Inspiratory muscle training prior to peripheral muscle training in children and adolescents with Cystic Fibrosis.

Cystic fibrosis (CF) primarily affects the respiratory and digestive systems in children and young adults. Due to the continual bronchial airway obstruction a chronic hyperinflation of the thorax develops, thereby decreasing the efficiency of inspiratory muscle work and increasing work of breathing (WOB) in rest and during exercise. The increased WOB and the corresponding fatigue of the inspiratory muscles (diaphragm and supportive inspiratory muscles) are thought to induce a so called ‘respiratory muscle induced metaboreflex’ causing a reflex vasoconstriction of the locomotor muscle blood vessels. It is feasible that this decreased blood supply to the locomotor muscles will limit exercise capacity.
The hypothesis of this study is therefore: A home-based peripheral muscle training program (Five Basic Exercises program (5BX)) is more effective in [a] increasing exercise capacity (e.g. peak work rate) and [b] patients’ preferred occupational performance when it is preconditioned by inspiratory muscle training (IMT).

A home-based peripheral muscle training program (Five Basic Exercises program (5BX)) is more effective in [a] increasing exercise capacity (e.g. peak work rate) and [b] patients’ preferred occupational performance when it is preconditioned by inspiratory muscle training (IMT).

1. t= 0 (baseline);

2. t= 6 weeks;

3. t= 12 weeks;

4. t= 18 weeks;

5. t= 24 weeks.

Both groups receive inspiratory muscle training (IMT) or sham (placebo)-IMT based on randomization. IMT last for 6 weeks, 5 days a week, 11 minutes a day.

After IMT or sham-IMT, both groups receive a standardized peripheral muscle training programme (5 Basic eXercises (5BX)) for 6 weeks, 5 days a week, 11 minutes a day.