Cystic fibrosis is an autosomal-recessive genetic disease that affects multiple organs with an ever-increasing life expectancy due to the actual ongoing advances in treatment. Patients affected by cystic fibrosis may present postural disorders such as thoracic hyperkyphosis and scoliosis, frequently associated with significant morbidity, back pain, poor body image, and low mood with an impact on quality of life. In this population, lung obstruction leading to air trapping and hyperinflation occur, causing alteration in posture to allow the chest and trunk muscles to assist with breathing. Physiotherapy based on airway clearance has a pivotal role in the respiratory management, but less is known about its application for postural abnormalities in these patients.
The review aims to determine if physical therapy improves quality of life, pain, trunk deformity, and pulmonary function in cystic fibrosis patients, including randomized controlled trials about any physical therapy relevant for postural rehabilitation, such as manual therapy, educational programs, exercise training (individualised or group-based or home-based), Pilates, stretching, Global Posture Re-education, Inspiratory Muscle Training, and yoga, compared with each other, no physical therapy, sham treatment, or usual care.
This review is important for patients with cystic fibrosis, their family / caregivers, health professionals dealing with cystic fibrosis, researchers, general practitioners, and policymakers.
Results show that manual therapy may make little or no difference in the change of trunk deformity (low-certainty evidence). No data could be analysed for quality of life (very low-quality evidence) and pain (very low-quality evidence) because of the high heterogeneity between trials, and it is uncertain whether the intervention improves lung function in terms of forced vital capacity (very low-quality evidence), forced expiratory volume in one second (very low-quality evidence), or Tiffeneau’s index.
Authors conclude that evidence is limited about the benefits of physical therapies on postural abnormalities in people with cystic fibrosis and further well-conducted studies are needed.
Commented by Maria Soriano Micò