To Whom It May Concern:

____________ is a 49.0 year old woman with a diagnosis of myotonic dystrophy. The most recent reevaluation in the Jerry Lewis Muscular Dystrophy Association clinic was on 04/12/01. The respiratory examination was significant for underexpansion of the chest and lungs with a vital capacity of 1400.0 ml when sitting and 0.0 ml supine, maximum insufflation capacity (MIC) (air stacked breaths from a manual resuscitator) of 1900.0 ml, peak cough flow (PCF) 3.1 L/s from the MIC and 0.0 L/s from the MIC with the addition of an abdominal thrust. The SaO2 ranged from 94.0 to 95.0 percent. Maximum end-tidal pCO2 was 43.0 mm Hg.

Assessment: This patient has chronic inspiratory muscle failure that results in ventilatory insufficiency and severe expiratory muscle (cough) insufficiency. Because of severe, progressive neuromuscular disease, unless trained and equipped the patient will develop pneumonia and acute respiratory failure during every severe chest cold. This will require hospitalization, intensive care, intubation for respiratory support, and probably lead to pressure to undergo otherwise unnecessary tracheostomy.

Intervention: She will practice air stacking. She will be referred to PT for training in sliding board transfers. We trained this patient in nasal and mouthpiece intermittent positive pressure ventilation (IPPV), manually assisted coughing, and in mechanical insufflation-exsufflation. Since acute respiratory failure can be prevented with the use of these noninvasive inspiratory and expiratory muscle aids (1), and this patient has been trained in their use, the following equipment is now required for home use:

• 1. A portable volume ventilator (PLV-100) to use via mouthpiece during daytime hours while in the wheelchair and another to use at bedside via nasal interface for nocturnal assistance on assist control mode, rate 10, volume 900 to 1300 ml.
• 2. An oximeter for feedback to maintain SaO2 over 94% at all times with the use of noninvasive IPPV and assisted airway secretion elimination as needed (1).
• 3. A mechanical insufflator-exsufflator to clear airway secretions as necessary.

The patient may eventually need an occupational therapy evaluation for adaptive equipment, a ventilator tray installed on the wheelchair, and a mouthpiece held adjacent to the mouth by gooseneck clamp or telephone holder to facilitate insufflations and air stacking while sitting in the wheelchair.

Noninvasive IPPV and assisted airway secretion elimination should be used as needed to prevent oxyhemoglobin desaturation and, therefore, respiratory failure. If you have any questions, please feel free to contact me at: _____________.

John R. Bach, M.D.
Professor of Physical Medicine and Rehabilitation
Co-Director, Jerry Lewis Muscular Dystrophy Association Clinic of UMDNJ-NJMS

1) Bach JR et al. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest 1997;112:1024-1028.

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