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Hospital Therapy Improves Exercise Tolerance and Lung Function in Cystic Fibrosis FREE

Frank J. Cerny, PhD; Gerd J. A. Cropp, MD, PhD; Michael R. Bye, MD
[+] Author Affiliations

Reprint requests to Children's Lung Center, Children's Hospital of Buffalo, 219 Bryant St, Buffalo, NY 14222 (Dr Cerny).


Am J Dis Child. 1984;138(3):261-265. doi:10.1001/archpedi.1984.02140410039013.
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• We studied the benefits of in-hospital therapy on exercise capacity and related these changes to improvements in lung function in 17 patients with cystic fibrosis (CF) of moderate to extreme severity, as defined by results of pulmonary function tests performed at admission and discharge. Tolerance and adaptations to exercise were assessed from measures of peak work capacity (PWC), peak heart rate (PHR), and peak ventilation (PVE) obtained during an incremental exercise test. Treatment lasted from nine to 18 days. All measures of lung function improved; there also were significant increases in PWC, PHR, PVE, and PHR/PWC. Exercise-induced arterial desaturation was less at discharge than at admission. The PWC of the most severely affected patients remained abnormally low, and their adaptations to exercise were abnormal at discharge. We concluded that intensive in-hospital therapy will significantly improve exercise tolerance and lung function in patients with CF with moderate to severe pulmonary dysfunction.

(AJDC 1984;138:261-265)

REFERENCES

Matthews LW, Doershuk CF, Wise M, et al:  A therapeutic regimen for patients with cystic fibrosis . J Pediatr 1964;;65:558.
Doershuk CF, Matthews LW, Tucker AS, et al:  Evaluation of a prophylactic and therapeutic program for patients with cystic fibrosis . Pediatrics 1965;;36:675-688.
Strieder D, Khaw KT, Simpser M, et al: Inhospital treatment of chronic lung disease in cystic fibrosis: Improved lung function. Read before the Seventh International Conference on Cystic Fibrosis, Paris, 1976.
Redding G, Restuccia R, Cotton E, et al:  Serial pulmonary functions in children hospitalized with cystic fibrosis . Am Rev Respir Dis 1982;;126:31-36.
Gardner RM:  Snowbird workshop on standardization of spirometry: A statement by the American Thoracic Society . Am Rev Respir Dis 1979;;119:831-838.
DuBois AB, Botelho SY, Bedell GN, et al:  A rapid plethysmographic method for measuring thoracic gas volume: A comparison with a nitrogen washout method for measuring functional residual capacity in normal subjects . J Clin Invest 1956;;35:322.
DuBois AB, Botelho SY, Comroe JH Jr:  A new method for measuring airway resistance in man using a body plethysmograph: Values in normal subjects and in patients with respiratory distress . J Clin Invest 1956;;35:327.
Polgar G, Promadhat V: Pulmonary Function Testing in Children: Techniques and Standards . Philadelphia, WB Saunders Co, 1971;.
Zapletal A, Motoyama E, Cook C, et al:  Maximum expiratory volume curves and airway conductance in children and adolescents . J Appl Physiol 1969;;26:308.
Weng TR, Levison H:  Standards of pulmonary function in children . Am Rev Respir Dis 1969;;99:879.
Bates DV, Macklem PT, Christie RV: Respiratory Function in Disease . Philadelphia, WB Saunders Co, 1971;, pp 93-94.
Briscoe WA, DuBois AB:  Relationships between airway resistance, airway conductance and lung volume in subjects of different ages and body size . J Clin Invest 1958;;37:1279.
Cropp GJ, Pullano TP, Cerny FJ, et al:  Exercise tolerance and cardiorespiratory adjustments at peak work capacity in cystic fibrosis . Am Rev Respir Dis 1982;;126:211-216.
Borg G:  Perceived exertion: A note on history and methods . Med Sci Sports Exerc 1973;;5:90-93.
Mihevic P:  Sensory cues for perceived exertion: A review . Med Sci Sports Exerc 1981;;13: 150-163.
Snedecor GW, Cochran WG: Statistical Methods , ed 6. Ames, Iowa, Iowa State University Press, 1967;.
McFadden ER, Linden DA:  A reduction in maximum midexpiratory flow rate: A spirographic manifestation of small airway disease . Am J Med 1972;;52:725.
Nickerson BG, Lemen RJ, Gerdes CB, et al:  Within-subject variability and percent change for significance of spirometry in normal subjects and in patients with cystic fibrosis . Am Rev Respir Dis 1980;;122:859-866.
Taussig LM, Kattwinkel J, Friedewald WT, et al:  A new prognostic score and clinical evaluation system for cystic fibrosis . J Pediatr 1973;;82:380-388.
Cerny FJ, Pullano T, Cropp GJA:  Adaptation to exercise in children with cystic fibrosis , in Nagle F, Montoya H (eds): Exercise in Health and Disease . Springfield, Ill, Charles C Thomas Publisher, 1981;, pp 36-42.
Cerny FJ, Pullano TP, Cropp GJA:  Cardiorespiratory adaptations to exercise in cystic fibrosis . Am Rev Respir Dis 1982;;126:217-220.

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References

Matthews LW, Doershuk CF, Wise M, et al:  A therapeutic regimen for patients with cystic fibrosis . J Pediatr 1964;;65:558.
Doershuk CF, Matthews LW, Tucker AS, et al:  Evaluation of a prophylactic and therapeutic program for patients with cystic fibrosis . Pediatrics 1965;;36:675-688.
Strieder D, Khaw KT, Simpser M, et al: Inhospital treatment of chronic lung disease in cystic fibrosis: Improved lung function. Read before the Seventh International Conference on Cystic Fibrosis, Paris, 1976.
Redding G, Restuccia R, Cotton E, et al:  Serial pulmonary functions in children hospitalized with cystic fibrosis . Am Rev Respir Dis 1982;;126:31-36.
Gardner RM:  Snowbird workshop on standardization of spirometry: A statement by the American Thoracic Society . Am Rev Respir Dis 1979;;119:831-838.
DuBois AB, Botelho SY, Bedell GN, et al:  A rapid plethysmographic method for measuring thoracic gas volume: A comparison with a nitrogen washout method for measuring functional residual capacity in normal subjects . J Clin Invest 1956;;35:322.
DuBois AB, Botelho SY, Comroe JH Jr:  A new method for measuring airway resistance in man using a body plethysmograph: Values in normal subjects and in patients with respiratory distress . J Clin Invest 1956;;35:327.
Polgar G, Promadhat V: Pulmonary Function Testing in Children: Techniques and Standards . Philadelphia, WB Saunders Co, 1971;.
Zapletal A, Motoyama E, Cook C, et al:  Maximum expiratory volume curves and airway conductance in children and adolescents . J Appl Physiol 1969;;26:308.
Weng TR, Levison H:  Standards of pulmonary function in children . Am Rev Respir Dis 1969;;99:879.
Bates DV, Macklem PT, Christie RV: Respiratory Function in Disease . Philadelphia, WB Saunders Co, 1971;, pp 93-94.
Briscoe WA, DuBois AB:  Relationships between airway resistance, airway conductance and lung volume in subjects of different ages and body size . J Clin Invest 1958;;37:1279.
Cropp GJ, Pullano TP, Cerny FJ, et al:  Exercise tolerance and cardiorespiratory adjustments at peak work capacity in cystic fibrosis . Am Rev Respir Dis 1982;;126:211-216.
Borg G:  Perceived exertion: A note on history and methods . Med Sci Sports Exerc 1973;;5:90-93.
Mihevic P:  Sensory cues for perceived exertion: A review . Med Sci Sports Exerc 1981;;13: 150-163.
Snedecor GW, Cochran WG: Statistical Methods , ed 6. Ames, Iowa, Iowa State University Press, 1967;.
McFadden ER, Linden DA:  A reduction in maximum midexpiratory flow rate: A spirographic manifestation of small airway disease . Am J Med 1972;;52:725.
Nickerson BG, Lemen RJ, Gerdes CB, et al:  Within-subject variability and percent change for significance of spirometry in normal subjects and in patients with cystic fibrosis . Am Rev Respir Dis 1980;;122:859-866.
Taussig LM, Kattwinkel J, Friedewald WT, et al:  A new prognostic score and clinical evaluation system for cystic fibrosis . J Pediatr 1973;;82:380-388.
Cerny FJ, Pullano T, Cropp GJA:  Adaptation to exercise in children with cystic fibrosis , in Nagle F, Montoya H (eds): Exercise in Health and Disease . Springfield, Ill, Charles C Thomas Publisher, 1981;, pp 36-42.
Cerny FJ, Pullano TP, Cropp GJA:  Cardiorespiratory adaptations to exercise in cystic fibrosis . Am Rev Respir Dis 1982;;126:217-220.

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