Exercise, physical activity, and cystic fibrosis

Abstract

Cystic fibrosis (CF) is the most common genetic autosomal recessive disease of the Caucasian race, generally leading to death in early adulthood.1 The frequency of the gene carrier (heterozygote) is 1:20–25 in Caucasian populations, 1:2000 in African-Americans, and practically non-existent in Asian populations. The disease occurs in about 1 in every 2500 life births of the white population. Mean survival has risen from 8.4 years in 1969 to 32 years in 2000 due to improvements in treatment. The genetic defect causes a pathological electrolyte transport through the cell membranes by a defective chloride channel membrane transport protein [cystic fibrosis transmembrane conductance regulator (CFTR)]. With respect to the function, this affects mainly the exocrine glands of secretory cells, sinuses, lungs, pancreas, liver, and the reproductive tract of the human body leading to a highly viscous, water-depleted secretion. The secretion cannot leave the glands and in consequence causes local inflammation and destruction of various organs. The main symptoms include chronic inflammatory pulmonary disease with a progressive loss of lung function, exocrine and sometimes endocrine pancreas insufficiency, and an excessive salt loss through the sweat glands.1 A summary of the signs and symptoms of CF will be given with a special emphasis on the effect of exercise performance and capacity.

Health-related quality of life of Spanish children with cystic fibrosis

“…To prevent rapid health deterioration in people with CF, a healthy lifestyle is vital. Sufficient calorie intake is stimulated because a healthy body weight is positively related to exercise capacity, pulmonary function, and survival [ 4 , 5 ]. A sex- and age-adjusted body mass index (BMI) percentile ≥50 is recommended [ 5 ].…”

“…A sex- and age-adjusted body mass index (BMI) percentile ≥50 is recommended [ 5 ]. Regular physical activity (PA) is encouraged, as PA may slow the decline in pulmonary function [ 6 ], improve the ability to cope with activities of daily living, and prolong survival [ 4 ]. However, not only quantity, but also quality of life is an issue of concern in this chronically ill population.…”