Ventilator-Assisted Living©

Spring 2003, Vol. 17, No. 1

ISSN 1066-534X

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Read selected articles from this issue ...

A Classic Care of Hypoventilation
Bryna Golden

What Is Hypoventilation?
Robert A. Lebby, MD

Louie Boitano, MS, RRT, Northwest Assistive Breathing Center, Pulmonary Cliic, University of Washington, Seattle, Washington

Treating Bronchitis with H2O
Jerry Daniel

Luke's Progress
Mary Garrett

Which Ventilator? Who Decides?

Home or Institution?

Reimbursement for Ventilator Equipment: How It Works

Phrenic Nerve Pacing

Advocacy/Policy Websites

Ninth International Conference on Noninvasive Ventilation: "From the ICU to Home"

Journal Articles

IVUN News Now 12 Pages

Potpourri: ALS Sanctuary; When a Loved One Has ALS: A Caregiver's Guide;
Kaleidoscope: Exploring the Experience of Disability Through Literature and the Fine Arts;
Non-Invasive Respiratory Support: A Practical Guide; DMD Pioneers


A Classic Case of Hypoventilation

Bryna Golden

Photo of BrynaAlthough I would not be diagnosed with a neuromuscular disease until I was 21 years old, I grew up with a number of ailments and health problems. In my teens, I was diagnosed with restrictive lung disease and vital capacity of about 30% that was attributed to scoliosis. Yet, even after fully recovering from a spinal fusion to correct the scoliosis, my shortness of breath continued and walking from class to class carrying my books caused inordinate fatigue.

By my last year in high school, I was having a terrible time waking up every day. I felt hung over in the mornings, my head throbbing and feeling like a bowling ball, my stomach so upset and jittery that I was unable to eat until lunchtime. I occasionally would wake up from my sleep choking and consequently developed an aversion to going to bed.

I had a very hard time getting myself to school, and some days I did not make it. My doctors’ only explanations were that there is no illness that makes a person sick in the morning. They tried to convince my parents that my symptoms were the result of my being a nervous child and suggested that I just didn’t want to go to school. Fortunately for me, my parents believed me when I insisted that there was something physically wrong, and it was their empathy, support, and belief in me that helped me through an extremely difficult and painful time.

After high school, I attended a few semesters of college, but walking around the campus exhausted me so much that, despite my enthusiasm for my studies and schoolwork, I had no energy left for learning and was often too ill and tired to concentrate. I dropped out of college and found a market research job that allowed me to work afternoons and evenings (the times of day I felt best). I also began playing keyboard in a rock band.

By February 1985, I was 21, living on my own with roommates, working fulltime, playing gigs, recording with my band ... and feeling physically awful. The strangling episodes were happening several times a night, and I was terrified of going to sleep. I often had the sensation of floating above myself while watching as I strangled and choked. I would awaken shaking and feeling like the room was spinning. I actually wondered if I really was crazy, since my doctor (supposedly the best in his field) was not concerned that there was anything wrong with me.

Finally, after suffering for weeks with a cough that would not go away, having choking spells every time I fell asleep, and experiencing frequent incontinence while sleeping – a new problem I found terribly embarrassing and perplexing – I felt I could not go on. I insisted to my doctor that something was really wrong with me, but his response was to yell at me and tell me to stop complaining because there was nothing wrong with me.

One week later, I ended up in the ER in respiratory failure with bronchitis, strep throat, and mononucleosis. Later in the ICU, it was recognized that I stopped breathing in my sleep, my oxygen saturation (SaO2) levels were dangerously low, and my carbon dioxide (CO2) levels were dangerously high. I had been experiencing the classic signs and symptoms of hypoventilation (underventilation). (See "What Is Hypoventilation?")

My new doctors suspected that I had a neuromuscular disease that caused the respiratory weakness that in turn caused the pulmonary problems. When I related all my symptoms and how long they had been going on, they told me that I was lucky to be alive. Rather than feeling depressed at the discovery of these new health problems, I felt great relief that finally someone acknowledged an actual physical cause for what I had been going through all those years. It was not just all in my head!

After a week in the hospital, and with the bronchitis almost gone, I was discharged with an O2 compressor and told to use it whenever I slept. I felt like I was in a haze the first couple of days at home, and by the third day I was almost in a coma. Rushed back to the ER, I was admitted in respiratory failure again. This time the doctors considered a tracheotomy, but then decided to transfer me to the Pulmonary ICU at the University of California, Irvine Medical Center. I remained there for a month while many tests were conducted, including an inconclusive muscle biopsy. I was finally discharged with a diagnosis of unidentified neuromuscular disease, sleep apnea, restrictive lung disease, and chronic respiratory failure.

One month later, after trying several negative pressure devices, I began using an Emerson rocking bed and O2 through a nasal cannula whenever I slept. I used the rocking bed from 1985 to 1989 and while it helped to a certain degree, I felt I should have been breathing and sleeping better. I was still having strangling dreams, headaches, and confusion. Also, the large cumbersome nature of the rocking bed meant I was unable to spend a night away from home. This situation made it impossible for me to travel with the band for out-of-town performances.

My pulmonary doctor at UCI attempted to switch me over to a portable ventilator, but was unable to find a mask that I was able to tolerate. I have a very thin face and prominent nose, so the masks would only make a good seal if they were so unbearably tight that they left painful pressure sores on the bridge of my nose. In 1989, he made arrangements for me to see Ahmet Baydur, MD, at Rancho Los Amigos Medical Center and to spend a night there trying different types of noninvasive interfaces. The stay was very successful, and soon after I had my own PLV®-100 volume ventilator with an ADAM circuit and nasal pillows. (I now use nasal pillows with Breeze™ SleepGear™ for more comfort.)

The ventilator changed my life. Once I became accustomed to using it, it was a huge relief to go to sleep and let the machine breathe for me. I was able to wake up feeling rested and with-out headaches, and I was able to travel with the psychedelic rock band I formed, Babylonian Tiles ( I am the lead singer, keyboardist, and songwriter. Our band recorded three internationally released CDs, and we have had five US tours, on the road for more than a month at a time. Touring would not be possible for me without the ventilator.

An extensive surgical cervical fusion in 1995 stabilized my very weak neck. I remained intubated about six hours after surgery but once fully awake, I was extubated and my pulmonologist switched me over to my PLV®-100, which I used until I could sit up and breathe on my own.

Although my unlabeled neuromuscular disease has progressed to the point that I am now only able to walk a few steps with assistance and must use a wheelchair whenever I leave the house, I still only use the ventilator when sleeping or lying down.

Currently, I am experiencing problems that I think are related to my night-time ventilation: daytime headaches and fatigue (on an almost daily basis), and frequent confusion upon awakening. My respiratory therapist suggested I might benefit from a ventilator such as the Newport HT50®, which provides both volume and pressure support, but it may be difficult to obtain. My current physician, Robert Lebby, MD, Barlow Respiratory Hospital, is ready to tackle and resolve these latest problems so I can again awake from a good night’s sleep feeling refreshed and ready to seize the day.

What is Hypoventilation?

Robert A. Lebby, MD

The work of inspiring air, or breathing in, is performed primarily by the diaphragm although other muscles play a smaller role. With many neuromuscular diseases, these muscles become weak and less able to expand lungs and draw air in. Other factors making it harder to fully expand the lungs in some people, such as Bryna, include lung and chest wall stiffness/inelasticity and scoliosis (spinal curvature).

Smaller breaths can result in less overall airflow or hypoventilation. If adequate airflow does not occur through the lungs, then carbon dioxide (CO2) is not effectively eliminated and may build up in the blood. Blood oxygen levels (O2) may also decrease due to the hypoventilation, high CO2 levels, or other reasons.

The symptoms of hypoventilation may be subtle at first, especially in people with neuromuscular disease whose problems evolve very slowly allowing the body to adapt to the gradual changes. The earliest problems usually occur during sleep because the control of breathing changes with different sleep stages and because sleeping in the supine position may worsen breathing.

The signs and symptoms of nocturnal (sleep-related) hypoventilation include many that Bryna describes in her article:

Other symptoms of hypoventilation include:

Anyone experiencing a combination of these symptoms deserves a respiratory evaluation, preferably by a pulmonary physician experienced in neuromuscular diseases, sleep, and hypoventilation. Together, the physician and the individual can formulate a treatment plan for assisted ventilation.

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