Ventilator-Assisted Living©

Fall 1994, Vol. 8, No. 2

ISSN 1066-534X

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Read selected articles from this issue ...

Demands of Caregiving for Home-Based Ventilator-Assisted Individuals: A Preliminary Report
Mary Ann Sevick, ScD, RN, Pittsburgh, Pennsylvania

Home Mechanical Ventilation: Obstacles to Living at Home
E.A. Oppenheimer, MD, Los Angeles, California

Noninvasive vs. Invasive Ventilation

From Trach-Free to Trached to Trach-Free Again
Nancy Quinn, Saint Petersburg, Florida

Forum for Ventilator Users:

Poland Needs Ventilators

Dr. Spencer Is Retiring

Potpourri: Learning Obstacles for Positive Pressure Ventilation in the Home; Domiciliary Ventilatory Support, policy statement of the British Thoracic Society; Electromagnetic Interference (EMI); Tracheostomy Tube Adult Home Care Guide


Muscular Dystrophy

Daniel M. Goodenberger, MD, Washington University, Saint Louis, Missouri

Daniel M. Goodenberger, MD, is a pulmonary specialist with a particular interest in the lung problems of people with neurologic and muscle diseases. He is on the faculty at Washington University, where he is Director of Pulmonary Fellowship and Director of the Internal Medicine Training Program.

The history of ventilatory support for neuromuscular disease was, until the mid-'80s, heavily influenced by external negative pressure ventilation, which is cumbersome and can lead to accentuation of obstructive sleep apnea. It is still obviously useful in some patients, but I have not used it in six years. I treat patients based on their symptom complexes and some screening. I question them about symptoms of night-time hypoventilation, including morning headaches, day-time sleepiness, swelling in the legs, etc. If the patient's vital capacity is less than 15% of predicted and they are asymptomatic, I have found it worthwhile to screen for evidence of nocturnal hypoventilation, usually with oximetry in the home.

One patient I had was a young man with Werdnig-Hoffmann's disease who had complaints of morning headaches, fatigue, malaise, and a vital capacity of about 200 ccs, with CO2 elevation on room air at rest. An overnight sleep study showed severe repetitive desaturation associated with nonobstructive hypopneas. He was admitted to the respiratory ward (I do not use the ICU) to initiate nocturnal ventilation with nasal CPAP and an LP6 ventilator. He had difficulty with oral air leaks, nasal bridge irritation, and, because, he had supported his head lying down, he had an ear irritation that required some adjustments and foam rubber. However, after nearly four and a half years of successful nocturnal nasal ventilation, he developed a pneumothorax – a collapse of one of his lungs. He is one of two patients that I have seen with delayed onset barotrauma-related pneumothorax after years of ventilation, something which is not yet discussed in the literature.

My preference is to use a home ventilator as opposed to BIPAP® for several reasons, not the least of which is that it irritates me that the home care companies amortize the cost of BIPAP® in about six months, and I feel certain that is one of the reasons why they push it. In addition, there are no alarms on BIPAP®, and nasal resistance can vary dramatically from day to day and hour to hour. I prefer to know that my patients are getting the tidal volume that I want them to have all the time.

Other complications in addition to pneumothorax and nasal irritation include dryness and congestion which usually respond to nasal irrigation with decongestants and application of humidification. Mask leak causing eye irritation in its most dramatic form can cause actual ulceration of the cornea. Gastric distention occurs in nearly everyone at the onset, but in most people it goes away in about 10 days.

Noninvasive ventilation is important, but it is not the whole answer, and I think tracheostomy ventilation has an undeservedly bad rap. One of my patients, a young woman with muscular dystrophy, presented with symptoms suggesting nocturnal hypoventilation. She underwent initiation of nasal ventilation and had trouble with an oral air leak during deep sleep. She also had mild nares discomfort. For reasons that I am unable to explain she had substantial troubles with post-ventilation discharge from her nose.

Subsequently an episode of pneumonia resulted in emergency intubation and mechanical ventilation. Thereafter, despite repeated attempts, it was not possible to liberate her from the ventilator, and she underwent tracheostomy. This was complicated by difficulty in obtaining an adequately sized trach tube in her very narrow trachea that would allow speech. Today, she is very active, maintaining a telemarketing business and advising people with disabilities on a variety of subjects including air travel. She herself has flown successfully all over the USA and to Europe.

The Passy-Muir Tracheostomy Speaking Valve can improve the quality of speech for those who are able to be ventilated with either cuff down or cuffless tracheostomy ventilation. However, the same result can be achieved by putting a cork in the exhalation circuit of the ventilator; it is completely feasible, disposable, and costs about a nickel. In our experience with Passy-Muir valves, we found that multiple interventions were necessary by both physician and nursing staff, and included changing the tidal volume to adjust for leak, changing the respiratory rate, diminishing the ventilator sensitivity, and changing the trach size which was often the most time-consuming intervention. We concluded that Passy-Muir valves, though quite labor intensive, are safe and highly successful if the patient is intelligent and highly motivated, and patients with neuromuscular disease are much more likely to succeed than are people with obstructive lung disease.

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