“ALS: Take a Deep Breath”
Stephen Kolb, MD, PhD
Stephen Kolb, MD, PhD, Assistant Professor in the Department of Neurology at the Ohio State
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.
This information is intended for additional research purposes only. It is not to be used as
a prescription or advice from Ivanhoe Broadcast News, Inc. or any medical professional
interviewed. Ivanhoe Broadcast News, Inc. assumes no responsibility for the depth or
accuracy of physician statements. Procedures or medicines apply to different people
and medical factors; always consult your physician on medical matters.
If you would like 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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