Ventilator-Assisted Living

Summer 2004, Vol. 18, No. 2

ISSN 1066-534X

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Read selected articles from this issue ...

Quality of Life: Profiles of Living with ALS
Herb Ingham, Long Island, NY
Jack O'Neil, New York City, NY

What is ALS?
More about ALS

Home Mechanical Ventilation in Brazil Revisited
Luiz Gustavo Ghion, PT, and Walmar Augusto Miranda, MD, Home Doctor, São Paulo, Brazil

Homebound Exception Demonstration
David Jayne, National Coalition to Amend the Homebound Restriction, Rex, Georgia

Retrofitting Ventilators for Power Chairs
Jerry Daniel, Vancouver, Washington

Preparing for a Respiratory Emergency
Louie Boitano, MS, RRT, Northwest Assistive Breathing Center, Pulmonary Clinic, University of Washington, Seattle

In Memoriam: Ira Holland, 1939-2004

Respironics and IVUN's Ventilatory Equipment Exchange

Potpourri: ResLink from ResMed; OSA/CPAP users; Medicaid Waivers; Toll-Free Hotline for Airline Disability-Related Problems; The Association for Respiratory Technicians and Physiologists; Chin-Neck Bandage; Rolling On: The Story of the Amazing Gary McPherson

Letter to the Editor from Jeannette Hoitt, PhD and Robert Banzett, PhD, University of Arizona


Quality of Life: Profiles of Living with ALS

The decision by individuals with amyotrophic lateral sclerosis (ALS) to use assisted ventilation when their breathing muscles become weaker needs careful consideration. Some people do not choose to use any form of assisted ventilation. Others, who start early with noninvasive ventilation, may later face a decision about continuing with tracheostomy ventilation. Some may decide to refuse a tracheostomy. In March 2004, The ALS Association’s Clinical Conference in Woodland Hills, California, focused on pulmonary care during each stage of the disease and emphasized early use of noninvasive positive pressure ventilation. Theresa Imperato, RN, Nurse Coordinator, ALSA Greater New York Chapter, a speaker at the conference, contacted two of her patients who wrote these profiles describing their use of assisted ventilation.

Herb Ingham, Long Island, New York

Photo of Herb and JackieI am 72 years old and was diagnosed with bulbar ALS in January 2000. Symptoms started with slurred speech and muscle twitches and progressed to difficulty with swallowing. I was mobile until recently and can stand for short periods of time with help. I can still use my hands on a limited basis and type on a LightWRITER communication device (www.zygo-usa.com). My wife Jackie and I took a course in sign language, but my signing ability has declined and signing my needs at night is difficult. Soon I will need a word-scanning system for my computer because it is becoming more tedious to type.

In the last quarter of 2001, I experienced severe bouts of choking and gagging on mucus. I tried using BiPAP® (www.respironics.com) and CoughAssist® , but neither was successful because of ill-fitting masks. My wife and I were aware that a tracheostomy could help alleviate the choking problem and discussed that prospect with the ALS clinic. The clinic staff was not encouraging because of the long-term ramifications of a tracheostomy — prolonging life with progressive ALS leading to complete paralysis. However, because I still had good limb mobility and was so uncomfortable with the heavy secretions, we were leaning toward the tracheostomy for suctioning, but not for ventilation.

Fate intervened. Hospitalized for an impacted bowel, I experienced such severe gagging that my pulmonologist ordered surgery for a tracheostomy. The tracheostomy eliminated the choking problem but introduced another — throat spasms. Initially these were frequent and severe, sometimes lasting for an hour. (A respiratory therapist told me that such throat spasms are uncommon.) Initially mucus was suctioned up to 20 times per day, and each time the suctioning triggered the spasms. We learned that partial inflation of the trach tube cuff helped to prevent secretions from dropping past the trach, and that, coupled with Lorazepam drops squirted against my inner cheek, helped to control the spasms. Spasms are now infrequent and less arduous. The CoughAssist® has been very effective in removing mucus from the airway and has virtually eliminated suctioning.

In the hospital, I started using tracheostomy positive pressure ventilation at night to rest fatigued breathing muscles and continued that for the past year at home with the LP10 ventilator (www.puritanbennett.com). I am certain that the trach and the ventilator have prolonged my life and kept me comfortable. In recent months I have been sufficiently short of breath to need to use the ventilator fulltime.

A power chair with provision for a ventilator and oxygen is on order and should afford me more freedom. My wife rigged up a dolly with a milk crate containing a battery on top of which rests the ventilator. This is wheeled alongside my wheelchair when we go anywhere; two helpers are required. We had use of a small portable ventilator that could have been attached easily to any wheelchair but after two operating failures, we decided to use a larger ventilator with proven reliability so as not to risk another malfunction.

An important consideration in deciding to use assisted ventilation is long-term care costs. Ventilators, cough machines and associated supplies are expensive. Although I’m covered by Medicare, the copayment has to be paid by private insurance or out of my own pocket. With advancing ALS, a caregiver must be on hand all of the time to respond to alarms warning of ventilator detachment, leaks or other malfunctions. We have an excellent live-in aide. Jackie takes over at night and weekends.

Quality of life issues continue to concern us. I am still able to communicate and do the simple things I enjoy: watching TV, working and playing at the computer, visiting with family and friends. The concern lies with the future as ALS progresses. Ventilator use, although prolonging life, has complicated that issue.


Jack O'Neil, New York City, New York

I am presently 69 years old and was first diagnosed with ALS in 1979. The prognosis was not good; I was told I would have about two years to live. This was an especially difficult time for me because I had just gotten married for the first time in 1978 when I was 43. My wife Eileen, a very smart and dynamic woman, is also a registered nurse with an EdD.

Everyone knows that they are going to die but if someone gives you a date, even an approximate one, it has a way of focusing your attention. Things went downhill very fast because I experienced weakness in my arms and legs, and had a low energy level, but after about six months I seemed to plateau, neither getting any better nor any worse. My mental state was in bad shape because I became very depressed while I worked on my exit strategy. Eileen made me visit a psychiatrist who succeeded in helping me focus on life instead of death.

When my two years left to live were up, I was weak, but I was still working and getting around with a cane and leg braces. My physical condition continued to decline, and I no longer had enough energy to work. I stopped working in 1985 and used a scooter to get around.

New York is a great place to live and, despite my physical problems, Eileen and I enjoyed the theater, movies, concerts and restaurants. We also had a summer home on Fire Island. Our quality of life was good, although I suffered from aggravation and frustration which even healthy people experience.

The major change in my life came on Easter 1999, twenty years after the diagnosis. I woke up struggling for breath, in respiratory failure. Eileen called 911. I ended up in the ICU at Beth Israel Hospital, drifting in and out of consciousness, sure that I was going to die.

After a week in intensive care, I was feeling better and became aware that I was wearing a nasal mask connected to a BiPAP® (www.respironics.com) unit. The mask was very uncomfortable, and although I tried different types of masks, I couldn’t find relief. After two weeks I was able to breathe about nine hours on my own.

The physicians did not think that I was going to survive and transferred me to the hospice program at Beth Israel. I had very mixed emotions about it, but the hospice unit prepared me to return home. By then, I could no longer move my arms or legs more than a few inches. I required help with all of the activities of daily living and acquired an electric wheelchair, Hoyer lift, BiPAP® unit and a variety of accessories.

Fortunately our large two-bedroom/two-bathroom apartment was able to accommodate all of this new equipment. The hospice program also provided an aide for eight hours a day, weekly visits from a nurse and biweekly visits from a social worker. But after 14 months, I was kicked out of hospice because my health improved.

The first thing I did when I came home was to search for a comfortable mask and found one that caused the least discomfort — the Phantom® (www.sleep-net.com).

I have been using BiPAP® for more than five years and have three machines: one in our apartment, one on Fire Island and one in our van. I use BiPAP® at night and when it gets too hot or muggy. I am not thrilled with this arrangement, but I feel that it increases my chances of waking up in the morning by about 70%. I have a home health aide about eight hours a day that I pay for myself, and I see a nurse and a social worker during my visits (every three months) to the ALS clinic.

What is ALS?

Amyotrophic lateral sclerosis (ALS) is also known as motor neuron disease and Lou Gehrig’s disease. It is a degenerative disease of the neurons (nerve cells) in the brain and spinal cord that causes progressive weakness of the voluntary muscles, eventually leading to total paralysis and death.

More about ALS ...

The ALS Association provides an excellent set of manuals entitled Living With ALS. Volume 6 discusses “Adapting to Breathing Changes.” All are available to people with ALS, either online (www.alsa.org) or in hard copy through the chapter offices.

“Disparities in perceptions of distress and burden in ALS patients and family caregivers” by E.E. Adelman, S.M. Albert, J.G. Rabkin, et al. appears in Neurology 2004, 62, 1766-1770 (www.neurology.org). The conclusion: “However, patients and caregivers each overestimated the psychosocial impact of the disease on the other.”

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