Using a BiPAP: Page 1

• Warning Signs of Respiratory Problems
• What is a BiPAP Anyway?
• Dr. Oppenheimer's comments
• Chris' Dad

• BiPAPs and Power Outages
• BiPAPs and Power Outages 2
• Battery back up for BiPAPs
• Battery back up for BiPAPs 2
• Kara's Story

There are warning signs of respiratory problems in ALS that I've heard or of read of: waking up with a headache, daytime drowsiness, and less often vivid dreams or nightmares. If you are having these symptoms, you need to discuss your situation with a doctor and pulmonologist who are familiar with ALS.

Here's what we were told by a pulmonologist at an ALS seminar. Headaches at night or when you first wake up is a typical sign of weakening breathing muscles in ALS. As the illness progresses a point is reached where breathing is too shallow to completely clear carbon dioxide from the lungs. This usually happens at night because our breathing is naturally shallower at night than during the day. When CO2 pools in the lungs it reduces CO2 exchange with the blood, which elevates the CO2 level in the blood. High blood CO2 can cause drowsiness. Also, CO2 is a vasodilator, and dilated blood vessels in the brain can cause headaches. So you wake up with a headache, and maybe a half hour later it's gone because the stronger daytime breathing has cleared the CO2 from your lungs. CO2 retention can be worse if you have lung disease such as emphysema as well as ALS.

Some medical folks may want to put you on oxygen to fix the symptoms, but using oxygen with ALS is not without risk. There are 2 drives for breathing: the need to eliminate CO2 and the need to acquire O2. Your body can become accustomed to a high level of CO2 and "turn off" the first drive. If you go on oxygen your body will sense the higher blood level of oxygen and reduce the second drive. Your breathing may become shallower or may stop completely. If you use oxygen the rate needs to be low to prevent this.

This can also come up if you go to an emergency room with respiratory distress. A doctor who is unfamiliar with ALS may automatically put you on oxygen.

Using oxygen doesn't eliminate CO2. What is needed is full breaths, which requires a ventilator or BiPAP. When I started having morning headaches I went to my pulmonoligist who ran some tests on me and put me on a BiPAP. That cured the headaches.

God bless you on your journey, Wayne S. Phillips

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A BiPAP is a non-invasive machine that assists breathing by pumping air into the lungs. "Non-invasive" means that there is no surgery and no tubes going into the body. This is different than a "ventilator" or "respirator" which usually work through a tracheostomy, a hole surgically cut into the trachea (windpipe). A flexible hose connects the BiPAP to a mask which covers the nose or mouth and nose. Using a BiPAP is an intermediate step between no assisted breathing and using a ventilator. A BiPAP can be useful for those who don't want to go on a ventilator, providing easy breathing while breathing muscles decline.

BiPAP is short for Bilevel Positive Air Pressure. "Positive Air Pressure" means that the BiPAP pushes air into you. Bilevel means that there are different pressures for inhaling, referred to as "IPAP", and exhaling, or "EPAP". This is different than a CPAP which provides a continuous pressure level. Sometimes medical folks use the term CPAP generically to refer to either type of machine. These machines are primarily used for treating sleep apnea. PALS need the BiPAP instead of the CPAP because as the illness progresses it becomes harder to exhale against the higher pressure.

One difference between a BiPAP and a ventilator is that a BiPAP delivers a preset air pressure but a ventilator delivers a preset volume of air. BiPAPs are not intended for use as life support, whereas ventilators are. Also, ventilators have internal backup batteries, but my BiPAP didn't, and many, perhaps all, don't. See my story and Don's story for information on battery backup for BiPAPs.

There are a number of adjustable settings on the BiPAP. These values for these settings are ordered by the doctor as part of the prescription for the BiPAP. A respiratory therapist usually sets up the machine, sets the prescribed values, and trains the PALS and caregiver(s) in it's use. No one is supposed to change the settings without a new prescription from the doctor, but my doctor wrote "titrate to comfort" which let us fiddle with some settings. There are knobs for setting the IPAP, EPAP, BPM, and mode. BPM is breaths per minute. I had a Respironics model BiPAP S/T which has more modes than other Respironics models. The "S" in S/T stands for spontaneous, the "T" stands for timed. In timed mode the BiPAP gives you only the number of breaths set on the BPM knob. In spontaneous/timed mode the BPM is only used as a backup rate. The BiPAP senses when you try to breathe (by the pressure in the hose) and gives you as many BPM as you need. If your breathing falls below the set BPM then the BiPAP will give you a breath.

I always used the S/T mode. I had my EPAP set at 3 or 4 on the advice of my pulmonologist and Dr. Oppenheimer, even though Dr. Bach recommended an EPAP of 0. Having a small pressure during exhalation helps keep the alveoli in the lungs from collapsing. I began with the IPAP at 7 and worked my way up to 16. I had the BPM set at 12.

God bless you on your journey, Wayne S. Phillips

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Most people with ALS do not need to use oxygen (and should not unless there is a pneumonia, or an additional lung disease). However a humidifier may be helpful when using bilabial assisted ventilation (such as the BiPAP unit) without oxygen.

The nasal mask or nasal pillows need to be selected from the many available -- to be comfortable, certainly the correct size, and not strapped too tightly.

Sometimes a chin strap is helpful to keep the mouth closed. I usually advise having 2-3 interfaces that are comfortable and the right fit: one nasal mask, one nasal pillows, and a mouth piece (this way you can switch from one to another, and often avoid discomfort to the skin or nose -- or skin breakdown). The mouth piece is particularly helpful during a "cold" if nasal congestion occurs (a regular mouthpiece, or a Bennett mouthseal). Using a nasal steroid spray, such as Flonase or Beconase, daily helps too.

And, it is important to be familiar and skilled with "assisted cough" techniques, and the use of a the mechanical cough machine (In-Exsufflator -- made by Emerson and also distributed by Respironics) -- so that if a respiratory infection occurs it is possible to effectively clear airway secretions. These skills need to be learned when respiratory muscle weakness is first identified regardless of whether or not any assisted ventilation device will be used.

Also, it is very important to regularly check the respiratory equipment settings to be sure they are correct, as respiratory muscle weakness progresses. Many people with ALS do not have the IPAP and EPAP settings adjusted optimally. In general the EPAP should be kept low (about 3-4 cm H2O); and the IPAP may initially be set at 8 cm H2O but usually needs to be increased up to about 12-14 cm H2O. The IPAP setting should feel comfortable, and needs to give a large enough breath in with each cycle. To check that the settings are satisfactory, a finger oximeter should show an oxygen of 95% or greater (without added oxygen). The oximeter monitoring needs to be done regularly. Each person needs regular evaluation and advice from his/her doctor since each person's situation is unique.

Warm regards,

Edward A. Oppenheimer, M.D., FCCP
Pulmonary medicine -- Los Angeles
(from the ALS Digest)

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My Dad passed away after a 3 year battle with ALS, and I took away a few lessons learned that I feel I should pass on to everyone. This is extremely important from a comfort standpoint if you are anticipating a Bi-Pap for breathing. (They are great). MAKE SURE THE MASK FITS PROPERLY!! Initially my Dad had a nose piece, but found that he preferred breathing out of his mouth too. So when we ordered the Nose and Mouth mask, the rep came, but mis-fitted his size. Too small. Accordingly, when opening his mouth, the top slid down the bridge of his nose. This became very aggravated after a few days and became, cut, bruised and swollen. When using a BiPAP in concert with Oxygen, DEFINITELY INSIST ON A WATER BOTTLE ATTACHMENT for humidifying the O2. This is a must. Again we stumbled across this feature very late and by the time the hospital offered up the bottle to remedy the situation (I think it was simply overlooked by technician) Dad's tongue, lips and mouth were so dry they were bleeding. Stay ahead of this. Very, very important.

Back to the mask fit. Do what you can to achieve a good fit and solid seal without really cynching down on the face. One night, Dad went to bed with it too tight, and as he relaxed on his back in sleep his mouth fell open. The mouth opened wider with the atrophied muscles, and actually locked open. This really exacerbated the dry mouth situation. Very uncomfortable for my Dad. Also a caution here which may sound funny but I think it contributed to his locked open mouth. He yawned quite a bit, and again with the weakened muscles his mouth probably over-extended. In either case I tried to discourage him from really big yawns.

He passed away due to a weakening of the respiratory system and was in no pain related to that. But there was discomfort due to his mask and dry mouth which was absolutely avoidable had we been able to anticipate such problems and address them up front.

My prayers are with all of you with ALS and your families.

Christopher

Read his comments on his dad's feeding tube: Chris' Dad on the feeding tube page.

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