The new recommendations for respiratory management of SMA
In 2018 the new recommendations on the management of SMA were published and the management of the respiratory aspects was organized on the basis of the natural history and the classification of the patient
In the event that the patient presents weakness of the inspiratory and expiratory muscles and bulbar muscles, the pulmonary function tests will be performed including the determination of the cough peak flow and the strength test of the respiratory muscles. In these conditions, moreover, it will be necessary to start training for cough assistance maneuvers. In the event of respiratory disorders occurring during sleep, associated with the presence of ineffective cough or peak flow during reduced cough, chest X-rays are performed, sleep studies and non-invasive ventilation are initiated. Polysomnography or nocturnal polygraphy should be performed every 6 months in children with vital capacity <60% of expected value, otherwise at least once a year.
Non-invasive ventilation should be performed continuously when ventilatory insufficiency also occurs during the day, due to the increased risk of death in such conditions. The frequency of evaluation always depends on the clinical status and the stage of progression of the disease for each individual. In young children, whose rate of disease progression is uncertain, or in older children who have demonstrated clinical deterioration or who suffer from repeated infections or who develop symptoms of disordered sleep, the sleep assessment may be more frequent than once a year .
In patients with type 2 SMA, on the other hand, functional tests are scheduled according to the patient's age and the frequency of measurements is established in relation to the child's condition.
Finkel RS, Mercuri E, Meyer OH, et al. Diagnosis and management of spinal muscular atrophy: Part 2: Pulmonary and acute care; medications, supplements and immunizations; other organ systems; and ethics. Neuromuscular Disorders 2018; 28: 197-207.