Children with juvenile rheumatoid arthritis (IRA) often exhibit fatigue and prolonged exercise recovery. Improved fitness through physical conditioning has not been a goal of standard medical or physical treatment regimens for IRA, and fitness levels of children with IRA have rarely been studied. We compared physical fitness in 20 6 to 11-year-old patients with polyarticular JRA with sex-, age-, and size-matched controls, using the Health Related Physical Fitness Test (HRPFTJ, (I national, standardized, norm-referenced test. We correlated fitness scores with summary joint counts, and with a n articular severity index (sum of joint swelling, tenderness, pain, and limited range for each child). The results showed that children with polyarticular IRA were less physically fit than normally active (noncompetitively athletic) children of the same sex, age, and size. There was no statistically significant relationship between increased joint counts, andlor disease severity scores, and reduced fitness scores. This suggests that physical' fitness levels are less related to degree of "disease activity" than is often thought. We conclude tha,t ( 1 ) a readily available, nationally standardized fitness test can be used to assess children with IRA: and [Z)fitness levels and measures of disease activity do not correlate. We believe that multiple factors, perhaps including family, physician, and school concerns about potential disease exacerbation following exercise, may account for the low fitness levels observed in children with IRA.
Background and Purpose. This investigation identified ventilation distribution, gas mixing, lung function, and arterial blood oxyhemoglobin saturation (Spo2) physiologic responses to 2 independent airway clearance treatments, high-frequency chest wall oscillation (HFCWO) and low positive expiratory pressure (PEP) breathing, for subjects who had cystic fibrosis (CF) and who were hospitalized during acute and subacute phases of a pulmonary exacerbation. Subjects. Fifteen subjects with moderate to severe CF were included in this study. Methods. Subjects performed single-breath inert gas tests and spirometry before and immediately after HFCWO and PEP breathing at admission and discharge. Arterial blood oxyhemoglobin saturation was monitored throughout each treatment. Results. At admission and discharge, PEP breathing increased Spo2 during treatment, whereas HFCWO decreased Spo2 during treatment. Ventilation distribution, gas mixing, and lung function improved after HFCWO or PEP breathing. Discussion and Conclusion. High-frequency chest wall oscillation and PEP breathing are similarly efficacious in improving ventilation distribution, gas mixing, and pulmonary function in hospitalized people with CF. Because Spo2 decreases during HFCWO, people who have moderate to severe CF and who use HFCWO should have Spo2 monitored during an acute exacerbation.
Background and Purpose. Individuals with cystic fibrosis (CF) have large amounts of infected mucus in their lungs, which causes irreversible lung tissue damage. Although patient-administered positive expiratory pressure (PEP) breathing has been promoted as an effective therapeutic modality for removing mucus and improving ventilation distribution in these patients, the effects of PEP on ventilation distribution and gas mixing have not been documented. Therefore, this preliminary investigation described responses in distribution of ventilation and gas mixing to PEP breathing for patients with moderate to severe CF lung disease. Subjects and Methods. The effects of PEP breathing on ventilation distribution, gas mixing, lung volumes, expiratory airflow, percentage of arterial blood oxyhemoglobin saturation (Spo 2 ), and sputum volume were studied in 5 patients with CF (mean ageϭ18 years, SDϭ4, rangeϭ13-22) after no-PEP, low-PEP (10 -20 cm H 2 O), and high-PEP (Ͼ20 cm H 2 O) breathing conditions. Single-breath inert gas studies and lung function tests were performed before, immediately after, and 45 minutes after intervention. Single-breath tests assess ventilation distribution homogeneity and gas mixing by observing the extent to which an inspired test gas mixes with gas already residing in the lung. Results. Improvements in gas mixing were observed in all PEP conditions. By 45 minutes after intervention, the no-PEP group improved by 5%, the low-PEP group improved by 15%, and the high-PEP group improved by 23%. Slow vital capacity increased by 1% for no PEP, by 9% for low PEP, and by 13% for high PEP 45 minutes after intervention. Residual volume decreased by 13% after no PEP, by 20% after low PEP, and by 30% after high PEP. Immediate improvements in forced expiratory flow during the middle half of the forced vital capacity maneuver (FEF 25%-75% ) were sustained following high PEP but not following low PEP. Discussion and Conclusion. This study demonstrated the physiologic basis for the efficacy of PEP therapy. The results confirm that low PEP and high PEP improve gas mixing in individuals with CF, and these improvements were associated with increased lung function, sputum expectoration, and Spo 2 . The authors propose that improvements in gas mixing may lead to increases in oxygenation and thus functional exercise capacity. [Darbee JC, Ohtake PJ, Grant BJB, Cerny FJ. Physiologic evidence for the efficacy of positive expiratory pressure as an airway clearance technique in patients with cystic fibrosis. Phys Ther. 2004;84:524 -537.]
An alternating-condition, single-subject research design was used to examine the effect of ambulation on three measures of school performance in three students with myelomeningocele. The subjects, aged 9, 10, and 15 years, had a physiological cost index greater than 1.00 beats per meter when walking with crutches or a walker. Subjects propelled a wheelchair at school for 5 days, ambulated with crutches or a walker for 5 days, and propelled a wheelchair for an additional 5 days. Performance in reading fluency, visuomotor accuracy, and manual dexterity was assessed at the end of each school day. Results were graphed and analyzed using the two-standard-deviation band method. All subjects had significantly lower visuomotor accuracy scores during the assistive-device ambulation phase than during the wheelchair phases. Performance in manual dexterity during the assistive-device ambulation and wheelchair phases varied among the subjects. Reading fluency was not affected by method of mobility. The results suggest that the high energy cost of walking may have a negative effect on certain aspects of the subjects' school performance. [Franks CA, Palisano RJ, Darbee JC. The effect of walking with an assistive device and using a wheelchair on school performance in students with myelomeningocele.
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