To Whom It May Concern:

_______ is a 9 month old girl with a diagnosis of spinal muscular atrophy type 1. The most recent reevaluation was on 4/11/01. The respiratory examination was significant for underexpansion of the chest and lungs with paradoxical chest wall motion on inspiration.

Assessment: This patient has chronic inspiratory muscle dysfunction that results in ventilatory insufficiency and severe expiratory muscle (cough) insufficiency. Because of severe, progressive neuromuscular disease, unless the patient's family is 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. Without the use of nocturnal nasal ventilation this patient will also develop a pectus excavatum and severe undergrowth and deformity of the lungs and chest wall.

Intervention: We trained this patient's family in nasal 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 (1,2), and this patient has been trained in their use, the following equipment is now required for home use:

• 1. A BiPAP-ST to use on IPAP 15-20, EPAP 3, rate 14, overnight and whenever sleeping.
• 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 (1,2).
• 3. A mechanical insufflator-exsufflator to clear airway secretions as necessary.
• 4. A manual resuscitator for air insufflations to assist coughing.
• 5. A chest percussor.
• 6. Portable suction equipment.

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.

2) Bach JR, Niranjan V. Spinal muscular atrophy type 1: a noninvasive respiratory management approach. Chest 2000;117:1100-1105.

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