Thoracic Mobility in Cystic Fibrosis Care

Abstract

Introduction: Cystic fibrosis (CF) is an incurable and highly life-limiting hereditary disease. However, due to significant medical advancements over recent decades, life expectancy has risen to close to 50 years. As such, CF has evolved from mainly a respiratory and digestive system disease in children, to a multi-organ disease in adults. Consequently, there is both a need and an opportunity to focus more on previously understudied areas of CF, such as musculoskeletal aspects of respiration. Hence, the overall aim was to explore musculoskeletal aspects of respiration in CF, with thoracic mobility as the starting point.

Methods: In Paper I, a retrospective single-blinded cohort study was conducted, in which 344 high-resolution computed tomography images were assessed to investigate structural changes to the thorax throughout life with CF. In Paper II, functional musculoskeletal aspects of CF were assessed and compared between a group of 21 people with CF and 42 healthy controls. In Paper III, 15 people with CF took part in a pilot intervention study that explored manual therapy through eight weekly 30-minute sessions as complementary care to their ongoing physiotherapy regimen. In Paper IV, experiences of musculoskeletal aspects of CF and of manual therapy were explored through 10 semi-structured interviews.

Results: Structural changes to thoracic configuration were heterogeneous on a group level and no correlation was found between thoracic area measurements and lung function. Compared to healthy controls, thoracic expansion was decreased, and the number of musculoskeletal tender points was increased in a group of participants with CF. The same comparison also displayed a trend of decreased respiratory muscle strength in the CF group compared to healthy controls. Pre-test-post-test measures following manual therapy showed a decrease in the number of tender points, an increase in respiratory muscle strength and tendencies of increased thoracic mobility. No change in lung function measures was noted. When interviewed, respondents reported experiences of musculoskeletal symptoms as part of everyday life and that manual therapy had a positive effect on pain and stiffness and had also led to increased body awareness and refinement of self-care practices.

Conclusions: Thoracic structural changes varied, with no correlation to lung function. People with CF showed a smaller thoracic expansion and increased number of tender points compared to healthy controls. Respiratory muscle strength also tended to be lower in the CF group. Manual therapy mainly showed positive effects regarding subjective outcome measures such as musculoskeletal soreness, ease of breathing and increased body awareness, which also led to positive changes in self-care practices.