| Abstract: | OBJECTIVE: Respiratory complications are the main causes of morbidity and mortality in
patients with neuromuscular disease (NMD). The objectives of this study were to
determine the effects that routine daily home air-stacking maneuvers have on
pulmonary function in patients with spinal muscular atrophy (SMA) and in patients
with congenital muscular dystrophy (CMD), as well as to identify associations
between spinal deformities and the effects of the maneuvers. METHODS: Eighteen NMD patients (ten with CMD and eight with SMA) were submitted to routine
daily air-stacking maneuvers at home with manual resuscitators for four to six
months, undergoing pulmonary function tests before and after that period. The
pulmonary function tests included measurements of FVC; PEF; maximum insufflation
capacity (MIC); and assisted and unassisted peak cough flow (APCF and UPCF,
respectively) with insufflations. RESULTS: After the use of home air-stacking maneuvers, there were improvements in the APCF
and UPCF. In the patients without scoliosis, there was also a significant increase
in FVC. When comparing patients with and without scoliosis, the increases in APCF
and UPCF were more pronounced in those without scoliosis. CONCLUSIONS: Routine daily air-stacking maneuvers with a manual resuscitator appear to
increase UPCF and APCF in patients with NMD, especially in those without
scoliosis. |
