Pulmonary rehabilitation for patients with spinal muscular atrophy type Ⅱ

Abstract

  Objective: Respiratory care management is necessary for patients with spinal muscular atrophy (SMA), and training regarding respiratory rehabilitation is routinely provided for those patients as well as their caregivers by our hospital. In the present study, we examined the significance of respiratory rehabilitation in SMA type Ⅱ patients by performing sequential evaluations of their respiratory functions. Methods: We retrospectively evaluated sequential changes in vital capacity (VC), cough peak flow (CPF), and maximum insufflation capacity (MIC) in 13 SMA type Ⅱ patients, as well as CPF accompanied with MIC (MIC-CPF) in 8 such patients. Furthermore, we evaluated the rate of change for those parameters in 5 who underwent spinal fusion surgery before and after the operation. Results: The median age at the first evaluation was 6 years (range 3-17 years) and the median observation period was 4 years (2-9 years). VC level was less than 2,000 ml in each patient, while the MIC level exceeded 2,000 ml in only 1. None had a CPF or MIC-CPF level that exceeded 270 L/min, which is needed to promote expectoration, while those exceeded 160 L/min in 2 and 4 patients, respectively. The rate of change after spinal fusion surgery was −10.9% for VC (n=5), −0.25% for MIC (n=5), −7.3% for CPF (n=5), and 14.4% for MIC-CPF (n=4). Conclusions: Respiratory tract clearance is disturbed in SMA type Ⅱ patients due to respiratory function deterioration. To prevent pulmonary complications, it is important to continue respiratory rehabilitation from the early disease stage.