October 31, 2005 – Breakthrough for Lou Gehrig's
More than 5,000 patients in the United States are diagnosed with amyotrophic lateral sclerosis (ALS) each year. ALS, often referred to as Lou Gehrig's disease, is a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to patients' death. When motor neurons die, the ability to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed. However, for the majority of people with the disease, their minds remain unaffected. Early symptoms of ALS often include increased muscle weakness, especially involving the arms and legs, speech, swallowing or breathing. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles start becoming smaller. Limbs begin to look "thinner" as muscle tissue atrophies.
CURRENT TREATMENTS: Although the cause of ALS is not completely understood, recent research has brought a wealth of understanding about the disease. Currently, there is no cure or treatment that reverses the disease. However, there is one FDA-approved drug called Rilutek that modestly slows the progression of ALS.
PRECIOUS BREATH: Patients with ALS progressively lose their ability to breathe. Raymond Onders, M.D., from the University Hospitals of Cleveland, says, "You use a lot of different muscles to breathe, but the diaphragm is the most important muscle – the diaphragm being the muscle right in the middle of our abdomen & when it contracts, it allows you to bring in air, and with Lou Gehrig's disease, it's a slow, progressive decline of your ability to breathe." To survive, most patients must be put on a ventilator at some point, but Dr. Onders says that can raise the risk of pneumonia. He says, "The mechanical ventilator has a lot of risks involved with it. People may choose not to have it because they don't want to have that risk, even though they are progressing. Also, it's extremely expensive. We know that the cost of a mechanical ventilator, along with the support people to watch that, is anywhere between $100,000 and $150,000 per year."
A NEW OPTION: Now, Dr. Onders may have a new solution for patients with ALS. It's called a diaphragm pacing system, and it's the same device that the late actor Christopher Reeve had implanted to avoid a mechanical ventilator. With special instruments, doctors place two electrodes on each diaphragm. An electronic device stimulates the muscles and nerves, allowing muscles to contract and air to enter the lungs. The procedure is performed in an outpatient setting. Dr. Onders says, "What we're trying to do in these patients is let them live longer without the need for that mechanical ventilator. Our data has shown that we have a simple operation where we're trying to stimulate their own muscle ... We have shown so far that we are able to use their own muscle and try to allow them through training just five times a day to maintain that mass and hopefully prevent the progression."
So far, the device has been used on three patients. It has actually improved forced vital capacity in two of those patients. Dr. Onders also says the device is much less expensive than a mechanical ventilator. The surgery and device cost about $15,000, and insurance has covered the cost for patients so far.