Summary. Exercise tolerance may be reduced in patients with cystic fibrosis, but it is not always possible to predict this from standard lung function measurements. Formal exercise testing may, therefore, be necessary, and the test should be simple and readily available. We have developed a ''3-minute step test'' and compared it with the standard 6-minute walking test. Subjects stepped up and down a 15-cm-high single step at a rate of 30 steps per minute for 3 minutes. The effect of the step test on spirometry was tested first in 31 children with CF (mean age, 12.0 years), who had a mean (range) baseline forced expired volume in 1 second (FEV 1 ) of 64% (18-94%) of predicted values. The step test was then compared with the standard 6-minute walk in a further 54 patients with cystic fibrosis (mean age, 12.5 years), with mean (range) baseline FEV 1 of 61% (14-103%) of predicted values. Outcome measures were minimum arterial oxygen saturation (SaO 2 ), maximum pulse rate, and the modified Borg dyspnea score.Post-step test spirometry showed mean (95% CI) changes of −1.1% (−6.0+3.9%) for forced vital capacity, of −1.6% (−4.2+1.1%) for FEV 1 , and +0.25% (−2.8+3.3%) for peak expiratory flow, although 5/31 children showed >15% drop in one or more parameters. The step and walk tests both produced significant changes (P < 0.0001) in all outcomes, with a mean (range) minimum SaO 2 of 92% (75-98%) versus 92% (75-97%), a maximum pulse rate of 145 b.p.m. (116-189) versus 132 (100-161), and a Borg score of 2.5 (0-9) versus 1.0 (0-5), respectively. Comparison of the two tests showed that the step test increased breathlessness (mean change Borg score, 2.3 vs. 0.8; P < 0.0001) and pulse rate (mean change, 38% vs. 24%, P < 0.0001) significantly more than the walk, whereas the decrease in SaO 2 was similar (mean change, −2.9% vs. −2.6%; P = 0.12). Some patients with a significant drop in SaO 2 (>4%) would not have the decrease predicted from their baseline lung function. Reproducibility for the two tests was similar. The step test is quick, simple and portable, and is not dependent on patient motivation. Although the step test is more tiring, its effect on SaO 2 is similar to the 6-minute walking test. It is a safe test that may prove to be a valuable measure of exercise tolerance in children with pulmonary disease, although longitudinal studies are now needed. Pediatr Pulmonol. 1998; 25:278-284.

Appropriate, regular exercise is of benefit to patients with cystic fibrosis (CF). As with other segments of the population, it has been difficult to devise exercise programs to which most patients will adhere for long periods of time. In healthy children, factors that are related to positive exercise compliance include social support, perceptions of competency and self-esteem, enjoyment of activity, and availability of a variety of activities. In patients with CF, complications associated with the disease, e.g., time required for other treatment and fatigue, make compliance with recommended exercise activities more difficult. Factors likely to increase compliance in this population include explicit and continued encouragement and support from the family and healthcare team, and the introduction of behavior-changing strategies.

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Urinary incontinence (UI) is recognized as a significant problem in adult females with cystic fibrosis and can often have a marked impact on day-to-day activities. The prevalence and severity of UI in the pediatric cystic fibrosis (CF) female population is less clear and there are no comparative data with healthy children or children with other respiratory disorders. An anonymous self-completed semi-structured questionnaire was used to study the prevalence rates of UI in girls with CF aged between 11 and 17 and compared it to age-matched asthmatic and healthy girls. The prevalence of UI in girls with CF was significantly higher (17/51, 33%) than the asthmatic (4/25, 16%) and healthy girls (2/27, 7%) (P = 0.02). It may manifest as early as 11 years of age and is associated with increasing lung disease. Surprisingly it is perceived as a relatively minor problem in terms of the distress it causes. Pediatric CF clinics should be routinely addressing UI as a potential problem in all girls from the age of 11 years.

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Cystic fibrosis-related diabetes (CFRD) is associated with a shortened life expectancy and greater deterioration in lung function than in CF patients with normal glucose metabolism. There are few published data on how CFRD affects growth in childhood. We carried out a retrospective case controlled study of growth and lung function in 34 children with CFRD attending three specialist centers in London. We found that for the 2 years leading to CFRD diagnosis (at a mean age of 13.1 years), the mean height velocity was significantly less compared to controls: 4.9 (standard deviation-SD 1.6) cm/year vs. 6.0 (SD 1.9) cm/year (P = 0.005). For the 2 years following diagnosis, height velocity remained significantly lower (3.4 (SD 2.2) cm/year vs. 4.4 (SD 2.2) cm/year, P = 0.02). Mean FEV(1) was reduced prior to diagnosis and at diagnosis, but was similar to controls 2 years after diagnosis. This study highlights the compromise in height velocity and lung function that occurs prior to diagnosis of CFRD in children with CF, and a reduction in height velocity should be considered an indicator of impaired glucose metabolism. It would be useful to know whether early treatment with insulin can help promote catch up growth.

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