin to a small external battery pack that electrically stimulates the muscle and the phrenic nerves, causing the muscle to contract and air to enter the lungs. Diaphragm contraction accounts for most of the ventilation required for normal breathing. Because the surgical placement of the device is performed in an outpatient setting through minimally invasive techniques, risks and costs of this diaphragm pacing system are significantly less than standard procedures to electrically activate the diaphragm.

The standard technique requires surgeons to make large incisions in the chest (thoracotomy) to place electrodes in direct contact with the phrenic nerves. That procedure carries substantially more risk and requires a prolonged hospital stay. The standard procedure typically costs more than $100,000 (device plus surgery, hospitalization and follow-up), whereas the laparoscopic surgery and implantation of the new device could cost less than half that amount.

Dr. Onders, a surgeon at University Hospitals of Cleveland specializing in minimally invasive techniques and associate professor of surgery at CWRU, implanted the first such device in a patient five years ago. Since the implantation of that first device by Dr. Onders five years ago, biomedical engineers J. Thomas Mortimer, PhD, professor emeritus of biomedical engineering at CWRU, and Anthony R. Ignagni, project director and chief biomedical engineer, have improved the operation of the pacing device.

The investigational diaphragm pacing system, portions of which were patented by CWRU and Synapse Biomedical, is now being developed by Synapse Biomedical, Inc., of Oberlin, OH. Funding assistance for the spinal cord application was provided by the Food and Drug Administration, U.S Surgical Corporation, University Hospitals of Cleveland, the VA, and the National Center for Research Resources of the National Institutes of Health.

The ALS study is partially funded by the Winters family, Feintech Family Foundation, and the Elizabeth Severence Prentiss Foundation but is awaiting a full funding decision by the Orphan Product division of the FDA.