Pulmonary rehabilitation improves exercise capacity in subjects with kyphoscoliosis and severe respiratory impairment.

BACKGROUND Patients with kyphoscoliosis and severe respiratory impairment frequently experience reduction in exercise tolerance, limitation in daily life activities, and deterioration in health-related quality of life (HRQOL). Noninvasive ventilation (NIV) as an add-on treatment to long-term oxygen therapy (LTOT) was shown to improve symptoms and HRQOL in these patients. Pulmonary rehabilitation can increase exercise capacity and HRQOL in patients with COPD, but its role in patients with restrictive thoracic disease, such as kyphoscoliosis, is uncertain. The aim of this study was to analyze the effects of combining pulmonary rehabilitation with LTOT and NIV treatments on arterial blood gases and the 6-min walk test (6MWT) in a homogeneous group of subjects with kyphoscoliosis. METHODS Twenty-three subjects with kyphoscoliosis and respiratory failure who were being treated with both LTOT and NIV and who had been referred to a pulmonary rehabilitation program were retrospectively analyzed. Eighteen subjects were included, and there was no control group. Pulmonary rehabilitation involved educational and physical training sessions and was carried out daily for 4-6 weeks. Exercise intensity was personalized based on individual tolerance, physiologic parameters, or physiotherapist judgment. RESULTS Upon completion of pulmonary rehabilitation, a significant improvement in 6-min walk distance was observed (P = .04). The dyspnea score at the end of the 6MWT improved as well, although the improvement did not reach statistical significance (P = .06). These changes were not confirmed at a 12-month follow-up visit. No significant effects of pulmonary rehabilitation on arterial blood gases were observed. CONCLUSIONS A combined intervention including a tailored pulmonary rehabilitation program together with LTOT and NIV seems to be of short-term benefit in subjects with kyphoscoliosis and severe respiratory impairment.