ALS, also known as Lou Gehrig’s Disease, is a progressive neuro-degenerative disease that affects nerve cells in the brain and spinal cord.
Sooner or later, it will attack every muscle in a patient's body, making it impossible to move or even breathe.
Now, a new machine is helping people who suffer from ALS breathe easier.
Mary Pat Murray has lived with ALS for more than four years. Checkups are not just routine for her; they can be life-saving.
“Today, I am really clogged up, which is why my vice is all kind of whacky," said Murray.
Traditionally, patients like Murray would use a bi-pap machine to help her breathe. She would wear a mask tethered to a ventilator. It would force air into Mary’s lungs.
But now, she is the first ALS patient at the Ohio State University Wexner Medical Center to be implanted with a diaphragmatic pacing system to help her strengthen her muscles.
"It feels like a really hard hiccup at first and then eventually you get used to it," said Murray.
The external device sends electrical signals to the nerve that controls the diaphragm, replacing signals normally sent by the brain telling it to expand and retract.
"All she needs to do when she is ready at night is to turn that on and it'll begin to, ah, help to contract the diaphragm as she's breathing," said Stephen J. Kolb, neurologist at Ohio State.
The DPS conditions the muscle while Murray sleeps.
"This gives us another tool in our tool belt where we can maybe engage the motor-neurons in the diaphragm; maybe allow the diaphragm to maintain strength longer in ALS, and improve quality of life and lifespan, so it's very exciting," said Kolb.
In just six months, Murray says she has felt a difference.
"I can carry on a conversation,” said Murray.” I can eat. I can drink."
She is thankful for the technology that allows her to do what most of us take for granted.
There are 30,000 Americans living with ALS right now.
Next year the device will be part of a multi-clinic study to determine if DPS actually helps ALS patients live longer in addition to breathing easier.
TOPIC: ALS: TAKE A DEEP BREATH
REPORT: MB #3711
BACKGROUND: Amyotrophic lateral disease (ALS) is a neurodegenerative disease that affects nerve cells in the spinal cord and brain. Often known as Lou Gehrig's disease, this deadly disease affects the motor neurons, which is related to the brain's ability to control muscle movement. ALS usually leads to involuntary muscle action, leaving patients paralyzed during late stages of the disease. (Source: http://www.alsa.org/about-als/what-is-als.html and http://www.mayoclinic.com/health/amyotrophic-lateral-sclerosis/DS00359)
SYMPTOMS: Early signs of ALS include trouble with speech, swallowing, muscle weakness, muscle cramps, and eventually trouble breathing. The disease will usually begin in the hands, feet, or limbs; then, it will spread to other regions of the body. Once the disease begins to spread, the muscles weaken until they become paralyzed. (Source: http://www.mayoclinic.com/health/amyotrophic-lateral-sclerosis/DS00359/DSECTION=symptoms)
TREATMENT: There are various forms of treatment available for ALS patients, depending on how severe the patient's symptoms are. There is not a cure for this disease, but medicine, physical therapy, occupational therapy, and speech therapy can aid in the relief of symptoms. Some medicines can slow down the disease and weaken symptoms, while therapy can provide exercises to improve mobility, muscle strength, and communication. (Source: http://www.mayoclinic.com/health/amyotrophic-lateral-sclerosis)
NEURX DIAPHRAGM PACING SYSTEM: The NeuRX Diaphragm Pacing System helps people breathe who have both ALS and breathing problems. It is implanted using a device with a camera at the end of it (laparoscope) in an outpatient procedure. The NeuRX Diaphragm Pacing System consists of four electrodes that are implanted in the breathing muscle (or the diaphragm), a fifth electrode is implanted under the skin, an electrode connector, which groups the five electrodes exiting the skin into a socket, a holder to hold the electrode connector in place on the skin, and a battery-powered stimulator box. The stimulator box sends the electrical signals to the diaphragm replacing the signals normally sent by the brain along the nerves. The signals cause the breathing muscle to contract, which helps to condition and exercise the muscle. A patient's diaphragm must show its ability to respond to stimulation. The device is used in patients 21 years of age or older and helps them breathe longer without the aid of a ventilator. (Source: http://www.fda.gov/MedicalDevices/ProductsandMedicalProcedures/DeviceApprovalsandClearances/Recently-ApprovedDevices/ucm278684.htm)
FOR MORE INFORMATION, PLEASE CONTACT:
Jerold Reynolds, PhD, RCP, RRT
Associate Professor of Neurology
The Ohio State University Wexner Medical Center