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ALS: Take A Deep Breath

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COLUMBUS, Ohio (Ivanhoe Newswire) - ALS, also known as Lou Gehrig's disease, is a progressive neurodegenerative 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, even breathe. Now, a new machine is helping people suffering 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 lifesaving.

Traditionally, patients like Mary would use a BiPAP machine to help her breathe. She'd wear a mask, tethered to a ventilator. It would force air into Mary's lungs.

Now, Mary is the first ALS patient at The Ohio State University 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," Mary told Ivanhoe.

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 help to contract the diaphragm as she's breathing," Stephen J. Kolb, MD, PhD, Co-Director, ALS/MND Clinic, Department of Neurology, The Ohio State University Wexner Medical Center, told Ivanhoe.

The DPS conditions the muscle while Mary 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," Dr. Kolb said.

In just six months, Mary says she's felt a difference.

"I can carry on a conversation. I can eat. I can drink," Mary explained.

She is thankful for the technology that allows her to do what most of us take for granted.

Thirty thousand Americans are 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.

RESEARCH SUMMARY

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)

INTERVIEW

Stephen Kolb, MD, PhD, Assistant Professor in the Department of Neurology at the Ohio State University Wexner Medical Center, talks about a new therapy for patients with amyotrophic lateral sclerosis (ALS).

What are the concerns of doctors who are not using this procedure and why aren't they?

Dr. Kolb: This is a relatively new procedure.  It's something that until recently was not available to patients with ALS.  There aren't any good studies that have been performed and so most people are waiting.

What exactly is ALS?

Dr. Kolb: ALS is short for amyotrophic lateral sclerosis. It is a disease of the motor neuron.  Motor neurons are those cells that connect the brain to muscles and allow us to move, but also to speak, breathe, and swallow. What happens in ALS is that there is a death and loss of these motor neurons leading to progressive weakness.  No one knows the cause of ALS, but it is a neurodegenerative disease; so in that sense it is in the same category as Alzheimer's, Parkinson, and Huntington's.   

Do most people know it as Lou Gehrig's disease? 

Dr. Kolb: Most people do know it as Lou Gehrig's disease in the United States. Also, sometimes people call it motor neuron disease, but it's a spectrum.  It's probably not all one disease, but we know it as ALS or Lou Gehrig's here in the U.S. 

Can you tell us about the diaphragmatic pacing system?

Dr. Kolb: As you progressively become weaker, you have more and more difficulty opening up the lungs to breathe. Ultimately because we are still not able to stop the progression of ALS, the way people die is usually with respiratory failure.  The current management for that has to do with making sure that the patient is more comfortable and able to breathe as much as possible without any invasive ventilation.  Normally, someone will come in with ALS, have some shortness of breath, and we would measure their breathing and then start them on noninvasive ventilator support. You may have heard of BIPAP or CPAP. Anyone who has had sleep apnea or any kind of problem breathing at night usually wears a facemask. That machine basically puffs a little bit of air into the lungs when someone goes to inhale and it assists them when they are weak.  So, we do that with ALS as well.  Unfortunately, as the disease continues to progress that kind of therapy becomes less and less effective. 

How do you know when is the right time to use this device?  

Dr. Kolb: There is no red flag that pops up on your bed post in the morning that says now is when you need it.  It's usually a decision that is made with the patient.  It depends on how much discomfort the patient is having with their shortness of breath and it is guided by objective

So, how would say it would work for Mary?

Dr. Kolb: She was the first person at the Ohio State University to have it implanted.  So, where the diaphragmatic pacing system comes in, is when a patient already has shortness of breath.  There is already some loss of the strength of their diaphragm. I think most people are familiar with cardiac pacing where there is an electrode that's placed into the muscle of the heart or a part of the heart that stimulates the electrical activity.  This is very similar to the idea where there is an external power source and an electrode that connects directly to the motor neuron just as it connects into the diaphragm.  By stimulating the motor neuron directly, it increases the contraction of the diaphragm and improves strength.

How effective has it been with Mary?  Have you noticed a change or a difference?  

Dr. Kolb: From my perspective, I'm used to seeing people progress at a certain rate as far as their shortness of breath goes and she seems to have plateaued a bit with the decrease in her respiratory function with the procedure. So, I would say that overall, we have had a positive outcome with her.  

Do you really see any risks or are we still waiting to see if there are any risks associated with the device?  

Dr. Kolb: Any time you have a surgical procedure, there are risks and we have some outstanding thoracic surgeons who are working with us to put the device in.  It is a laparoscopic procedure, meaning there is not a big open OR field. So, as far as that goes there is a risk, but it's an acceptable risk if it helps you breathe better.  Once it's in place, some of the risks that could occur are include an infection at the site, but infection would be true in any type of procedure where you have a wire sticking into your body somewhere.  Other than that, it seems to be very well tolerated.

Are you excited about this device?

Dr. Kolb: I am. I think that we are entering sort of a new era here in ALS research. We are becoming much better at trying to address problems before they happen and it's clear that that's really the secret to successful management. This gives us another tool in our tool belt where we can maybe engage the motor neurons in the diaphragm and allow the diaphragm to maintain strength longer in ALS and improve quality of life and life span.  So, it's very exciting.

You said it was available now for some patients, but when does the study actually begin?

Dr. Kolb: So, just to clarify any confusion, the FDA has made the diaphragmatic pacing system available to all patients with ALS under a humanitarian care sort of advice, even though there is no actual proof that this works or not.

FOR MORE INFORMATION, PLEASE CONTACT:

Jerold Reynolds, PhD, RCP, RRT
Associate Professor of Neurology
The Ohio State University Wexner Medical Center
Jerold.Reynolds@osumc.edu

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