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My topic today is respiratory therapeutics for neuromuscular
diseases, and as I was trying to put together this talk, I
was trying to decide, "Well, what is it that would be
of interest to patients, particularly to family members or
patients who have underlying neuromuscular diseases?"
So, I thought I would keep it a little bit simplistic because
I know that when I go to a physician, or a dentist, or some
other person where I usually feel uncomfortable, I like to
kind of decide, "What's going to be asked of me? How
am I going to be evaluated?" So, today I'm going to review
what it would be like for someone to come to our pulmonary
clinic: To evaluate the patient, what tests would I have to
perform to see what my lung function is like? And then, lastly,
I'd like to know something about the therapy, particularly
in patients who have neuromuscular disease who are developing
problems with breathing.
O.K., this first goes
over what I just mentioned: the evaluation of the patient.
If you were to come to the clinic, I would basically do what
most other physicians would do: I would take a history and
physical examination first. You say, "What are the questions
that might be asked of me?" Well, I would ask something
particularly about your background, what you've been doing,
let's say, for the last several years, maybe something about
your occupation. But specifically, from a respiratory standpoint,
I would ask about how you're breathing: Do you get short of
breath? Are there certain activities during the day that make
you short of breath? For example, if you were in a wheelchair,
is there something that occurs during the day that you feel
that you can't breath as well as if you're just sitting and
maybe not ambulating the and using the wheelchair in any way.
Other questions would be: Have you had any infections recently?
Are you taking any anti-biotics? Are your flu shots up to
date? (Very important particularly for patients who might
have some neuromuscular involvement of their respiratory muscles
to be able to prophylax themselves. That is, we want to prevent
patients from having respiratory problems so that they won't
have difficulties if they were to get a bioinfection. Have
you had your pneumonia shots? Other things might be something
about your sleep history. Frequently we can be breathing pretty
o.k. during the daytime, but as we go to bed at night, muscles
further relax, and sometimes you can have breathing difficulties
during the night. I might want to know from the family members
whether you snore, because a lot of times there is increased
airway resistance at night, and people who have certain neuromuscular
diseases might indeed be prone to have what we call 'sleep
apnea,' where the back of the throat collapses at night, and
you would have some difficulty breathing. So these are the
type of questions I would be asking of you. I would also ask
you something about your cough: Are you having any difficulty
swallowing your food? Are you able to clear your secretions?
As some of you may know, certain neuromuscular diseases, as
they progress, your cough becomes somewhat limited, so that
if you do get an infection, you may have difficulty clearing
your secretions, which may develop into an enormity.
Respiratory History and Physical Exam.
I then would probably
say, "O.K. Now we'll go ahead and do a physical exam.î
And the physical exam for the lungs doesn't necessarily just
include a listening to the lungs. We usually start at the
nose, because sometimes people might have problems with the
nose which could contribute to their breathing. So we take
some history: we look at the nose, we look at the back of
the throat to see if the tonsils are enlarged, we see if there
are any problems with the gag reflex (so we put that little
tongue blade back there), we even ask you to cough to see
how your cough mechanism is present. Other things: we'll listen
to your heart, and we'll also listen to your lungs. When we
listen to your lungs, we listen for abnormal sounds, perhaps
evidence that you've had some old problems with your lungs
or maybe have even had an infection.
Well, after we do the
history and physical exam, we usually request some baseline
lab studies, or tests. These tests are not done at every visit.
In fact, they may be done every couple of years. But specifically,
they would include a chest x-ray, perhaps, and some pulmonary
function tests. This is just an example of a chest x-ray,
and for some of you who have not seen what a chest x-ray looks
like, this is looking from the front out towards yourself.
So, this would be the right-side of your lung, and this would
be the left-side. This white blob right here is your heart,
and since air looks black on the chest x-ray film, this is
all the lung here, and these lines right here are your ribs.
The other important thing to look at here is the fact that
there is a muscle right here, which is the right hemi-diaphragm,
and this is the left hemi-diaphragm. And that's important
to know because that's the large muscle that's responsible
for most of our breathing. And as patients progress with their
neuromuscular disease, frequently they will have problems
with this muscle actually ventilating the lungs. So you're
hearing today, over and over things like 'ventilation' and
'hypoventilation' and 'a restrictive type of process,' and
basically, that's because as the muscles become weaker in
neuromuscular disease, particularly the muscles of respiration
such as the diaphragm, the patient has more and more difficulty
'ventilating.' (i.e. getting air in and out of the lungs)
It should also be mentioned that between these rib spaces,
although you can't see them here on the chest x-ray, are various
muscles called intercostal muscles that also function and
help promote breathing in individuals.
Perhaps I then might
say, 'O.K. Mr. or Mrs. Jones, what I would like to do today
also is some baseline pulmonary function tests. These are
tests that are usually in our main hospital at the UC Davis
Medical Center on the 5th floor. It takes about 40 to 45 minutes
to do, o.k.' And basically what we want to know is 'How is
the lung functioning?' So, we'll do some spirometry, where
you sit in front of a spirometer, take a deep breath in and
blow it out. O.K., blow all your air out so that we can get
an idea as to the vital capacity of your lungs. We'll do certain
tests to test the volumes of your lungs. As I mentioned previously,
patients who have neuromuscular diseases frequently are restricted.
That is, they have small lungs because they can't take their
breath in and get it out successfully, and so the lung volume
will give us some idea as to a baseline of how you're functioning.
And lastly, we have some inspiratory and expiratory pressures
that we do to get an idea specifically as to how that diaphragm,
that large muscle that I spoke to you about, how that specifically
works. And a MIP stands for a 'maximum inspiratory pressure,'
and a MEP stands for a 'maximum expiratory pressure.' So,
for example the MIP gives me an idea of how hard you can suck-in,
you can pull in, to ventilate your lungs, and the MEP gives
me an idea because on expiration you're blowing out as far
as you can to see how weak your cough is, to see how you can
expel air from your chest cavity. So, this is kind of an old
slide which basically shows a patient sitting in front of
one of the old water-scale spirometers. Nowadays we have everything
computerized, but basically, the same information is obtained.
The patient sits here, the technician is like the cheerleader,
and says, 'O.K., take a deep breath in and blow everything
out,' and this again gives us an idea as to lung function.
So, in this cartoon here, you would be sitting in front of
this machine with a mouthpiece on. You would be asked to take
a couple of normal breaths, then you take a deep breath in
and blow everything out: BLOW! BLOW! BLOW! BLOW! BLOW! BLOW!
EVERYTHING OUT! And this distance from here to here is called
the 'vital capacity.' And with some other tests, we were able
to actually decide how big your lung actually is which is
called 'total lung capacity.' And again, patients as they
progress to have more problems with their neuromuscular problems,
they have more of a restrictive pattern as these lung volumes
get a little smaller.
This is called a manometer,
and if you had a mouthpiece right down here, we ask you to
put that into your mouth, and that's how you do the MIP and
MEP measurements. So again, you would suck in as hard as you
could, and this little wheel would go around and would give
me an idea of how much negative pressure you could suck in.
And then you would switch the dial, and you would blow out,
and that would give me an idea as to how you can expire your
air. Again, these tests take about 40 minutes; they're very
helpful, and the physician then can give feedback to you from
year to year as to how well your lung function is doing.
Q1: At what age for
children can you do the pulmonary function tests?
Bonekat: Good question.
The question was, "At what age, probably how young can
you be?" In our pulmonary function lab, probably around
six, five or six. If you have an attentive child who can actually
pay attention to instructions and basically take a deep breath
in, hold it, and blow it out, then it could be just about
any age. But probably around five or six years of age is about
the youngest that we're able to get them to pay attention.
Well, what are some
other tests? Some of you in the audience probably have had
pulmonary function tests. You've also had things like a pulse
oximetry. The pulse oximetry is a nice little machine that
has a little probe on it that fits over the distal part of
your finger. And this is a little test that by various wavelengths
can pick up a light source from your blood and give you an
idea as to what your oxygen is in the blood. It's very non-invasive;
it takes about 5 or 10 seconds to do. A lot of time respiratory
therapists from the community can come in and do tests even
in your home. And basically, if your saturation, which is
the saturation of oxygen in your blood, is over 92% or 93%,
then you're doing pretty well. Again, that only gives you
an idea of what your oxygen is, but it's usually a nice, non-invasive
test you're doing.
Some people complain,
'Well, Dr. Bonekat, I also have some problems at night in
my home when I am sleeping. Is there any way we can look at
the oxygenation at night? And the answer is yes! We take the
same pulse oximetry, but a lot of times the technician or
the respiratory therapist from the community will drop off
that oximeter, and you'll wear it overnight, and we can get
a little computerized read-out of eight hours of sleep that
you've done the night before. So that information can then
be brought back to the doctor who says, "O.K. This gentleman
is doing very well. Although you're having a little shortness
of breath, your oxygenation is quite adequate.
This last test over
here is a little more invasive, and it's called an 'arterial
blood gas,' or ABG, and that basically means that we take
a sample from your blood. And this is an arterial sample;
it's not like the vein where the doctor or the nurse will
put on a little strap over your arm, have you do this with
your hand, and then takes the blood out of the vein. It's
actually taken directly from the artery. So, it's a little
more involved, a little more invasive, but basically what
this gives you is a more accurate assessment as to your oxygenation,
but more importantly, it also tells you something about the
carbon dioxide. And if you know about breathing, and you breathe
in your air, you take in the oxygen, and you expel the carbon
dioxide, and the ability to get rid of your carbon dioxide
tells you how well you're ventilating. And if you're not ventilating
very well, you do what's called 'hypoventilate,' that is that
your carbon dioxide can build up and be at a higher level.
And so sometimes we want to know that because if it gets to
the point that you're not ventilating very well, as you'll
see at the end, we have some ventilatory assistive devices
that may be helpful for you to use.
Restrictive Lung Disease and Neuromuscular Disease.
We've talked a little
about what would be occurring at a visit to the clinic, some
of the questions that we ask, some of the tests that we do
to evaluate your respiratory status. I now want to just swing
on a little bit and talk about what we're talking about today
which is sort of restrictive lung disease. Specifically, that
is where the neuromuscular disorders fall in place. So, they
encompass a lot of disorders: there could be patients who
have underlying poliomyelitis, Lou Gehrigís disease-ALS, certain
muscular dystrophies, and certainly patients who have spinal
cord problems, which leave them with para and quadriplegia.
So, you might say, 'Well, where does this restriction occur?
Does it occur mostly in the lungs? Is it the chest wall? Give
me a little idea as to anatomically what is going on.' Well,
these are your lungs, and we've taken them out of the chest
wall, and most patients, I can say, who have neuromuscular
disease usually have normal lungs. So unless you've smoked
a lot or had some previous injury, like recurrent pneumonias,
most of the time the problem is not so much in the lungs themselves
but in the casing in which they fit. So here's your thoracic
cage, or this is your chest wall cavity here, and inside fits
those two lungs which are not shown here. And then on the
bottom part right here is where the diaphragm sits. So, you've
got the diaphragm, which is pushing in and out on the lungs
to ventilate yourself. And then there are the muscles that
fit between these intercostal spaces here that also can be
weakened in patients who have neuromuscular disease. So, the
problem is the chest wall. As you know, sometimes you can
get some deformity: you get what's called a 'scoliosis,' which
is where the back becomes more 'S' shaped, or where you can
actually have a little humping in the back, which is called
a 'kyphosis.' So as you develop these abnormalities in the
chest wall, also the muscles themselves are affected, and
it is this combination of chest wall problem and muscle dysfunction
or weakness that leads patients to not be able to ventilate
as well. We have what we call a 'restrictive pathway.' In
patients, just for comparison, who have other types of lung
disease, such as emphysema, they actually get big lungs. They
have what we call chronic obstructive pulmonary disease, and
their diaphragm is actually forced down, and they actually
have big chests. That's a different problem, however, from
patients who have restrictive lung diseases, such as neuromuscular
disease. What happens is this diaphragm becomes so lax that
it actually doesn't work very well, so it kind of pushes up
a little so that the overall lungs themselves are somewhat
restricted.
Exercise and Restrictive Lung Disease.
Q2: Can you restrict
the capacity of lungs by lack of exercise?
Bonekat: O.K. The question
was, "Can you restrict your lungs by lack of exercise?"
Is that correct? In other words, if you don't exercise, does
your lung shrivel up and just "PUH?" And that's
a very, that's a good question. Actually, exercise in itself,
by taking deep breaths, probably does not improve lung function.
For example, we have a lot of data from the patients who have
emphysema. And they go through a pulmonary rehab program.
And when they go through that, yes, they feel better, their
exercise tolerance is better because they can walk further,
they seem to breathe easier, but their overall lung function
does not really change. So, if you take someone, for example,
with a neuromuscular disease, the importance is not so much
exercising in a way that we think of getting out and moving
around, let's say running or jogging; the idea is to during
the day, do some breathing exercises, and those exercises
are mostly to keep the lungs open so that we don't get collapses,
called "atelactasis". So, if you are in a wheelchair,
and you can't do a lot of exercise, I think there is some
good effect of doing breathing exercises, what we call deep
breathing, incentive spirometry, and it's particularly useful
particularly when you have an infection. The idea is to be
able keep the lungs open and generate as much cough as you
can, when you have an infected process. But specifically,
exercise doesn't make your lungs better.
Q3: Well, eastern medicine
suggests so.
Bonekat: Uh, huh. Well,
I think what I'm saying here now is: If you exercise, and
I guess we're talking, let's say you're not in a wheel chair.
You exercise, those muscles that normally would atrophy, that
is, if we will not be able to walk further, your exercise
tolerance will improve. But the vital capacity of your lungs
has not been shown to improve with exercise. But see, that's
o.k. because if you exercise and do things that you want to
do, because of the exercise, that's really the bottom line.
It doesn't matter what the value of your lung is. It's whether
or not you can do those things that you wish to do in you
daily life activities.
O.K., so we're now
going to come back to this term again, 'oxygenation' versus
'ventilation.' If you've got normal lungs and normal ventilation,
then you're going to oxygenate very well. The problem is,
some people can oxygenate o.k, but as their neuromuscular
disease progresses, their ventilation becomes worse, that
is, the ability to bring the air in and breathe it out. Eventually,
if ventilation becomes so bad, it will also affect the oxygen,
and the oxygen will also go down. So, if for example, over
a period of time I see a patient, and they say, 'Well, Dr.
Bonekat, I've been doing relatively well for the last several
years, but now I'm becoming a little short of breath,' we'll
do certain tests including pulmonary function studies. We
may do an arterial blood gas or oximetry at night. And we
will decide that maybe this individual is starting to have
problems with ventilation. And then, we'll sit down with a
patient and say, 'O.K. I think we're having a problem with
ventilation. Your carbon dioxide, the CO2, carbon dioxide,
has increased. It is leading you to have problems not only
of feeling bad during the day, but you're getting headaches,
you're not feeling good at night. You're kind of listless,
it affects your concentration, and your family members are
concerned that you're not breathing very well.'
So then we say, 'Well,
maybe at this point, maybe you need some type of ventilatory
assistance,' and then all of a sudden, there is a crashing
down. Your brain sort of says, 'Oh my God, do I need to have
a trachaeostomy? Am I going to be in bed and my quality of
life is going to fall off?' And I think in the past, maybe
some of this was very realistic; the quality of life may not
have been so good, particularly if you needed an invasive
type of ventilator with a trachaeostomy that inhibited your
talking and eating and conversing with friends and the like,
but over the last couple of decades, we actually have developed
several different types of ventilators, some of which are
non-invasive and even some that are invasive but we've made
significant improvements in them. So, for the latter part
of the talk, I'd like to go over some of the newer types.
Negative Pressure Ventilators.
Many of these are actually
old ones that are now coming back into being such as negative
ventilators. And then we've had some recently over the last
decade which I think that you may or may not be aware of.
So, if we were to look at those ventilators that are not invasive,
that is, we don't have to put any holes in your throat, any
trachaeostomies, we go back to actually to the 1920's when
an engineer at Harvard by the name of Drinker actually invented
the Drinker iron lung, and I'm sure there are some of you
in the audience, like myself, who can remember as children
going and getting our polio sugar tablets. But we can also
remember when our mothers told us that, 'Don't go out in the
rain because you could get polio,' so back in the 1950's before
we had an adequate vaccine the iron lung was the main source
of ventilation for these individuals. And it worked very well.
It was sturdy, it was reliable, but then after the 1950's
as newer ventilators came into play, it sort of fell out of
use. But now it's kind of making a comeback.
So we'll talk a little
about the iron lung, and then we've had spin-offs of this.
Again, the cuirass, as some of you may know, is sort of like
a turtle-shell that fits over the anterior chest and creates
a negative pressure to breathe, and then we have certain wraps,
certain garments that also work by being involved with the
negative pressure for ventilation. This is just kind of what
some of the older type of ventilator cartoons kind of look
like. You have a patient in here with a bellows, and when
the person pushed up on the bellows, it would create a sort
of a negative pressure. The way I like to think of it is if
you take your vacuum cleaner and you're vacuuming, and then
you put you hand on the vacuum cleaner, it pulls your hand
into the vacuum. You can do that with your mouth too, which
is kind of funny. That negative pressure that pulls everything
in basically is what the iron lung does. In other words, it
creates a negative pressure, allows the diaphragm to come
down, picks the chest wall up, allows air-flow to go into
the lungs, and then basically the bellows is pulled back in.
The pressure becomes more atmospheric, the chest wall goes
down, the diaphragm comes up, and the air is expelled. So
it's this constant negative pressure and then back to atmospheric
pressure that allows you to breathe, and that was the concept
of the iron lung, which is what you would see here. So the
patient inside the iron lung, the head would be out here with
the neck, if any type of assistance would be needed, the porthole
would be open. So if you were in the hospital, the nurse would
come in, change things, change lines. But as you can see,
it is rather bulky. This thing also probably weighed probably
six or seven hundred pounds, so although it is quite reliable
and sturdy, it's maybe a little difficult to have a couple
of these in your house. Here is the end part of the iron lung,
and this is equivalent to the diaphragm, the muscle that I
told you. So that as the motor is turned on, this diaphragm
is pulled back, creates that negative pressure that the patient
is able to take a deep breath in. And then, eventually, the
diaphragm comes forward again, a more positive pressure comes
into being, the patient can exhale as the chest wall goes
down. The nice thing, however, certain companies specifically
'Life Care' has now put out certain porta-lungs, so you've
got smaller size even for children. And so, for those individuals
who are developing problems with breathing, this might be
a workable solution for you. I think in the last five years,
we've had two children, one with spinomuscular atrophy and
I think the other, I can't recall, but maybe was a post-polio
person, who is still using the negative pressure ventilator.
As you can see, they come in all sizes and are still quite
effective. Here is a young boy who uses this at night, and
then comes out of the negative pressure, iron lung during
the day.
This is the cuirass
shield. This is sort of like an umpire's protection here:
It fits across the anterior part of the chest and it is strapped
around. It then has a hose leading to this negative pressure
ventilator. And basically it is like a mini-iron lung. The
patient is able to take a deep breath in as the negative pressure
pulls the chest outward, and eventually the chest collapses
and then expires. So, you can see that it is now becoming
more compact, it is portable, it can be used even in sitting
positions. The disadvantages, you might say, well what are
they? Well, sometimes, particularly in patients who have a
somewhat deformed back or are very thin or very heavy, you
have to get these things sort of self-fit so it becomes a
little more difficult. It doesn't actually give you as good
a negative pressure as the iron lung, so if you have more
severe neuromuscular problems, it might not be effective to
you. As you can see, there might be some air leaks around
the side, and as that shield goes in and out sometimes, you
get some rubbing across the chest. So there are indeed some
disadvantages, but this is a very nice example of something
that could be easily used in people who so significantly need
and want to do so. Likewise, it can be for smaller people,
like the cuirass sheild for young people.
This is the so-called
'poncho,' or wrap. Basically, it's the same idea of a negative
pressure. This is a mesh shield that goes over again the anterior
chest, and the patient lies flat against this little back
plate. Then, they put on this little 'poncho', and there's
some drawstrings across the upper arms and the lower abdomens.
It's hooked onto a machine, a negative ventilator. And that's
what it looks like. So the patient might go to bed at night,
get into this outfit, the machine is turned on, it's cycled
at so many breaths per minute, particularly the machine by
'Life Care' can do that. And the patient falls asleep, and
during the night, we get this negative pressure which again,
lifts the chest for inspiration and then it collapses on expiration.
So, these are very nice, innovative things that are very portable
that may be helpful for certain individuals. This again, is
also another type, which is called the 'poncho' which actually
fits over the entire body. Part of the disadvantages to something
like this is the hassle to get into these jackets at night,
but it certainly, as you can see, beats having some type of
invasive thing in your throat. You can talk, you can eat,
and it is even actually fairly well tolerated.
O.K. so these are kind
of some of the negative pressure devices that are available.
Now, let's look at some that actually displace the abdomen.
Basically, instead of pulling the chest up via negative pressure,
we have a couple of devices that actually push up under the
diaphragm. That is, here is belly down here; it pushes up
the diaphragm to exhale. And then the device is released and,
on inspiration, the diaphragm comes down as the abdominal
contents are pushed away from diaphragm. And two of the examples,
again, that has been around for many years but are still being
used are 'rocking bed' and the 'pneumoventil'. I think I was
looking in the literature as of a year ago, the 'Life Care'
company who again puts out this rocking bed has about eighty
of these rocking beds used across the United States. And they
have also a fair amount of people who use those 'pneumovents'.
So just to show you what I mean, if anything that's going
to push on the abdomen, on expiration, pushes the diaphragm
up so the patient exhales and then on inspiration, there's
a relaxation of the pressure and the diaphragm falls down.
So this is the 'rocking
bed'. When I was a resident back in Maryland some years ago,
I would have to go in and take blood gases on patients who
were on a rocking bed. And that's very difficult because as
the patient is rocking back and forth. Basically, this is
a bed that rocks. And as I told you, as you can all attest
to, when you're standing up, your diaphragm is usually pushed
down. But when you all stand on your head at home, that means
the diaphragm is pushed down and you get your air actually
expelled. That's what the rocking bed does: As it goes up
to a forty degree angle, the diaphragm is down, air comes
into the lung. And as it drops back to the horizontal position,
the abdominal contents push up against the abdomen and expel
the air. This is actually kind of nice. We still have some
patients who use this. O.K., well you might say, 'Well, what
are the disadvantages?' Well, it's a pretty significantly
heavy bed which weighs about, I think about 150 to 200 pounds.
But again, it's non-invasive, and if you can get used to rocking
in bed about 14 times a minute, then this might be a device
that's helpful to you.
"The next is the
'pneumovent'. This is a device that you have to be able to
be sitting at a forty five degree angle. But basically, it's
a, sort of like a 'bladder' that's hooked onto a machine,
and as the 'bladder' is filled up, think of it as a big bubble.
As it fills up, it pushes in on the diaphragm, and pushes
it up, and air comes out of the mouth. As the bladder is deflated,
the diaphragm then descends, and air comes back into the lungs.
This is what it looks like; this is a corsette hooked up to
this machine that supplies the air pressure. Inside that corsette
is this large bladder, o.k. And this is what it looks like
on an individual, so a patient can be using it during the
daytime. This could be another, it doesn't mean that you canít
have more than one assistive device. They may use some type
of ventilatory device at night to sleep, and then during the
day, just to help them breathe, they put this little 'pneumobelt'
on. And it helps them in that it pushes in to expire the air
and releases the air to inspire. So again there are these
devices out there that may be a very nice choice for some
people with certain types of neuromuscular problems.
Positive Pressure Ventilation.
Finally we're kind
of coming down to positive ventilation, not necessarily positive
pressure but positive ventilation with a face mask. So this
is different now. Instead of having something like a tube
in your throat or a trachaeostomy, we're basically doing the
same thing that a regular positive pressure ventilator is
doing, only we're doing it to a mask that goes over the nose
of the individual. And these can be of a volume type, much
like the regular ventilators that you're used to, or the volumes
can be brought about by various pressure changes. So, this
is an example of a non-invasive, intermittent, positive pressure
ventilatory system. Here's a ventilator here. A patient goes
to sleep, tubing comes from the machine, and that's all it
is. It's a little nasal mask. You don't see any trachaeostomy
or anything like that. The patient gets accustomed to that,
usually trying it a couple of nights in a row for longer periods
of time. As you become accustomed, the ventilator is timed,
and you might get 12, 14, 15 breaths at certain volumes. It
goes in through the back of the nose, helps the patient breathe
at night. Again, this is becoming a very nice mode of ventilation.
And as opposed to some of the other devices that I was mentioning,
you can see that this is much smaller. It's portable. Some
of these devices are very light-weight and portable, as I
mentioned.
This is another device
which acts on a pressure gradient. This happens to be one
put out 'Respironics' called 'BiPap.' Maybe perhaps some of
you have heard that, or maybe you even utilize that at home.
But basically, it's the same thing where the mask goes over
the nose. You turn on the machine, and there's a certain pressure
that gives you a volume on inspiration and then the pressure
is somewhat reduced on expiration. And it's that difference
between the pressures that cause the ventilatory assistance.
And again, although this is an old head mask, this is basically
what would then again be hooked on: the 'BiPap' machine that
gives you ventilatory assistance. Some people like BiPap ventilators,
some like the pressure support ventilators, and that really
is actually dependent upon what your choices are.
Well, at this point
in the talk, I thought that I would show you a little video.
I personally think that as a physician that you as patients
still it's hard for you to grasp how perhaps simplistic some
of these devices actually are. I believe that this machine
(the VCR or projector) is probably more complex than this
ventilator." (Audience laughs)
O.K., thanks. Well,
that video was shown to you not to sell you a 'BiPap' machine,
but that 10 minutes was supposed to give you a feeling of
how easy these devices are that are now available and how
easy they are to use. We have excellent physical therapists
in the community who will come out, service the machine, make
sure that everything is working properly, and I think it should
dispel some myths about being chained onto this permanent
ventilator all of the time and decreasing your quality of
life. So those ventilators that I just mentioned were basically
non-invasive ventilators, and I'm just going to take a few
more minutes and talk about some of the invasive ventilators
that are also available.
O.K., so the idea here
is that everything, sometimes with neuromuscular diseases,
doesn't go right all the time, and some people, unfortunately
progress to the point that their respiratory status will get
worse, and some of these non-invasive ventilators aren't going
to quite do the job. So, we still have some other ventilators
present, which are called invasive because they're usually
associated with a trachaeostomy, for ventilatory support.
But I also want to show you that some of these ventilators
are still very portable, very user-friendly and can be used
in a home situation as well as putting them on the back of
wheel-chair. Here you have a gentleman who has a trachaeostomy
in place, and this is the ventilator right here, very small,
not much different in the size that you saw for some of the
other non-invasive ventilators. The only difference is that
this patient has a trachaeostomy in place. The patient can
then go home from the hospital, and as you can see, can converse.
There are certain valves that can be placed around the trach
to allow one to talk. One can eat, converse, and basically,
hopefully have some very good quality of life. This ventilator
can also be placed on the back of a wheelchair and be portable
in nature. So, if it comes to a point that none of the other
non-invasive ventilators are very successful, we then have
some of these very nice small, portable ventilators, that
hopefully will be of assistance to you.
It can be done in combination,
and instead of saying "day" and "night",
I think the idea would be "non-invasive" and "invasive"
because the more invasive you have to usually start thinking
of 24 hour ventilation, so.... But it's very it's very interesting
and in reviewing for this talk, there's a lot of good literature
to suggest that people can be in iron lung, they can be using
some of the non-invasive ventilators for long periods of time.
The only thing that prevents you from wearing it, from being
on a non-invasive ventilator, is usually the fact that you
have to wear something over your face, and it can be somewhat
abrasive. But a lot of times you can use a negative ventilator
part of the day and a positive ventilator, and so in combination,
it may be that you, as you progress, or if someone progresses
with neuromuscular disease, that you may not ever need to
have an invasive tracheostomy. Again, it depends upon the
disease and how quickly it progresses.
So, in summary, I tried to show you today what it's like to come and discuss your respiratory status with the physician. Basically, we are here to help you, and along the way, we can help decide whether or not you're having a problem with your lungs, then we can introduce you to some of these assistive devices. I have to admit that not all pulmonary physicians or general physicians know a lot about some of these ventilators. And even myself, you know, because I have not had a lot of experience with the negative ventilators in the past, but there are very knowledgeable people in the community that we can get to provide the various devices available, particularly companies like 'Life Care' and the like. So in summary then, I just wanted to show you on this last slide that there are various ways to help in ventilation: We've got the negative pressure iron lung, the cuirass shield, we have the small portable ventilators that even can be hooked up to young people when they can still live a fairly good quality of life. And I thank you very much."
Bonekat: We have time for questions? Do you have some?
Q4: If a person has severe sleep apnea, how often should they see their physician? And the other part of that is, does arthritis has an effect upon sleep apnea?
Bonekat: O.K., everyone heard that question. Sleep apnea actually isn't too far from what we were talking about today. Those positive pressure, like the video I showed to you came out in the 1980's, the "BiPap" machine, and it was actually in response to patients, particularly middle-aged men, who started snoring and had upper air-way collapse. So this positive pressure by wearing this nose piece at night prevented sleep apnea from occurring, and sleep apnea means to stop breathing at night, and it's associated with snoring and sleepiness the next day. We've also found that patients who have neuromuscular diseases, particularly the myotonic dystrophy patients, are more prone to maybe developing sleep apnea, so it's important to recognize that fact. And so, we get a history of some of our neuromuscular patients snoring at night. If you remember, I was taking a history about snoring, even in kiddies, young people, then what we'll frequently do is we'll evaluate their sleep at night and see if they're actually stopping breathing because they may require one of these positive pressure assistive devices at night to break that obstruction. The second part of the question was, ìIs there a relationship of arthritis to sleep apnea?" The answer is yes: Peripheral arthritis, like in the hands and the back probably don't play as much a role. Where it does play a role is if you can't lay on your side, let's say, because of your arthritis, but you have to lay on your back. What happens when you lay on your back? Everything flops back, right? So you can remember if your bed partner is snoring, what's the first thing you do? You go (hand motion), and then they're roll over on their side, and the snoring becomes less. So if you have an arthritis, and it makes you have to sleep, let's say, on your back in a supine position, then frequently, sleep apnea may be made worse. However, there is an arthritic condition that affects the jaw. Rheumatoid arthritis can affect the temperomandibular joints. The joints in the jaws and backward, sometimes that can lead to upper airway obstruction in sleep apnea, so the answer is arthritis, in some cases, can play a role in worsening sleep apnea. But I think you as a group should take home the idea that this condition of sleep apnea may be seen, or may be a little more prevalent in patients who have some of the muscular dystrophies.
Q5: I've met people that have sleep apnea should be aware that it can cause strokes. Now, this has happened to my son-in-law.
Bonekat: O.K., that question is a controversial in sleep apnea. O.K., we're already back on sleep apnea. That's o.k., cause I like sleep. The question was, "People who have sleep apnea may have an increased incidence of stroke. The data is out there to suggest that patients who have sleep apnea, that is, stopping breathing at night because of an obstruction, they have a higher incidence of high blood pressure, some heart disease, as well as stroke, o.k.
Q5 : It was lack of oxygen to the brain that they said caused the stroke.
Bonekat: Yeah, I'm not so sure we have a lot of data to say that there's a true cause relationship, but there is, if you took a whole bunch of people, a bazillion people, and if you had people who had sleep apnea in snoring, then you could say statistically that there probably might be a higher incidence of stroke. But we don't have a one-to-one relationship. We do know that if we treat the sleep apnea, and we take it away, then probably the life expectancy goes back to normal, so that's the important thing to recognize.
Excess Secretions and Ventilation.
Q6: If a person has trouble with influx and also has excessive saliva and excessive mucus all through the night, would the ventilating machine be of help or would it be a hindrance?
Bonekat: The question was, by, when you say influx, do you mean like reflux of gastric contents, so called heartburn type of thing? O.K. Then, that's sometimes is a problem. The question is, "If you have a lot of saliva and secretions and reflux and secretions, would it be good or bad to have a ventilator?" and it's not an easy question to answer. The best thing is that if you had reflux of the gastric contents, the idea would be to treat that, and there are certain therapies including blocking your bed up so that gravity would prevent you from having reflux. If you have secretions at night, then the idea would possibly be to try to take a medication or something to relieve the saliva, so patients who have Lou Gehrigís disease have a lot of salivation; we have some difficulty in treating that. But, on the other hand, using the positive ventilation, a lot of times that will prevent you from having a lot of airway problems, and so in that sense sometimes, it can be a help. So, usually we like to treat whatever the underlying problem is specifically before we would treat the positive ventilation.
Q7: You mentioned the pneumovacs and the influenza shots. Is there an RSV vaccine out yet?
Bonekat: Yeah, that's a "Respiratory-[interstitial?] Syncytial Virus." I'm not aware that is. There may be.... There's a number of vaccines for children: there is a flu vaccine, and there's usually no contraindication for neuromuscular disease. And then the pneumovac which you brought up is a shot that usually every four or five years, we give, but I don't think I can say I am aware of the other vaccine.
