gms | German Medical Science

Objective: Cystic fibrosis (CF) is a genetic autosomal recessive disease that influences many systems in the body, associated with decline of pulmonary function, pancreatic insufficiency, and important physical limitation. One aspect of the care of this chronic condition is the management of the systemic consequences of the disease, including exercise intolerance and skeletal muscle weakness. In this sense it has been developed a functional recovery program to analyze its effect over the muscle strength, exercise tolerance and quality life of a patient with CF after a hospital admission of 4 weeks.

Material/Methods: Prospective, longitudinal and descriptive study. Exercise tolerance, skeletal muscle strength and respiratory function were studied in a female subject with CF heterozygous for DF508 (age 21 years; height 164 cm; weight 54.4 kg; body mass index (BMI) 20.22 kg/m²; body fat mass 38.6%; Forced Expiratory Volume in one second (FEV1) 62% predicted). The CF diagnosis was verified by sweat testing (Cl, 60 mEq/L), clinical criteria, and genetic analysis. The following tests were performed: horizontal jumping ability; hand-grip strength; abdominal strength; a functional test of leg muscle endurance; and exercise tolerance the 6-Minute Walk (6MWT). To analyse the health-related quality of life (HRQL) Cystic Fibrosis Questionnaire-Revised (CFQ-R) was used. The intervention had a duration of 12 weeks, with a frequency of 3 days per week with training session of 60min. Workouts were based on aerobic volume capacity 50–65% heart rate reserve (HRR) and strength training (combining dynamic, reactivate and isometric method).

Results: The results of this study suggest a significant improvement in exercise tolerance (increase 190 meters in 6MWT) and static strength near to 80 % in the upper extremity and of 130 % in the strength resistance of the lower extremity. In relation to the FEV1 a moderate increase was observed after the physical activity period. Significant quality life improvements (CFQ-R) were found after long-term physical exercise program. Also, there were significant decreases in fat mass of 7.4 points (Table 1 [Tab. 1], Table 2 [Tab. 2], Table 3 [Tab. 3]).

Conclusion: The results of this study suggest that a physical exercise program of 16 weeks has significant influence on increasing a muscular strength, lung function, cardio-respiratory fitness and quality of life in a patient with CF. Therefore this intervention could be recommended in the functional recovery of CF patients. Further studies are needed to evaluate whether the small differences might be related to metabolic abnormalities in skeletal muscles in CF patients.