Evaluation of supported upper limb exercise capacity in patients with cystic fibrosis.

Physiological responses to upper limb exercise have not been well documented in patients with cystic fibrosis (CF). This is the first study to quantify ventilatory responses to supported incremental upper limb exercise in this patient group. Twenty-four subjects with CF, with a wide range of pulmonary impairment, and ten normal control subjects were studied. Subjects performed pulmonary function tests and incremental arm and leg exercise to peak work capacity on an arm crank and bicycle ergometer. All subjects performed less work with the arms than legs. At an equivalent oxygen consumption, ventilation was higher for arm work than leg work. This higher ventilation was achieved mainly through a higher frequency of breathing. Only CF subjects with severe pulmonary impairment (FEV1 < 40% predicted, FEF25-75% < 20% predicted) had a reduced arm work capacity compared with control subjects. At peak arm work, these subjects had a mean ventilation to maximum voluntary ventilation ratio (VE/MVV) of 106% +/- 25, while maximum heart rate was less than 80% predicted. Despite the high ventilatory requirement for arm exercise, arm work capacity was well maintained in subjects with CF until severe lung disease impaired the ability to further increase ventilation.