Topics Discussed:


What is BiPAP?
Is BiPAP a Ventilator/Respirator?
What is BiPAP used for?
How do I know if I would benefit from BiPAP?
What do I have to do to get BiPAP?
How much does BiPAP cost?
What is it like to use BiPAP?
Recommendations for the BiPAP beginner.



What is BiPAP?

The earliest form of respirator was the iron lung. It worked by creating a vacuum around the chest so that the rib cage would expand and air would be pulled into the lungs. In the 60's it became possible to force air into the lungs using a ventilator. Out of ventilator technology came something called C-PAP, Continuous Positive Airway Pressure. This increased the air pressure so that the patient's airway and lungs were always holding a little extra air. A simple analogy would be to say that breathing is like repeatedly blowing up a balloon (our lungs) and releasing the air. C-PAP keeps the balloon from completely deflating. In the 80's this ventilator technology was adapted for home use by patients with sleep apnea. In many cases of sleep apnea, the patient's upper airway tends to collapse and causes the person to repeatedly stop breathing then suddenly rouse with a huge gasping snore. The extra continuous pressure of C-PAP kept the airway from collapsing and allowed the person to sleep. The biggest adaptation and advantage in this technology was that the pressurized air was delivered through a face mask rather than through a tracheostomy (hole through the neck into the trachea.) With the 90's came a further refinement to this system. BiPAP stands for "Bi-level Positive Airway Pressure". This allowed alternating levels of pressure for inhaling and exhaling. On C-PAP the user has to exhale against all the extra pressure - something that makes adjusting to using it very difficult and makes it unsuitable for use with neuromuscular disease (Much to the dismay of insurance companies as C-PAP is less expensive!). BiPAP allows air to be at one pressure setting such as 12 cm, for inhaling and then is dropped to a lower setting such as 4 during exhaling, then cycles back to 12 for the next breath. This makes it much easier to adapt to and the correct choice in neuromuscular disease.

In short, BiPAP allows us to get more air in with less muscle effort. The best analogy I can think of is that it is kind of the reverse of going up into the mountains. You get short of breath easily there because the air pressure is lower. Putting on a BiPAP mask is like coming back down to sea level from the mountains.


Is BiPAP a Ventilator/Respirator?

BiPAP certainly is a ventilatory assist machine, but it is not a full featured traditional ventilator. A ventilator can do everything BiPAP does but a BiPAP can't do all the things a ventilator can. There are several critical distinctions between BiPAP and a traditional ventilator:

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BiPAP is "non-invasive", requiring only a mask that can put on or taken off as needed. The acronym "NIV" (Non-Invasive Ventilation) is used to differentiate traditional, trached ventilation from CPAP and BiPAP. A traditional ventilator is usually "invasive" meaning it requires that a tube be put down your throat or through your neck (tracheostomy) into the lungs. The tube remains in place as long as the patient needs to use the ventilator even if they reach a point where they can be off the ventilator for hours at a time. A growing trend however is to use a traditional ventilator with a mask or mouth held tube, giving the noninvasive benefits of BiPAP as well as the added settings available with a vent. This option also makes the transition to trach ventilation easier if it becomes necessary. Getting insurance coverage for an expensive vent to be used for BiPAP is likely to be difficult however.

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BiPAP is not generally considered "Life Support Equipment." A ventilator can be set to completely take over breathing, BiPAP can only assist the breathing.


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A ventilator is generally set to deliver air according to volume.  It pumps air in until a certain volume has been reached, then releases it.  BiPAP delivers air according to pressure, stopping when it senses resistance that tells it that the pre-set amount of pressure has been met.  That is a technical difference that probably means very little in how it feels to be on a ventilator versus BiPAP. It may however affect how well lung function is maintained. A volume based air delivery will continue to inflate lungs fully and help prevent atelectasis (loss of the ability of the little air sacs at the furthest reaches of the lungs to expand) and pneumonia. A pressure based delivery system will sense resistance and not try to push air into stiff or clogged air sacs and therefore won't be as helpful in keeping them working.


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Long term ventilator patients often are fitted with trach tubes that allow them to eat and even to speak, but this is not always possible for ALS patients. Because it does not require a trach, BiPAP does not interfere with speech or swallowing.


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ALS patients often reach the point of not being able to cough effectively. With a ventilator and trach, secretions can be removed by passing a suction tube through the trach to remove secretions. If suctioning is needed by patients using BiPAP, the tube has to be passed through the nose or mouth.


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The presence of the trach increases the risk of lung infections because it bypasses some of the normal protective barriers. Use of BiPAP also creates some increased risk for lung infections and sinus infections.


 

What is BiPAP used for?

BiPAP (and its predecessor, CPAP) are most commonly used for people with sleep apnea or with hypopnea.

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Obstructive Sleep Apnea (OSA)
The more commonly seen and therefore well known apnea is obstructive. The muscles of the soft palate and throat weaken and the upper airway is narrowed or even collapsed when the muscles relax as the patient falls asleep. That blocks the airway and the patient wakes up repeatedly, often not aware that the reason he is awakening is because he quit breathing. Some people find they feel startled or even panicky with these awakenings, but others are so tired and groggy from lack of sleep they may not even be aware they are waking up over and over. Snoring generally occurs, but it is possible to have apnea and not snore at all. Similarly, obstructive sleep apnea is often associated with obesity however there are apneaics who are of normal or low weight.


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Central Apnea
Another type of apnea that can occur is "central apnea" in which the little nerve center in the brain stem that is supposed to regulate when we take a breath and how long we hold it fails to work properly when we fall asleep. This can be a problem directly with the nerve center or with the motor nerves that carry the message to the muscles used in breathing. Even though we think of breathing as something that is continually on "autopilot", sleep seems to somehow interfere with the autopilot mechanism and apnea occurs. All it takes is a little stimulation to get breathing going again -- for babies with sleep apnea, just jiggling the crib is often all it takes. For adults, a spousal elbow in the ribs usually does it! BiPAP gives a breath and that breath is either enough stimulation to get you to breathe again or is enough to tide you over until you do breathe again.


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Hypopnea
A third possibility is that the muscles of respiration are weak and during sleep when breathing is normally shallower, breathing becomes too shallow. The patient doesn't stop breathing but breathing is inadequate. BiPAP can be used to increase the volume of air taken in without increasing muscle effort.



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.

 

How do I know if I would benefit from BiPAP?

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.
 

This gradually worsens and lying flat, whether on his back or side, becomes a problem. He finds it necessary to use several pillows or even begin sleeping sitting up or in a reclining chair. The human body was designed to breathe best when upright. Lying down makes it harder for the rib cage to move. Unfortunately, the human body was also designed to sleep lying down.
 

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.

 


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

Unfortunately, finding someone who is "cross trained" to understand both the effects of neuromuscular disease on respirations and the respiratory assistance machines available can be hard. The neurologist knows neuromuscular disease but diddly about the machines. The pulmonologist (physician specializing in respiratory problems) knows the machines but not neuromuscular disease.

And in our screwed up health care delivery system someone might require that you see a sleep specialist. The sleep guys are used to sleep apneaics who have good lung power and is very unlikely to be familiar with the problems of the neuromuscular patient.

Start with your neurologist. A neurologist who works with ALS patients should certainly be familiar with BiPAP. He may handle your respiratory care himself, but may prefer to refer his patients to a pulmonologist for respiratory care at that point. I would be reasonably comfortable with either of them, but if I were sent to a sleep specialist I would most definitely ask "How many ALS patients on BiPAP have you cared for?" and if he could not demonstrate a very good understanding of ALS, I would be looking elsewhere.

The doctor who handles your respiratory care is going to be a very important person in your care. Patients with ALS who do not want to go on a ventilator most often die of respiratory failure so this doctor will be the one who sees you through to the end or sees you through many years on a ventilator. You may still see other doctors, but odds are that this is the one who will be there directing most of your care. You need someone who is comfortable not only in deciding what pressure settings you need and dealing with any respiratory infection, but also in listening, explaining and helping you make decisions about things like tube feedings, code status, and whether or not to switch to full ventilation when BiPAP is no longer sufficient and perhaps when to discontinue the ventilator. At some point he or she will likely be the physician you depend on to make the end as easy and dignified as possible.
 

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Undergoing Testing
 There are several tests doctors can do to monitor how much ALS is affecting breathing.

 
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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.

To  accurately test you, your doctor can arrange for you to use an O2 Sat monitor at home overnight. You just put the device on your finger at bedtime and wear it all night. It monitors oxygen saturation and heart rate, and records it. The next day you return it, the results are retrieved from its memory, and reported to your doctor. If you are breathing too shallowly (hyponea) your O2 Sat should drop. (CO2 levels would be more accurate in spotting hypopnea but as discussed below, they are not as easily done accurately.) If you have apnea, your O2 Sat will drop briefly but dramatically at repeated intervals. These two tests along with a diagnosis of a neuromuscular disease such as ALS and a description of the problems you are having should be sufficient to get a prescription for BiPAP and insurance reimbursement for it. Unfortunately sometimes it gets more complicated. Physicians and/or insurance companies may want more diagnostic evidence and want other tests done.


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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.

If you are required to have a sleep study, be aware that ABG's are commonly done with them. Again it is important that the blood be drawn while you are asleep -- or as soon after awakening as possible. (No one is going to sleep through an arterial blood draw!) Ideally the blood should be drawn first thing upon awakening. I would refuse to have the test done if it was not done at least before I was out of bed. I have heard of instances where it was done after the patient was up to the bathroom, dressed, and ready to go home! No wonder the ABG's did not support her need for BiPAP! You always have a right to refuse a test or medication, and in this case not only is the test useless but it will work against you in getting reimbursement for BiPAP!!!

 

How much does BiPAP cost?

 

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

Medicare and most private insurance companies do what is called a "capped rental" on BiPAP machines. As with most durable medical equipment, they will pay 80 percent of the rental fee up for 13 months. Once they have paid that much in rental, the machine is considered paid for and belongs to you.

 

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.

 

 


What is it like to use BiPAP

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.

 

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.