Investigation of upper extremity exercise capacity muscle oxygenization balance and physical activity level in patients with cystic fibrosis

Cystic Fibrosis (CF) is a genetic disease that mostly affects the lungs, but also intestines, pancreas, liver and kidneys. Exercise intolerance, muscle weakness, mitochondrial involvement, and slow phosphocreatine metabolism are common, but extrapulmonary impairments in pediatric patients have not been adequately studied, yet. To compare respiratory functions (spirometer), functional exercise capacity (6-minute walking test (6-MWT), Pegboard and Ring test (6-PBRT), respiratory muscle strength (MIP, MEP) (mouth pressure device) and endurance (threshold loading test), peripheral muscle strength (dynamometer), muscle oxygenation (Moxy® monitor), static and dynamic balance (Biodex Biosway® Balance System, Y balance test), physical activity level (metabolic holter) and quality of life (Cystic Fibrosis Questionnaire Revised (CFQ-R)) in patients and healthy subjects was aimed. 28 patients and 23 healthy were compared. %FEV1, %FVC, %PEF, %FEF25-75%, MIP, muscle strength (%),CFQ-R subscales physical functionality, treatment burden and respiratory findings (6-13 years), the treatment burden score in patients (over 14 years) in scale was statistically (p<0.05), 6-MWT distance was clinically significantly (p>0.05) decreased in patients. Quadriceps femoris (QF) muscle oxygen saturation (SmO2min), hemoglobin level (Thbmin,max,top) during 6-MWT was statistically significantly lower, deltoid muscle THb at rest and ΔTHb during 6-PBRT were higher (p<0.05) in patients compared with controls. MEP, respiratory muscle endurance, 6-PBRT total ring count, QF and deltoid muscle resting SmO2, static postural stability indices and dynamic postural stability scores, physical activity parameter's score were similar in the groups (p>0.05). Lower extremity functional exercise capacity is clinically decreased, inspiratory, QF and deltoid muscle strength are decreased; respiratory functions, quality of life, physical functionality, respiratory symptoms and treatment burden sub-dimensions in patients aged 6-13 years and treatment burden sub-dimensions in patients over 14 years of age deteriorated in pediatric CF patients. The majority of patient and healthy children are physically inactive. Upper extremity exercise capacity, expiratory muscle strength, respiratory muscle endurance, static and dynamic balance of the patients are preserved. Lower extremity muscle oxygen saturation during submaximal activity is decreased, and lower and upper extremities hemoglobin are decreased less in patient. Pediatric CF patients should be evaluated for rehabilitation needs at the earliest and should be included in cardiopulmonary rehabilitation programs. Factors that decrease upper extremity exercise capacity and the effects of exercise training on muscle oxygen metabolism should be investigated in children with CF.