ABSTRACT

Background: Respiratory muscles impairment is the main cause of morbidity and mortality in neuromuscular disease (NMD) patients. Pediatric pulmonologist must be involved in an opportune and standardized evaluation to minimize complications.
Objectives: 1) to know, by asking a group of pediatric pulmonologist, the time between the NMD diagnosis and the request for the pediatric pulmonologist’s evaluation; and 2) to investigate the diagnostic and therapeutic approach.
Method: Transversal clinical research. A questionnaire was administered to pediatric pulmonologist who attended the Annual Meeting in August of 2011.
Results: 33 responses were collected (21.4% of pediatric pulmonologist in the country). The majority indicated that patients were referred to them 6 to 12 months after being diagnosed; their approach had consisted of cardiological, orthopedic and swallow disorders evaluation; the majority recommend immunization to prevent pneumococcal and influenza infections. Pulse oximetry was the most used functional test (96.9% of the cases), followed by spirometry (60.6%), measurement of maximal inspiratory and expiratory pressures (18.1%) and peak cough flow (12.1%); 16 pulmonologist (48.4%) indicated lack of opportune non-invasive mechanical ventilation, and 25 (75.7%) performed assisted cough techniques to improve mucociliary clearance.
Conclusions: Patients with NMD are referred late to the pediatric pulmonologist. Evaluation for the strength of respiratory muscles are rarely performed, and non-invasive mechanical ventilation is underused. This study highlights the urgent need for physicians, to comply with the guides to handling NMD.

KEYWORDS

Dystrophy, neuromuscular disease, pulmonary function test, diagnosis, treatment.

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