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BiPAP (and its predecessor, CPAP) are most commonly used for people with
sleep apnea or with hypopnea.
Whether it is a form of apnea or hypopnea, the patient does not get good quality sleep. Quality of sleep is not just dependent on the total number of hours, but also how that time is broken up by awakenings. In order to feel rested, it is necessary to get blocks of sleep that lasts at least 90 minutes -- that is when REM sleep occurs and REM sleep is apparently the stage of sleep where the brain has a chance to "recharge" itself. Without this good quality sleep, the patient becomes increasingly tired, has trouble staying awake during the day (yet has apnea and awakens if he falls asleep), finds it harder think clearly, concentrate, remember. Depression is very common. In addition the mental effects, the lack of sleep begins to affect physical health too.How does this apply to ALS?All three of the problems discussed above can occur in ALS. Bulbar weakness (weakness of the muscles controlled by the cranial nerves that arise from the bulb-shaped medulla portion of the brainstem) causes swallowing and speech problems and may also allow collapse of the upper airway during sleep when muscles relax. This results in obstructive apnea. If ALS affects the motor pathways the brain stem uses to send the impulses to trigger breathing, central apnea can occur. And of course ALS can affect the muscles used in breathing and cause hypopnea. Hypopnea is probably the most common problem in ALS but it certainly is possible for a combination of these problems to occur in ALS.
Like everything else with ALS, the onset of breathing problems can vary
from patient to patient.
Commonly the person with ALS finds he gets a little short of breath when
lying on his back. About the same time, it is likely he will notice that
he becomes short of breath easily -- with exertion especially, but also after
eating, when overly tired, or just in a stuffy room. Sleeping sitting up is not comfortable for
anyone and it is really unhealthy for someone with ALS.
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If you sleep in a
sitting position and then spend your entire day sitting, you will develop
bed sores, more appropriately called pressure sores, on your tailbone and
other bony points on your backside. This is not a possibility, it is an
inevitable result if you spend 24 hours a day on your butt! People with strokes
or spinal cord injuries may be unaware that they are developing a pressure
sore. You won't be because you haven't lost feeling, and pressure sores HURT! |
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Also, constant sitting, even with your feet elevated, will not allow good
circulation to your legs and swelling will be another problem.
(Click here to see info on "Swelling of Feet
and Legs") |
Using a BiPAP will allow you the wonderful and necessary pleasure of lying down, stretching out, turning on your side, and sleeping soundly.
Another sign of hypopnea is less obvious than being flat out short of
breath and may even be noticed before actually feeling short of breath. Even
in healthy people, breathing is shallower when we sleep. For people with
ALS, that little extra drop in volume can mean trouble since they are breathing
shallowly to start with. When hypopnea occurs, you notice that you are more
and more often waking up in the morning with a
headache that goes away when you get up. The reason
for this is that shallow breathing begins to affect the ability to "blow
off" carbon dioxide. The excess CO2 causes blood vessels in the brain to
dilate which in turn leads to a headache. The headache goes away when you
wake up and start moving around because you breathe more deeply when awake
and blow off the CO2.
We often associate that type of headache with "sleeping wrong" and tight
muscles in the neck. Getting up also eases that muscle strain so differentiating
between the two types of headaches is blurred. Try this: the next time you
awaken with a headache, stay put. Don't change position, just begin taking
deeper breaths. If the headache eases it is due to hypopnea and BiPAP will
help. Untreated, the hypopnea will get worse and you will begin to wake up
more and more tired and groggy. You may begin to feel confused and disoriented
in the morning and tired all day.
Although hypopnea is the more common problem with neuromuscular diseases,
apnea can also occur. It can be really obvious that something is wrong. You
find yourself drifting off to an exhausted sleep only to be jolted awake
by a wave of feeling that includes a galloping, thudding heartbeat, weakness,
dizziness, and a generally panicked feeling. This happens over and over until
you finally are so exhausted you sleep through it. It can also be much more
subtle. You wake up
frequently and may or may not remember doing so. Either
way, in the morning you know you just didn't get enough sleep. You are tired
all day, doze off frequently, have headaches, feel half sick from exhaustion,
have a poor appetite, can't think or remember as well as you did, are emotionally
on thin ice, and feel an undercurrent of constant anxiety. In short, you
have all the problems associated with sleep deprivation even though you may seem to spend hours in
bed and hours dozing in your chair. If you can't breathe well enough lying down to get a good night's sleep,
are sleeping sitting up, having morning headaches, or find yourself waking
up frequently, or just feel like you aren't getting enough sleep, it is a
pretty clear indication you have reached the point where BiPAP could really
improve your quality of life. I sleep better when I am wearing it, but that
is only part of the benefit. Since I began using BiPAP at night, my days
have improved drastically. Not only do I have less trouble breathing during
the day, I eat better, choke less, feel stronger - mentally, physically,
and emotionally, have more stamina, am not cold all the time, my heart is
not pounding along at 100 beat's per minute, and, in general, feel like I
took a giant step back from death's door.
Although hypopnea is the more common problem with neuromuscular diseases,
apnea can also occur. It can be really obvious that something is wrong. You
find yourself drifting off to an exhausted sleep only to be jolted awake
by a wave of feeling that includes a galloping, thudding heartbeat, weakness,
dizziness, and a generally panicked feeling. This happens over and over until
you finally are so exhausted you sleep through it. It can also be much more
subtle. You wake up
frequently and may or may not remember doing so. Either
way, in the morning you know you just didn't get enough sleep. You are tired
all day, doze off frequently, have headaches, feel half sick from exhaustion,
have a poor appetite, can't think or remember as well as you did, are emotionally
on thin ice, and feel an undercurrent of constant anxiety. In short, you
have all the problems associated with sleep deprivation even though you may seem to spend hours in
bed and hours dozing in your chair. If you can't breathe well enough lying down to get a good night's sleep,
are sleeping sitting up, having morning headaches, or find yourself waking
up frequently, or just feel like you aren't getting enough sleep, it is a
pretty clear indication you have reached the point where BiPAP could really
improve your quality of life. I sleep better when I am wearing it, but that
is only part of the benefit. Since I began using BiPAP at night, my days
have improved drastically. Not only do I have less trouble breathing during
the day, I eat better, choke less, feel stronger - mentally, physically,
and emotionally, have more stamina, am not cold all the time, my heart is
not pounding along at 100 beat's per minute, and, in general, feel like I
took a giant step back from death's door.
If you can't breathe well enough lying down to get a good night's sleep, are sleeping sitting up, having morning headaches, or find yourself waking up frequently, or just feel like you aren't getting enough sleep, it is a pretty clear indication you have reached the point where BiPAP could really improve your quality of life. I sleep better when I am wearing it, but that is only part of the benefit. Since I began using BiPAP at night, my days have improved drastically. Not only do I have less trouble breathing during the day, I eat better, choke less, feel stronger - mentally, physically, and emotionally, have more stamina, am not cold all the time, my heart is not pounding along at 100 beat's per minute, and, in general, feel like I took a giant step back from death's door.
What do I have to do
to get BiPAP?
You have to have a physician's order (prescription) to get a BiPAP machine even if
you do not plan to seek insurance reimbursement so the first step is . .
.
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Finding
a Doctor
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Undergoing Testing |
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A respiratory function test involves huffing and puffing into a machine to see how much lung capacity you have. The FVC (Forced Vital Capacity) which is basically how deep a breath you can take is one of the most important findings of the test . At about 70% of normal you begin to notice problems, less than 60% is considered moderate, less than 40% is severe. People with neuromuscular diseases should have the test done while they are lying down as that is when their breathing is the most impaired. A respiratory function test/FVC does not show whether you have sleep apnea or if hypopnea is even worse when you fall asleep, however. |
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Another helpful test is a simple test of blood oxygen level. This is reported
as Oxygen Saturation (O2 Sat)
percentage. An O2 Sat of 96% means that 96% of your red
bloods cells are carrying a full load of oxygen (are saturated) like they
are supposed to be. Normal is 90% or better (usually high 90's). O2 Sat
monitoring can be done with a simple little device you clip or tape on your
finger tip. No needles! For us, the important thing is to wear the device
while lying down and sleeping. A spot check in the middle of the day will
no doubt show excellent levels when breathing problems first begin because
our breathing is at its worst when lying down and/or sleeping. |
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Doctors tend to be irritatingly fond of lab tests called ABG's (Arterial Blood Gases). ABG's require drawing blood from an artery (usually in the wrist - an "uncomfortable" procedure to say the least). ABG's can tell a doctor a lot more about your respiratory status than just oxygen levels. For example, they tell the CO2 levels, can help determine if your breathing problems are due to lung problems or other problems such as liver or kidney disease and even how well your body is able to compensate for the problem. But ABG's are NOT always necessary. Although ABG's are a perfect way of checking CO2 levels, they are a total waste of time for the early stages of breathing problems in ALS if they are done when you are wide awake and breathing normally!! When breathing problems become severe CO2 levels will be high even when you are awake, but not early on when you first could begin benefitting from BiPAP. DO NOT allow them to draw ABG's during a daytime appointment (unless you have some other lung disease too) as a first step in determining if you need BiPAP. You have to be in pretty bad shape to have a high CO2 while you are wide awake and being stuck in an artery with a big needle! INSIST that the doctor explain what he needs to know from the ABG's that he can't figure out simply by knowing that you have ALS and overnight O2 Sat monitoring. Your doctor may believe that the insurance company will reject a claim for BiPAP without this bit of supportive evidence but I would ask him to file with all the other info he has on you first and would not agree to the test unless the insurance company rejected my claim. |
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Some physicians and/or insurance companies will want you to have a
sleep
study done to verify the need for BiPAP. This
expensive test involves spending a night in a sleep lab trying to sleep while
you are all wired up with monitoring devices. The information will tell the
doctor whether you have sleep apnea and help determine what kind of pressure
settings your machine will need to be at. A sleep study is necessary to get
a diagnosis of Sleep Apnea, but for an ALS patient that should not be not
necessary. The use of BiPAP for the breathing problems accompanying ALS is
well documented and standard care. Simply having a diagnosis of ALS and evidence
of the onset of breathing problems should be sufficient to justify ordering
Bi-PAP. A secondary diagnosis of sleep apnea or hypopnea by a sleep lab should
not be needed. But, depending on your insurer, you may have to jump through
their hoops. Again, your doctor may believe that the insurance company will
reject a claim for BiPAP without this bit of supportive evidence but I would
ask him to file with all the other info he has on you first and would not
agree to the test unless the insurance company insisted on it.
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The machines cost $2000-$3000. It has been my impression that they are covered by most medical insurances. Although it is possible to purchase your own machine, the most common thing is to "rent" one from a respiratory care company in such a way that you eventually "own" the machine. I have found that renting a machine is well covered but sorting through the finances is a headache. No one seems able to explain it fully. Therapists are seldom well versed in the billing process and billing people only seem to know what your current statement shows, not what is going to be billed in the future. It is also scary because no one seems willing to put anything in writing and rental fees can be $600/month or more! Anyway, the common way of "renting" BiPAP machines is called Capped Rental.
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Capped
Rental
In the past, capped rental programs through Medicare differed in that once the 13 months were up you were given the option of owning the machine but not receiving any further visits, maintenance, or repairs from the supplier, or agreeing that the machine would continue to belong to the supplier and be returned to them if you no longer needed it or changed supplier. With the second option the supplier provided visits by a Respiratory Therapist to check the machine or adjust the settings or help you find the mask that works best for you. Maintenance, repairs and replacement were the responsibility of the supplier at no charge.In 2006 Medicare dropped the second option by placing BiPAP in the category of medical equipment that does not need frequent or substantial servicing. Now, after 13 months the machine belongs to you and the supplier provides no further services.The machine is expected to last for at least 5 years and Medicare will pay for any needed repairs, but the routine maintenance is up to you, as is dealing with mask problems and setting changes. The loss of regular contact with a Respiratory Therapist who works closely with many BiPAP users leaves users without a valuable resource.Replacement of masks, tubing, etc. is 80% covered by Medicare on their time schedule: 90 days on masks, 2 pair of nasal pillows and new tubing per month, 180 days on headgear. |
There are no doubt variations from one insurance company to another, but it is likely that insurers will follow Medicare's lead and end their service contracts with BiPAP suppliers.
I'd had a very slow progression of ALS (first definite problems noted
9 years before I began noticing some breathing problems). At first it was
just feeling a little short of breath when lying on my back. With time that
got more definite. I began waking up with headaches occasionally but not
consistently. I saw a pulmonologist (physician specializing in respiratory
problems) and had pulmonary function tests done. My function was at 70%.
After a few more months I reached the point where I had to sleep on my side
and even that became uncomfortable at times. I got a hospital bed so I could
sleep with the head of the bed up and that helped for a while.
Then I
started experiencing repeated awakenings with an awful rushing feeling starting
in my chest and spreading. My heart would be racing and pounding. It was
not too bad during the day though there would be stretches of an hour or
two when I was uncomfortable even though sitting up. But nights were awful.
I would eventually fall into an exhausted sleep by turning on my side while
having the head of the bed up 45 degrees, but you can imagine what my back
and shoulder felt like after a couple of hours. It took hours to fall asleep
and I was waking my husband up so many times a night to be repositioned,
neither of us were getting enough sleep.
I went to see my pulmonary doctor again and he recommended a "poor man's"
sleep study. Rather than have me come into the Sleep Center for an overnight
full monitoring session, he sent me home with an O2 Sat. monitor. That is
a little machine that has a fingertip sensor you tape on to detect what your
oxygen level is. It also takes your pulse. I wore it overnight (a fairly
good night) and we found that my O2 dropped to 65 % at times. Normal is over
90%. My pulse was humming along at 90-110 even in my sleep. Normal is 60-90.
I was promptly visited by a home respiratory care agency and they set me
up with the Bi-PAP machine. (No attempt was made to define whether my problem
was apnea or hypopnea, nor were ABG's needed. The insurance accepted my claim
on the first try and didn't ask for any further proof that I needed BiPAP.)
The machine itself is small, about the size of a 4 slice toaster. It has
a hose that attaches to a soft, flexible plastic mask that fits over your
nose. (Doesn't cover your mouth.) There are velcro straps on the mask that
fit around your head so the mask fits snugly and air doesn't leak out. When
turned on, the machine blows air into the mask. It doesn't use oxygen, just
regular room air but it can use oxygen if you need it.
Now the fun part! The therapist selected a mask she thought would be the
best size for me, and went about strapping it on. Imagine being short of
breath to start with - not just a momentary event but a couple of months
of feeling miserable in stuffy rooms and suffocated by lying flat -- then
imagine having someone put a plastic mask over your nose. Instant panic!
Being the kind of person who never learned to swim because I was terrified
of water up my nose and dunking my head didn't help at all!
"Where is the Valium??" I whined as I sat forward in my chair and gasped
for air.
I convinced myself I could breathe through my mouth just fine and relaxed
after a moment and let her work with the straps. I told her I thought a lot
of my anxiety came from the fact that my arms are so weak and I knew I wouldn't
be able to just grab the mask and pull it off by myself. "A control issue,"
as they say.
Then she said I needed to breathe through my nose when the machine was
on or the air pressure was lost through my mouth.
"OK," says I, gamely. She
turned the machine on . . . Ever ride in a car at 80 miles an hour with your
head out the window? All that wind and you can't get a breath of air because
it is moving so fast! The mask was not snug and air was whooshing out and
I opened my mouth to take a breath and my ears popped from the change in
pressure. I found some arm strength I didn't know I had and off it came!!!
She assured me it would feel fine once the mask was snug and I braced
myself to try again. Mask on, air blowing wildly in my face as she worked
with with the straps and I gasped through my mouth.
"There," she said. "Breathe through your nose!"
I tried. Snorted. Tried again. I found that the pressure wasn't noticeable
at all as far as taking a breath was concerned. It was just as easy to breath
with the mask on. I sat there, trying hard to stay halfway relaxed as she
adjusted the straps.
Just as I was thinking I might be able to tolerate this,
I had to speak to answer her when she asked if there were any leaks or if
the straps were uncomfortably tight. When I opened my mouth the air rushed
out and my ears felt the pressure change. What a weird sensation! It was
hard to talk around the pumps of breath from the machine. Yuck!!!! I wanted
out!
She encouraged me to keep breathing through my mouth.
It took me a half
a minute to settle down and just breathe, but when I did I could feel a
tremendous difference. WOW! It worked! I was rewarded with the first full
breaths I'd had in months! That tight, achy, tiredness in my chest that I
had been living with was eased. That was all the encouragement I needed to
keep going. She decided I needed a different size mask as there were still
some small leaking areas and it seemed to be putting a lot of pressure on
the bridge of my nose. We swapped masks and tried again. Again the process
of getting it on was icky, but once it was halfway snug it felt not just
tolerable, but good. I didn't really want to take it off when
she left, but neither did I want to be home alone with this weird contraption
on me!
My husband had not been able to be home during all this, so when we went
to bed that night, he had to get the thing on me without instruction. It
is quite simple but he was not prepared for me to get all panicky while it
was loose and the air blowing in my face. (The therapist had recommended
having the machine on while putting it on.) We managed though and I was soon
breathing happily if a little tensely as I experimented with such death defying
maneuvers as tilting my head and swallowing. I stayed sitting up for a long
time, but just couldn't fall asleep and finally felt secure enough to try
putting the head of the bed down. No problems! Required a little adjusting
of the fit as it wanted to leak a little air, but that was easily fixed.
But I still couldn't get to sleep. I sleep best on my side and decided to
give it a try. Woke up my husband, told him what I wanted (getting better
with the talking part) and he turned me. Wriggled the mask a little to seal
the new leaks and I was comfy and snoozing shortly.
A couple of hours later I awoke in a panic and yelled for him to "Get
it off!" It took a little doing to get it undone in the dark. I decided I
had had enough fun for one night and left it off. Breathing was uncomfortable.
I had him reposition me a couple of times, put the head of the bed back up,
and soon decided I really wanted the mask back on. Long suffering hubby made
irritated noises, but put it back on. This time I had him leave the machine
turned off until the mask was in place and that worked better for me.
Finally
convinced that I was in control - it wasn't forcing breaths on me or fighting
my breathing at all, I curled up on my side and slept for 4 hours straight
-- except for one little wake-up by our kitty who had decided it was time
to check out what idiocy I was up to now with that thing on my face!
She
sniffed carefully at it, tickling me with her whiskers, sat and stared for
a bit, sniffed again, then turned away.
"Whatever gets you through the night," she shrugged as she wandered off.
I agreed.
I didn't realize how pervasive lousy breathing is until after a week or
two of using the machine at night and for a rest period during the day or
evening. I had been feeling so bad in so many ways and never associated it
with the breathing problem! I had been tired, weaker than ever, totally lacking
in stamina. I simply sat and played solitaire on the computer all day. I
experienced periods of dizziness, feeling faint, feeling nauseated. I had
no appetite and felt just stuffed after a few bites (a real change for me!!)
so I was losing weight. I was choking more easily, and if I did eat, I had
abdominal cramps and nausea immediately afterward. I was miserably cold most
of the time. It wasn't that the air around me felt cold, it was like my bones
were icicles and the cold was seeping outward. I knew my heartbeat was too
fast and suspect that my blood pressure was very low. Emotionally I was outwardly
OK but I could feel a constant state of anxiety that somehow existed side
by side with a sort of mental sluggishness and lack of initiative. Anything
that seemed to make it harder to breathe -- a stuffy room, a hug, lying down,
sent me into a momentary panic. Making love resulted in panicky gasping for
air. Bed time was no relief, in fact it was the worst because I knew I was
going to be jolted awake over and over.
Every one of those problems started to improve immediately with the Bi-Pap.
That isn't intended as a testimonial ("Step right up here folks! Get your
Dr. John's Famous Elixir! Cures the apoplexy, nervous conditions, gout, and
sweetens your breath!") but simply to show you how breathing problems sneak
into everything your body does. I had no idea that most of my problems were
related to my breathing. I can't promise you that you will have this kind
of results, but I do think you will find that overall you feel a lot better
if you start using Bi-PAP. (And no, I do not own stock in Respironics Co.!!
Wish I did!) Having to wear a stupid looking mask every night, putzing around
to get comfortable and get rid of annoying little leaks, the annoying cold
stream of exhaled air, dealing with a lumpy head of hair and facial crease
marks from the mask every morning is not fun, but it is so much more tolerable
than living without it.
Then I started experiencing repeated awakenings with an awful rushing feeling starting in my chest and spreading. My heart would be racing and pounding. It was not too bad during the day though there would be stretches of an hour or two when I was uncomfortable even though sitting up. But nights were awful. I would eventually fall into an exhausted sleep by turning on my side while having the head of the bed up 45 degrees, but you can imagine what my back and shoulder felt like after a couple of hours. It took hours to fall asleep and I was waking my husband up so many times a night to be repositioned, neither of us were getting enough sleep.
I went to see my pulmonary doctor again and he recommended a "poor man's" sleep study. Rather than have me come into the Sleep Center for an overnight full monitoring session, he sent me home with an O2 Sat. monitor. That is a little machine that has a fingertip sensor you tape on to detect what your oxygen level is. It also takes your pulse. I wore it overnight (a fairly good night) and we found that my O2 dropped to 65 % at times. Normal is over 90%. My pulse was humming along at 90-110 even in my sleep. Normal is 60-90. I was promptly visited by a home respiratory care agency and they set me up with the Bi-PAP machine. (No attempt was made to define whether my problem was apnea or hypopnea, nor were ABG's needed. The insurance accepted my claim on the first try and didn't ask for any further proof that I needed BiPAP.)
The machine itself is small, about the size of a 4 slice toaster. It has a hose that attaches to a soft, flexible plastic mask that fits over your nose. (Doesn't cover your mouth.) There are velcro straps on the mask that fit around your head so the mask fits snugly and air doesn't leak out. When turned on, the machine blows air into the mask. It doesn't use oxygen, just regular room air but it can use oxygen if you need it.
Now the fun part! The therapist selected a mask she thought would be the best size for me, and went about strapping it on. Imagine being short of breath to start with - not just a momentary event but a couple of months of feeling miserable in stuffy rooms and suffocated by lying flat -- then imagine having someone put a plastic mask over your nose. Instant panic! Being the kind of person who never learned to swim because I was terrified of water up my nose and dunking my head didn't help at all!
"Where is the Valium??" I whined as I sat forward in my chair and gasped for air.
I convinced myself I could breathe through my mouth just fine and relaxed after a moment and let her work with the straps. I told her I thought a lot of my anxiety came from the fact that my arms are so weak and I knew I wouldn't be able to just grab the mask and pull it off by myself. "A control issue," as they say. Then she said I needed to breathe through my nose when the machine was on or the air pressure was lost through my mouth.
"OK," says I, gamely. She turned the machine on . . . Ever ride in a car at 80 miles an hour with your head out the window? All that wind and you can't get a breath of air because it is moving so fast! The mask was not snug and air was whooshing out and I opened my mouth to take a breath and my ears popped from the change in pressure. I found some arm strength I didn't know I had and off it came!!!
She assured me it would feel fine once the mask was snug and I braced myself to try again. Mask on, air blowing wildly in my face as she worked with with the straps and I gasped through my mouth. "There," she said. "Breathe through your nose!" I tried. Snorted. Tried again. I found that the pressure wasn't noticeable at all as far as taking a breath was concerned. It was just as easy to breath with the mask on. I sat there, trying hard to stay halfway relaxed as she adjusted the straps.
Just as I was thinking I might be able to tolerate this, I had to speak to answer her when she asked if there were any leaks or if the straps were uncomfortably tight. When I opened my mouth the air rushed out and my ears felt the pressure change. What a weird sensation! It was hard to talk around the pumps of breath from the machine. Yuck!!!! I wanted out! She encouraged me to keep breathing through my mouth.
It took me a half a minute to settle down and just breathe, but when I did I could feel a tremendous difference. WOW! It worked! I was rewarded with the first full breaths I'd had in months! That tight, achy, tiredness in my chest that I had been living with was eased. That was all the encouragement I needed to keep going. She decided I needed a different size mask as there were still some small leaking areas and it seemed to be putting a lot of pressure on the bridge of my nose. We swapped masks and tried again. Again the process of getting it on was icky, but once it was halfway snug it felt not just tolerable, but good. I didn't really want to take it off when she left, but neither did I want to be home alone with this weird contraption on me!
My husband had not been able to be home during all this, so when we went to bed that night, he had to get the thing on me without instruction. It is quite simple but he was not prepared for me to get all panicky while it was loose and the air blowing in my face. (The therapist had recommended having the machine on while putting it on.) We managed though and I was soon breathing happily if a little tensely as I experimented with such death defying maneuvers as tilting my head and swallowing. I stayed sitting up for a long time, but just couldn't fall asleep and finally felt secure enough to try putting the head of the bed down. No problems! Required a little adjusting of the fit as it wanted to leak a little air, but that was easily fixed.
But I still couldn't get to sleep. I sleep best on my side and decided to give it a try. Woke up my husband, told him what I wanted (getting better with the talking part) and he turned me. Wriggled the mask a little to seal the new leaks and I was comfy and snoozing shortly.
A couple of hours later I awoke in a panic and yelled for him to "Get it off!" It took a little doing to get it undone in the dark. I decided I had had enough fun for one night and left it off. Breathing was uncomfortable. I had him reposition me a couple of times, put the head of the bed back up, and soon decided I really wanted the mask back on. Long suffering hubby made irritated noises, but put it back on. This time I had him leave the machine turned off until the mask was in place and that worked better for me.
Finally convinced that I was in control - it wasn't forcing breaths on me or fighting my breathing at all, I curled up on my side and slept for 4 hours straight -- except for one little wake-up by our kitty who had decided it was time to check out what idiocy I was up to now with that thing on my face!
She sniffed carefully at it, tickling me with her whiskers, sat and stared for a bit, sniffed again, then turned away. "Whatever gets you through the night," she shrugged as she wandered off.
I agreed.
I didn't realize how pervasive lousy breathing is until after a week or two of using the machine at night and for a rest period during the day or evening. I had been feeling so bad in so many ways and never associated it with the breathing problem! I had been tired, weaker than ever, totally lacking in stamina. I simply sat and played solitaire on the computer all day. I experienced periods of dizziness, feeling faint, feeling nauseated. I had no appetite and felt just stuffed after a few bites (a real change for me!!) so I was losing weight. I was choking more easily, and if I did eat, I had abdominal cramps and nausea immediately afterward. I was miserably cold most of the time. It wasn't that the air around me felt cold, it was like my bones were icicles and the cold was seeping outward. I knew my heartbeat was too fast and suspect that my blood pressure was very low. Emotionally I was outwardly OK but I could feel a constant state of anxiety that somehow existed side by side with a sort of mental sluggishness and lack of initiative. Anything that seemed to make it harder to breathe -- a stuffy room, a hug, lying down, sent me into a momentary panic. Making love resulted in panicky gasping for air. Bed time was no relief, in fact it was the worst because I knew I was going to be jolted awake over and over.
Every one of those problems started to improve immediately with the Bi-Pap. That isn't intended as a testimonial ("Step right up here folks! Get your Dr. John's Famous Elixir! Cures the apoplexy, nervous conditions, gout, and sweetens your breath!") but simply to show you how breathing problems sneak into everything your body does. I had no idea that most of my problems were related to my breathing. I can't promise you that you will have this kind of results, but I do think you will find that overall you feel a lot better if you start using Bi-PAP. (And no, I do not own stock in Respironics Co.!! Wish I did!) Having to wear a stupid looking mask every night, putzing around to get comfortable and get rid of annoying little leaks, the annoying cold stream of exhaled air, dealing with a lumpy head of hair and facial crease marks from the mask every morning is not fun, but it is so much more tolerable than living without it.
Recommendations
for the BiPAP beginner
There are a variety of
masks on the market. Do not settle for one that leaves you with a annoying
air leaks or leaves a red mark on your face. All masks will leak at times and leave some morning
creases on your face. A mask that seems to leak here or there all the time in spite of trying adjustments to the straps and headgear should also be replaced. Although a bit of leaking at times is to be expected and is not dangerous, it is annoying and unnecessary. (Don't confuse air flowing
from the exhale port with leakage. It can be noisy if it is exhaled against a blanket and cold if it is reflected back onto your face by the bunched up blankets.)
If the marks on your face do not go away completely within an hour or so of removing the mask, insist on a different style mask. One
that is a poor fit will break down the skin and leave a nasty sore. The only way to heal it will be to stop using the mask anyway.
You can adjust, revise, remodel, replace the headgear (straps) anyway
you can think of to get the mask to fit comfortably. The headgear always
seems too large for my head and I generally need to cut sections out of the
straps to shorten them. (The "classic" apneic is an overweight male
with a big head and huge neck and the headgear seems to be sized to fit them!)
Some of the straps have a rough or rubbery finish that makes them stick
to the pillow and make it even harder for me to move my head. There
is no danger in adjusting or changing or even creating your own headgear
to make the mask fit more comfortably and leak less. You cannot mess around
with the mask itself however. For example, you might be tempted to try to
redirect the flow of exhaled air but changing that flow can result in CO2
retention.
I haven't tried nasal
pillows -- soft little nose plugs that are used instead
of a mask -- but have heard from many people that they are the best solution
when a comfortable mask just can't be found. There are quite a few masks
and nasal pillow options available and no one should have to settle for something
really uncomfortable or leaky.
Probably the biggest problem patients with ALS and other neuromuscular
diseases face when trying to use BiPAP is that weak jaw muscles cause them
to sleep with their mouth open. The air escapes through the mouth so
that the necessary pressure cannot build up. This defeats the purpose
of BiPAP. A jaw strap
can be added to gently close the mouth. Sometimes however
the lips cannot be kept together tightly enough and air escapes anyway.
There are masks that cover both the nose and mouth that can be tried
in this case.
There are only a few brands of BiPAP machines available, but whichever
you end up with, you MUST have a
humidifier with
it unless you live in a jungle. All users seem to go through a period of
nasal congestion. Fine all night, wake up, take off the mask and start sneezing
and dripping. This is apparently a reaction of the nasal passages to the
change in air flow. The flow of air from the BiPAP causes the nasal
lining to produce extra fluid to keep them from dying out. When
you take off the BiPAP, it takes an hour or two for the fluid production
to slow down to normal. Humidification helps greatly to reduce the morning
sniffling. It also prevents the nasal pain that can be caused by drying.
There are two types of humidifies available, pass-over and heated. A pass-over humidifier is simply an enclosed water tray that the air passes over and picks up humidity from before reaching your mask. This type of humidifier is suitable for most ALS patients unless they are chronically
dehydrated because of swallowing problems. A pass-over humidifier is easy to use but requires frequent cleaning and water changes to prevent bacterial and fungal growth.
A heated humidifier warms the air and water so the air picks up more moisture. The warmth provides an ideal environment for bacteria and fungus so cleaning is critical. Many heated humidifiers now come with water trays that can be put in the dish washer for cleaning. Heated humidifiers need
to be plugged in so you will need yet another electrical outlet. The high humidity can cause "rain-out". Condensation in the hose can build up a lot of water and make the air flow gurgle. More annoying is the occasional spill of water into your mask and up your nose when you turn!
Cleaning either type of tray is not complicated but needs to be done frequently. Some can go through the dishwasher. Otherwise they need to be washed and/or soaked in a vinegar/water (vinegar has good germ killing properties!), bleach/water, soapy water, or another disinfectant solution. The hose
needs to cleaned as well. Whether to use sterile water, distilled water, boiled water, or tap water in the tray is controversial. Distilled water is the most often recommended.
Nasal pain can be caused by cold air -- as can waking up in the morning
with a really cold snout! A
heater can be
purchased, but simply putting the length of the tubing under the covers with
you is usually enough to warm it to a comfortable level.
Some folks have trouble with their jaw relaxing and their mouth opening
during sleep. This is a real problem for ALS patients with bulbar weakness
but can happen with anyone. You will know you are doing that if you wake
up with your mouth feeling like the Sahara. If you had severe classic sleep
apnea, it would probably be dangerous to allow this to happen at all but
since those of us with ALS don't quit breathing entirely, it will just cause
a dry mouth and return of the headache. If it becomes a continuous problem
that is canceling out the benefit of the machine, a jaw strap or full mask
must be tried.
Some machines are noisy. Spouses complain but also say it is better than
the restlessness of someone who can't sleep. My Respironics machine is fairly
quiet. It has a rhythmic breathing sound as I inhale and exhale. It is a
soothing, "white noise" to me, like having a fan running. It puts me right
to sleep. Putting the machine on the floor rather than the bedside
table will help if the noise bothers you. Some users even set it outside
the bedroom door or in a closet.
There is a newsgroup, "alt.support.sleep-disorder" where you can find a lot of info on types of masks, suppliers, etc. It is "populated" mainly by people using CPAP / BiPAP for sleep apnea rather than neuromuscular diseases so some of the info doesn't apply, but I have gotten great tips there on things like types of masks available, new masks on the market, cleaning routines, traveling with BiPAP, emergency power supply, etc.
