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Role of conventional physiotherapy in cystic fibrosis
ORIGINAL ARTICLES
Role of conventional physiotherapy in cystic fibrosis John J. Reisman, MD, Betty R M n g t o n - L a w , BSc, PT, Mary Corey, MSc, J a c q u e s M a r c o t t e , MD, Eleanor W a n n a m a k e r , Dip PT, Dawn Harcourt, MSc, a n d Henry Levison, MD From the Division of Respiratory Diseases, Research Institute of the Hospital for Sick Children, Toronto, Ontario, Canada Because of the time and the emotional cost involved in performing daily conventional chest physiotherapy in patients with cystic fibrosis, a 3-year prospective Study was undertaken to c o m p a r e the long-term effects of postural d r a i n a g e a c c o m p a n i e d by percussion and the forced expiratory technique with the effects of the forced expiratory technique alone. Patients who performed the forced expiratory technique alone had mean annual rates of decline that were significantly different from zero for forced expiratory volume in 4 second (p <0.004), forced expiratory flow between 25% and 75% of vital c a p a c i t y (p <0.004), and Shwachman clinical score (p <0.004). In the group performing conventional physiotherapy with percussion and postural drainage, only the mean annual rate of decline for forced expiratory flow between 25% and 75% of vital c a p a c i t y was significantly different from zero (p <0.03), and it was significantly different from the mean rate of decline associated with the forced expiratory technique alone (p <0.04). We conclude that conventional chest physiotherapy should remain a standard component of therapy in cystic fibrosis. (J PEDIATR1988;113:632-6)
The use of chest physiotherapy in the form of postural drainage with mechanical percussion is a traditional component of the daily therapeutic management of patients with cystic fibrosis. This technique facilitates the expectoration of mucus from the respiratory tract, 1-7 but its effect on the rate of pulmona/ry deterioration characteristic of this disease is not known. The short-term effects of chest physiotherapy in CF patients have been reported. Lorin and Denning2 found that chest physiotherapy produced significantly more sputum than cough alone. Wong et al. 8 reported that gravity alone helped mobilize secretions in CF patients. Many reports of improvements in pulmonary function tests in CF patients after chest physiotherapy can be found.9-13 However, Kerrebijn et al. ~4,15failed to show any difference in Supported in part by grant aid from the Canadian Federal Department of Health and Welfare. Submitted for publication Feb. 12, 1988; accepted April 19, t 988. Reprint requests: Henry Levison,MD, Hospital for Sick Children, 555 University Ave., Toronto, Ontario M5G 1X8, Canada.
632
pulmonary function tests in CF patients after the use of chest physiotherapy. No significant benefits of chest physiotherapy have been demonstrated in studies of patients with chronic bronchitis and uncomplicated pneumonia.7,16 CF CPT
FEF25_75 FET FEV~ FVC
W~aax
Cystic fibrosis Chest physiotherapy (used with forced~ expiratory technique) Forced expiratory flow between 25% and 75% of vital capacity Forced expiratory technique Forced expiratory volume in 1 second Forced vital capacity Maximum work capacity
There are no long-term studies showing that this form of therapy alters the course of CF. Desmond et al. 17reported immediate and sustained benefits of chest physiotherapy in eight CF patients with mild to moderately severe airflow limitation who were followed during alternating 3-week periods in which physiotherapy was either given or not.
Volume 113 Number 4 However, the number of patients was very small, the study period was short, and the observed treatment effect may have been due in part to inhaled medication that accompanied chest physiotherapy and that was discontinued along with the physiotherapy. The cost of daily home chest physiotherapy is significant in terms of the time spent and the emotional involvement of family members. Compliance with chest physiotherapy is significantly lower than any other aspect of routine CF therapy, and family conflict over this regimen is well known, especially during adolescence, is The forced expiratory technique, which is an alternative method for stimulating cough and mobilizing secretions, requires less time and energy expenditure than conventional chest physiotherapy.lg. 20 At the Hospital for Sick Children, in Toronto, Canada, approximately 80% of the CF clinic population of over 500 patients routinely use conventional chest physiotherapy. Because of the time and emotional costs involved, it was decided that this therapy should be evaluated in a controlled trial. A 3-year prospective study was undertaken, to compare the long-term effects of postural drainage accompanied by percussion and FET with the effects of FET alone. METHODS Patients from the Hospital for Sick Children CF clinic, with an established diagnosis of C F 1 based on elevated sweat chloride levels, characteristic clinical findings, or a positive family history, were considered to be possible subjects if they met the inclusion criteria: (1) age between 7 and 21 years and (2) forced expiratory volume in 1 second greater than 40% of the predicted value for height and sex. 21 The study was confined to patients with mild to moderate pulmonary disease because the object of the study was to determine whether rates of deterioration differ with the two modes of therapy; it was thought that differences would not be detected in patients with more severe pulmonary involvement. Patients involved in any other studies at that time were excluded. The subjects and their families were informed about the study protocol, and written informed consent was obtained in accordance with the policy of the Hospital for Sick Children Human Ethics Committee. Sixty-seven subjects were enrolled in the study. To provide balanced study groups, we stratified patients according to sex, age (7 to 11, 12 to 16, and 17 to 21 years), and pulmonary impairment (mild: FEV1 >80% predicted; moderate: FEVi 40% to 80% predicted). Subjects were randomly assigned within each stratum to one of two groups. One group was instructed to continue conventional chest physiotherapy according to their previous routine and to perform FET as well (The CPT group). The other
Physiotherapy in eystic fibrosis
633
Table I. Characteristics of patients on entry into study
No. of subjects Male/female ratio Age (yr) FVC (%predicted) FEV1 (%predicted) FEF25.7~(%predicted) Weight for height (%) Shwachman score
FET
CPT
33 20/13 11.8 _+ 3.0 87.8 - 13.1 89.5 ___ 13.4 86.3 -+ 24.2 98.7 +-- 10.1 91.7 _+ 6.9
30 18/12 12.6 _+ 4.2 86.7 +_ 13.5 88.0 + 18.6 80.1 + 30.1 99.7 ___9.8 91.7 + 7.4
Values reportedare mean +- SD.
group was instructed to discontinue percussion and postural drainage but to do FET only (FET group). All subjects were counseled by the study physiotherapist on the importance of deep breathing with maximal expiratory effort and coughing throughout their inhalational therapy, which consisted of ~2-bronchodilator inhalation delivered by mask and compressor. The subjects were then instructed on the technique of FET, which consisted of two maximal inspirations, each followed by a prolonged, controlled, forced expiration, and then three normal quiet inspirations, each followed by a prolonged, controlled, forced expiration. A minimum of three coughs was pe rformed, or coughs were performed until there was no more sputum to expectorate. The patients receiving CPT were instructed in the routine postural drainage positions to be performed each day, with 8 minutes of percussion per position. 2:,23 Percussion was performed manually or with the aid of a mechanical percussor. Several brands of percussor were used, but all had similar stroke force and frequency. All subjects were advised to participate in as much physical activity and sport as they could. Activity was monitored by the use of an annual questionnaire. All subjects received, in addition to verbal instructions, a written outline of their specific protocol, and they were asked to keep a diary reporting adherence to their physiotherapy regimen. Compliance was evaluated annually and scored with the scoring system of Passero et al. is Subjects were followed every 3 months to monitor health status. Acute exacerbations were treated in the hospital with vigorous physiotherapy and intravenous administered antibiotics. Once their condition stabilized, subjects were returned to their study groups. Pulmonary function studies were performed twice a year in conjunction with scheduled clinic visits. Spirometric measurements were made with the use of a computerized spirometer with unidirectional hot-wire flow detection (Gould Sentry System 80, Gould Inc., Saddlebrook, N.J.). Variables measured included forced vital capacity, F E V , and forced expiratory flow between 25% and 75% of vital
634
Reisman et al.
The Journal of Pediatrics October 1988
T a b l e II. Individual regression slopes of pulmonary function and clinical scores
FVC (%predicted/yr) FEV1 (%predicted/yr) FEF25_75(%predicted/yr) Shwaehman score (units/yr)
FET
CPT
FET vsCPT
-1.6 + 5.7 (NS) -4.7 _-2-7.1 (p
0.2 - 4.9 (NS) -1.9 _+ 5.8 (NS) -4.2 _+ 9.5 (p <0.03)* 1.5 + 5.0 (NS)
NS p <0.08 p <0.04 NS
Values are mean _+SD. NS, Not significant. *Significantlydifferentfrom zero. T a b l e III. Hospitalizations during study period Pulmonary exacerbations
No. of subjects No. of admissions Hospital days
RESULTS
Other admissions
FET
CPT
FET
CPT
8 (+1)* 15 (+15) 197 (+150)
5 8 73
8 12 43
7 10 48
*Values for one extraordinarycase in parentheses(see text).
capacity. Results were expressed as a percent of predicted value for sex and height. 21 Posteroanterior and lateral chest radiographs were obtained every 6 months and were scored by the study physician using the scoring system of Brasfield et al. 24 At each clinic visit the physician Calculated a score according to Shwachman et al., 25incorporating the most recent x-ray score. S p u t u m production was also monitored at each clinic visit by asking subjects to collect all their sputum for the 24 hours before their clinic visit. A Jones stage 1 graded exercise test 26 was performed with the use of an electronically braked cycle ergometer. When possible; testing was done when subjects entered the study and again after at least 1 year. Maximum work capacity was recorded, and heart rate and respiratory rate were monitored throughout exercise. Statistical analysis was carried out with use of the SAS statistical analysis system37 Least-squares linear regression was used to calculatelrates of decline in pulmonary function and clinical score for each subject. For comparison of baseline mean values and mean slopes of decline, the Student t test was used. The number of admissions and days spent in the hospital Were compared by means of the Mann-Whitney test because a large proportion of patients had no admissions. Categorical variables were compared by chi-square analysis. Potential risk factors predicting decline in pulmonary function were investigated by logistic regression analysis.
Sixty-seven subjects were initially recruited into the study. Four of these subjects (two in each group) withdrew: two sisters who were in the percussion-and-posturaldrainage group moved to another city 6 months after the study began, and the two subjects in the FET group withdrew after 5 and 6 months, respectively, because of family anxiety associated with discontinuation of the Conventional CPT. Neither of these subjects had demonstrated any physical decline. The two groups were similar with respect to age and sex distribution, and clinical status (Table I). The male/ female ratio was the same as that of the Hospital for Sick Children CF clinic population, reflecting better overall survival figures for male CF patients. Mean values for FVC, FEVt, and FEF2~_75reflected the selection of patients with only mild to moderate pulmonary impairment. Shwachman scores also reflected the good clinical status of the study population. Weight-for-height values were in the normal range. Bacteriologic studies revealed that the two groups were comparable with respect to organisms colonizing the sputum. Eighty-four percent (87% FET, 80% CPT) of the subjects were colonized with strains of Pseudomonas aeruginosa, whereas Pseudomonas cepacia was present in the sputa of 13% (5% lbET, 16% CPT). Treatments that were being administered at home were also similar in the two groups. All the CPT subjects and 94% of the FET subjects required pancreatic enzyme supplements. All of the CPT group and 97% of the FET group took daily antistaphylococcal antibiotics, and both groups used inhaled/32-bronchodilators regularly (93% of the CPT group and 91% of the FET group). Physiotherapy was prescribed for all patients in twice-daily sessions, although eight FET patients (24%) and five CPT patients (17%) reported doing all their therapy in one daily session. The mean number of minutes per day spent on physiotherapy before the study period was the same in both groups (35.7 + 16.6 FET, 36.0 _+ 12.2 CPT).
Volume 113 Number 4
Mean duration of follow-up, measured in years, was similar in both groups (2.4 _+ 0.4 FET, 2.4 + 0.4 CPT). Patients who did FET alone had mean rates of decline that differed significantly from zero for FEV~, FE~25.75, and Shwachman clinical score. In the CPT group, only FEF~5.75 decline differed significantly from zero and from the mean rate for FET alone (Table II). The number of hospitalizations and days in the hospital for nonpulmonary reasons were very similar in the two groups (Table III). These admissions were mainly for polypectomies or treatment of meconium ileus equivalent. o n l y five (1~/%) of the CPT group and nine (27%) of the FET group were hospitalized for pulmonary exacerbations. The differences in number of patients, number of hospitalizations, and days spent in the hospital were not statistically significant. The data for one patient in the FET group are reported separately in parentheses because her case was extreme, and her 15 admissions were for treatment of respiratory illness complicated by social problems. Two other patients in the FET group accounted for 43 and 60 days, respectively, in three admissions each. Twenty-five subjects underwent initial and follow-up graded exercise challenge tests. Some of the younger subjects were unwilling to complete the exercise test. In the 25 subjects completing the testing, the baseline CPT mean Wmax was 98 +_ 30% of the predicted Value and the FET group mean was 109 _+ 24% of the predicted value (the normal values reported by Godfrey et al? 8 were used for the calculations). Follow-up values were 99 + 23% and 94 + 19% of predicted values in the CPT and FET groups, respectively. The changes did not differ significantly (p = 0.10). To examine whether several potential risk factors could account for deteriorating pulmonary function and the differences between the two study groups, we classified each patient as declining or stable on the basis of the slope of FEV~ versus time being less than or greater than the median value of - 3 % predicted/yr. Other numerical variables were also dichotomized according to median values. Factors examined were sex, age (<12 years; 12 and older), sputum production (--<5 ml/day, >5 ml/day), activity level (low, average, high), initial FEV~ (<88% or >__88% predicted, the median value) and the presence or absence of P. cepacia in the sputum. None of these factors, alone or in combination, was significantly associated with declining FEV1, in either treatment group or in the total group. All but three patients were judged to be consistently compliant with their therapyl Two patients in the FET group who were sporadically noncompliant had declining pulmonary function. The one consistently noncompliant patient in the CPT group had stable pulmonary function throughout the study. Both groups performed an average
Physiotherapy in cystic fibrosis
635
level of physical activity (11 to 15 hr/wk), with no difference between the groups. DISCUSSION The results of this 3-year study demonstrate that subjects who discontinued CPT and did FET alone had a significantly greater degree of decline in their FEF25_75than the group performing conventional CPT. The rates of decline in the CPT group were very similar to those in a previous study in which the patients were drawn from a similarly selected subgroup of the CF clinic population? 9 Several studies have shown short-term improvement in pulmonary function tests, and others have shown no difference. This study strongly suggests that the long-term course of pulmonary function is adversely affected when conventional physiotherapy is abandoned. It is disappointing, but perhaps not surprising, that we were unable to provide a predictive profile for patients at risk of pulmonary decline if CPT is discontinued. The patients included in this study were a select group in whom the chronic progressive respiratory symptoms of CF were not yet firmly established. The factors that trigger this progressive deterioration and contribute to its extremely variable rate are still poorly understood. It has been Suggested that because exercise programs improve the pulmonary status of some patients with CF, 3~ the routine use of physiotherapy may be abandoned. Until the role of regular exercise and fitness programs has been studied longitudinally, we believe that conventional chest physiotherapy with percussion and postural drainage should remain a standard part of CF therapy. Even in patients in whom symptoms and sputum production were minimal, there is a strong suggestion that pulmonary disease progressed more rapidly when conventiona! CPT was discontinued. REFERENCES
1. MaeLusky I, McLaughlin FJ, Levison H. Cystic fibrosis. Part I; vol. XV. Chicago: Year Book Medical, 1985:6. 2. Lorin MI, Denning CR. Evaluation of postural drainage by measurement of sputum volume and consistency. Am J Phys Rehabil Med 1971;50:215-9. 3. Mellins RB. Pulmonary physiotherapy in the pediatric age group. Am Rev Respir Dis t974;110:137-42. 4. Rochester DF, Goldberg SK. Techniques of respiratory physical therapy. Am Rev Respir Dis !980;122:133-46. 5. Cherniack RM. Physical therapy. Am Rev Respir Dis 1980;122:25-7. 6. Jones NL. Physical therapy: present state of the art. Am Rev Resp Dis 1974;110:132-6. 7. Murray JF. The ketchup-bottle method [Editorial]. N Engl J Med 1979;300:1155-7. 8. Wong JW, Keens. TG, Wannamaker EM, Douglas PT, Levison H, Aspen N. Effects of gravity on tracheal transport rates in normal subjects and in subjects with cystic fibrosis. Pediatrics 1977;60:146-52.
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9. Motoyama EK. Assessment of lower airway obstruction in cystic fibrosis. In: Mangis JA, Talamo R, eds. Fundamental problems in cystic fibrosis and related diseases. Miami: Symposia Specialists, 1973;335-43. 10. Feldman J, Traver GA, Taussig LM. Maximal expiratory flows after postural drainage. Am Rev Respir Dis 1979;119:239-45. 11. Weller PH, Bush E, Preece MA, Norman AP, Matthew DJ. The short-term effects of chest physiotherapy on lung function tests in children with cystic fibrosis. Respiration 1980; 40:53-6. 12. Clark SW, Cochrane GM, Weber B. Effects of sputum on pulmonary function. Thorax 1973;28:262. 13. Cochrane GM, Weber BA, Clark SW. Effects of sputum on pulmonary function. Br Med J 1977;2:1181-3. 14. Kerrebijn KF, Veentjer R, Bonjet VD, Water E. Effect of four physiotherapy schedules on pulmonary function in children with cystic fibrosis. Monogr Pediatr 1979;10:60. 15. Kerrebijn KF, Veentjer R, Bonjet VD, Water E. The immediate effect of physiotherapy and aerosol treatment on pulmonary function in children with cystic fibrosis. Eur J Respir Dis 1982;63:35-42. 16. Graham WGB, Bradley DA. Efficacy of chest physiotherapy and intermittent positive pressure breathing in the resolution of pneumonia. N Engl J Med 1978;229:624-7. 17. Desmond K J, Schwenk WF, Thomas E, Beaudry PH, Coates AL. Immediate and long-term effects of chest physiotherapy in patients with cystic fibrosis. J PEDIATR 1983;103:538-42. 18. Passero MA, Remor B, Solomon J. Patient-reported compliance with cystic fibrosis therapy. Clin Pediatr 1981;20:2648. 19. Sutton PP, Parker PA, Webber BA. Assessment of the forced expiratory technique, postural drainage and directed coughing in chest physiotherapy. Eur J Respir Dis 1983;64:62-8. 20. Verboon JML, Bakker W, Sterk PJ. The value of forced expiration technique with and without postural drainage in
The Journal of Pediatrics October 1988
21. 22. 23. 24.
25.
26. 27. 28.
29.
30.
31.
32.
adults with cystic fibrosis. Eur J Respir Dis 1986;69:16974. Weng TR, Levison H. Standards of pulmonary function in children. Am Rev Respir Dis 1969;99:879. Gaskell DV, Weber BA. The Brompton Hospital guide to chest physiotherapy. Oxford: Blackwell Scientific, 1972. Thacker W. Postural drainage. London: Lloyd-Luke, 1972. Brasfield D, Hicks G, Soong S, Tiller RE. The chest roentgenogram in cystic fibrosis: a new scoring system. Pediatrics 1979;63:24-9. Shwaehman H, Kulcnycki LL. Long-term study of one hundred five patients with cystic fibrosis: studies made over a five- to fourteen-year period. Am J Dis Child 1958;96:6-15. Jones NL, Campbell M. Clinical exercise testing. Philadelphia: WB Saunders, 1982. SAS Institute. SAS user's guide: Basics, version 5 ed. Cary, N.C.: SAS Institute, 1985. Godfrey S, Davies CTM, Wonjnaik E, Barnes CA. Cardiorespiratory response to exercise in normal children. Clin Sci 1971;40:419-31. Nolan G, Mclvor P, Levison H, Fleming PC, Corey M, Gold R. Antibiotic prophylaxis in cystic fibrosis: inhaled cephaloridine as an adjunct to cloxacillin. J PEDIATR 1982;101:62630. Orenstein DM, Franklin BA, Doershuk CF, et al. Exercise conditioning and cardiopulmonary fitness in cystic fibrosis. Chest 1981;80:392-8. Keens TG, Krastins I, Wannamaker E, Levison H, Crozier DN, Bryan AC. Ventilatory muscle endurance training in normal subjects and patients with cystic fibrosis. Am Rev Respir Dis 1977;116:853-60. Andreasson B, Jonson B, Kornfalt R, Nordmark E, SandStrom S. Long-term effects of physical exercise on working capacity and pulmonary function in cystic fibrosis. Acta Pediatr Scand 1987;76:70-5
Role of conventional physiotherapy in cystic fibrosis John J. Reisman, MD, Betty R M n g t o n - L a w , BSc, PT, Mary Corey, MSc, J a c q u e s M a r c o t t e , MD, Eleanor W a n n a m a k e r , Dip PT, Dawn Harcourt, MSc, a n d Henry Levison, MD From the Division of Respiratory Diseases, Research Institute of the Hospital for Sick Children, Toronto, Ontario, Canada Because of the time and the emotional cost involved in performing daily conventional chest physiotherapy in patients with cystic fibrosis, a 3-year prospective Study was undertaken to c o m p a r e the long-term effects of postural d r a i n a g e a c c o m p a n i e d by percussion and the forced expiratory technique with the effects of the forced expiratory technique alone. Patients who performed the forced expiratory technique alone had mean annual rates of decline that were significantly different from zero for forced expiratory volume in 4 second (p <0.004), forced expiratory flow between 25% and 75% of vital c a p a c i t y (p <0.004), and Shwachman clinical score (p <0.004). In the group performing conventional physiotherapy with percussion and postural drainage, only the mean annual rate of decline for forced expiratory flow between 25% and 75% of vital c a p a c i t y was significantly different from zero (p <0.03), and it was significantly different from the mean rate of decline associated with the forced expiratory technique alone (p <0.04). We conclude that conventional chest physiotherapy should remain a standard component of therapy in cystic fibrosis. (J PEDIATR1988;113:632-6)
The use of chest physiotherapy in the form of postural drainage with mechanical percussion is a traditional component of the daily therapeutic management of patients with cystic fibrosis. This technique facilitates the expectoration of mucus from the respiratory tract, 1-7 but its effect on the rate of pulmona/ry deterioration characteristic of this disease is not known. The short-term effects of chest physiotherapy in CF patients have been reported. Lorin and Denning2 found that chest physiotherapy produced significantly more sputum than cough alone. Wong et al. 8 reported that gravity alone helped mobilize secretions in CF patients. Many reports of improvements in pulmonary function tests in CF patients after chest physiotherapy can be found.9-13 However, Kerrebijn et al. ~4,15failed to show any difference in Supported in part by grant aid from the Canadian Federal Department of Health and Welfare. Submitted for publication Feb. 12, 1988; accepted April 19, t 988. Reprint requests: Henry Levison,MD, Hospital for Sick Children, 555 University Ave., Toronto, Ontario M5G 1X8, Canada.
632
pulmonary function tests in CF patients after the use of chest physiotherapy. No significant benefits of chest physiotherapy have been demonstrated in studies of patients with chronic bronchitis and uncomplicated pneumonia.7,16 CF CPT
FEF25_75 FET FEV~ FVC
W~aax
Cystic fibrosis Chest physiotherapy (used with forced~ expiratory technique) Forced expiratory flow between 25% and 75% of vital capacity Forced expiratory technique Forced expiratory volume in 1 second Forced vital capacity Maximum work capacity
There are no long-term studies showing that this form of therapy alters the course of CF. Desmond et al. 17reported immediate and sustained benefits of chest physiotherapy in eight CF patients with mild to moderately severe airflow limitation who were followed during alternating 3-week periods in which physiotherapy was either given or not.
Volume 113 Number 4 However, the number of patients was very small, the study period was short, and the observed treatment effect may have been due in part to inhaled medication that accompanied chest physiotherapy and that was discontinued along with the physiotherapy. The cost of daily home chest physiotherapy is significant in terms of the time spent and the emotional involvement of family members. Compliance with chest physiotherapy is significantly lower than any other aspect of routine CF therapy, and family conflict over this regimen is well known, especially during adolescence, is The forced expiratory technique, which is an alternative method for stimulating cough and mobilizing secretions, requires less time and energy expenditure than conventional chest physiotherapy.lg. 20 At the Hospital for Sick Children, in Toronto, Canada, approximately 80% of the CF clinic population of over 500 patients routinely use conventional chest physiotherapy. Because of the time and emotional costs involved, it was decided that this therapy should be evaluated in a controlled trial. A 3-year prospective study was undertaken, to compare the long-term effects of postural drainage accompanied by percussion and FET with the effects of FET alone. METHODS Patients from the Hospital for Sick Children CF clinic, with an established diagnosis of C F 1 based on elevated sweat chloride levels, characteristic clinical findings, or a positive family history, were considered to be possible subjects if they met the inclusion criteria: (1) age between 7 and 21 years and (2) forced expiratory volume in 1 second greater than 40% of the predicted value for height and sex. 21 The study was confined to patients with mild to moderate pulmonary disease because the object of the study was to determine whether rates of deterioration differ with the two modes of therapy; it was thought that differences would not be detected in patients with more severe pulmonary involvement. Patients involved in any other studies at that time were excluded. The subjects and their families were informed about the study protocol, and written informed consent was obtained in accordance with the policy of the Hospital for Sick Children Human Ethics Committee. Sixty-seven subjects were enrolled in the study. To provide balanced study groups, we stratified patients according to sex, age (7 to 11, 12 to 16, and 17 to 21 years), and pulmonary impairment (mild: FEV1 >80% predicted; moderate: FEVi 40% to 80% predicted). Subjects were randomly assigned within each stratum to one of two groups. One group was instructed to continue conventional chest physiotherapy according to their previous routine and to perform FET as well (The CPT group). The other
Physiotherapy in eystic fibrosis
633
Table I. Characteristics of patients on entry into study
No. of subjects Male/female ratio Age (yr) FVC (%predicted) FEV1 (%predicted) FEF25.7~(%predicted) Weight for height (%) Shwachman score
FET
CPT
33 20/13 11.8 _+ 3.0 87.8 - 13.1 89.5 ___ 13.4 86.3 -+ 24.2 98.7 +-- 10.1 91.7 _+ 6.9
30 18/12 12.6 _+ 4.2 86.7 +_ 13.5 88.0 + 18.6 80.1 + 30.1 99.7 ___9.8 91.7 + 7.4
Values reportedare mean +- SD.
group was instructed to discontinue percussion and postural drainage but to do FET only (FET group). All subjects were counseled by the study physiotherapist on the importance of deep breathing with maximal expiratory effort and coughing throughout their inhalational therapy, which consisted of ~2-bronchodilator inhalation delivered by mask and compressor. The subjects were then instructed on the technique of FET, which consisted of two maximal inspirations, each followed by a prolonged, controlled, forced expiration, and then three normal quiet inspirations, each followed by a prolonged, controlled, forced expiration. A minimum of three coughs was pe rformed, or coughs were performed until there was no more sputum to expectorate. The patients receiving CPT were instructed in the routine postural drainage positions to be performed each day, with 8 minutes of percussion per position. 2:,23 Percussion was performed manually or with the aid of a mechanical percussor. Several brands of percussor were used, but all had similar stroke force and frequency. All subjects were advised to participate in as much physical activity and sport as they could. Activity was monitored by the use of an annual questionnaire. All subjects received, in addition to verbal instructions, a written outline of their specific protocol, and they were asked to keep a diary reporting adherence to their physiotherapy regimen. Compliance was evaluated annually and scored with the scoring system of Passero et al. is Subjects were followed every 3 months to monitor health status. Acute exacerbations were treated in the hospital with vigorous physiotherapy and intravenous administered antibiotics. Once their condition stabilized, subjects were returned to their study groups. Pulmonary function studies were performed twice a year in conjunction with scheduled clinic visits. Spirometric measurements were made with the use of a computerized spirometer with unidirectional hot-wire flow detection (Gould Sentry System 80, Gould Inc., Saddlebrook, N.J.). Variables measured included forced vital capacity, F E V , and forced expiratory flow between 25% and 75% of vital
634
Reisman et al.
The Journal of Pediatrics October 1988
T a b l e II. Individual regression slopes of pulmonary function and clinical scores
FVC (%predicted/yr) FEV1 (%predicted/yr) FEF25_75(%predicted/yr) Shwaehman score (units/yr)
FET
CPT
FET vsCPT
-1.6 + 5.7 (NS) -4.7 _-2-7.1 (p
0.2 - 4.9 (NS) -1.9 _+ 5.8 (NS) -4.2 _+ 9.5 (p <0.03)* 1.5 + 5.0 (NS)
NS p <0.08 p <0.04 NS
Values are mean _+SD. NS, Not significant. *Significantlydifferentfrom zero. T a b l e III. Hospitalizations during study period Pulmonary exacerbations
No. of subjects No. of admissions Hospital days
RESULTS
Other admissions
FET
CPT
FET
CPT
8 (+1)* 15 (+15) 197 (+150)
5 8 73
8 12 43
7 10 48
*Values for one extraordinarycase in parentheses(see text).
capacity. Results were expressed as a percent of predicted value for sex and height. 21 Posteroanterior and lateral chest radiographs were obtained every 6 months and were scored by the study physician using the scoring system of Brasfield et al. 24 At each clinic visit the physician Calculated a score according to Shwachman et al., 25incorporating the most recent x-ray score. S p u t u m production was also monitored at each clinic visit by asking subjects to collect all their sputum for the 24 hours before their clinic visit. A Jones stage 1 graded exercise test 26 was performed with the use of an electronically braked cycle ergometer. When possible; testing was done when subjects entered the study and again after at least 1 year. Maximum work capacity was recorded, and heart rate and respiratory rate were monitored throughout exercise. Statistical analysis was carried out with use of the SAS statistical analysis system37 Least-squares linear regression was used to calculatelrates of decline in pulmonary function and clinical score for each subject. For comparison of baseline mean values and mean slopes of decline, the Student t test was used. The number of admissions and days spent in the hospital Were compared by means of the Mann-Whitney test because a large proportion of patients had no admissions. Categorical variables were compared by chi-square analysis. Potential risk factors predicting decline in pulmonary function were investigated by logistic regression analysis.
Sixty-seven subjects were initially recruited into the study. Four of these subjects (two in each group) withdrew: two sisters who were in the percussion-and-posturaldrainage group moved to another city 6 months after the study began, and the two subjects in the FET group withdrew after 5 and 6 months, respectively, because of family anxiety associated with discontinuation of the Conventional CPT. Neither of these subjects had demonstrated any physical decline. The two groups were similar with respect to age and sex distribution, and clinical status (Table I). The male/ female ratio was the same as that of the Hospital for Sick Children CF clinic population, reflecting better overall survival figures for male CF patients. Mean values for FVC, FEVt, and FEF2~_75reflected the selection of patients with only mild to moderate pulmonary impairment. Shwachman scores also reflected the good clinical status of the study population. Weight-for-height values were in the normal range. Bacteriologic studies revealed that the two groups were comparable with respect to organisms colonizing the sputum. Eighty-four percent (87% FET, 80% CPT) of the subjects were colonized with strains of Pseudomonas aeruginosa, whereas Pseudomonas cepacia was present in the sputa of 13% (5% lbET, 16% CPT). Treatments that were being administered at home were also similar in the two groups. All the CPT subjects and 94% of the FET subjects required pancreatic enzyme supplements. All of the CPT group and 97% of the FET group took daily antistaphylococcal antibiotics, and both groups used inhaled/32-bronchodilators regularly (93% of the CPT group and 91% of the FET group). Physiotherapy was prescribed for all patients in twice-daily sessions, although eight FET patients (24%) and five CPT patients (17%) reported doing all their therapy in one daily session. The mean number of minutes per day spent on physiotherapy before the study period was the same in both groups (35.7 + 16.6 FET, 36.0 _+ 12.2 CPT).
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Mean duration of follow-up, measured in years, was similar in both groups (2.4 _+ 0.4 FET, 2.4 + 0.4 CPT). Patients who did FET alone had mean rates of decline that differed significantly from zero for FEV~, FE~25.75, and Shwachman clinical score. In the CPT group, only FEF~5.75 decline differed significantly from zero and from the mean rate for FET alone (Table II). The number of hospitalizations and days in the hospital for nonpulmonary reasons were very similar in the two groups (Table III). These admissions were mainly for polypectomies or treatment of meconium ileus equivalent. o n l y five (1~/%) of the CPT group and nine (27%) of the FET group were hospitalized for pulmonary exacerbations. The differences in number of patients, number of hospitalizations, and days spent in the hospital were not statistically significant. The data for one patient in the FET group are reported separately in parentheses because her case was extreme, and her 15 admissions were for treatment of respiratory illness complicated by social problems. Two other patients in the FET group accounted for 43 and 60 days, respectively, in three admissions each. Twenty-five subjects underwent initial and follow-up graded exercise challenge tests. Some of the younger subjects were unwilling to complete the exercise test. In the 25 subjects completing the testing, the baseline CPT mean Wmax was 98 +_ 30% of the predicted Value and the FET group mean was 109 _+ 24% of the predicted value (the normal values reported by Godfrey et al? 8 were used for the calculations). Follow-up values were 99 + 23% and 94 + 19% of predicted values in the CPT and FET groups, respectively. The changes did not differ significantly (p = 0.10). To examine whether several potential risk factors could account for deteriorating pulmonary function and the differences between the two study groups, we classified each patient as declining or stable on the basis of the slope of FEV~ versus time being less than or greater than the median value of - 3 % predicted/yr. Other numerical variables were also dichotomized according to median values. Factors examined were sex, age (<12 years; 12 and older), sputum production (--<5 ml/day, >5 ml/day), activity level (low, average, high), initial FEV~ (<88% or >__88% predicted, the median value) and the presence or absence of P. cepacia in the sputum. None of these factors, alone or in combination, was significantly associated with declining FEV1, in either treatment group or in the total group. All but three patients were judged to be consistently compliant with their therapyl Two patients in the FET group who were sporadically noncompliant had declining pulmonary function. The one consistently noncompliant patient in the CPT group had stable pulmonary function throughout the study. Both groups performed an average
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level of physical activity (11 to 15 hr/wk), with no difference between the groups. DISCUSSION The results of this 3-year study demonstrate that subjects who discontinued CPT and did FET alone had a significantly greater degree of decline in their FEF25_75than the group performing conventional CPT. The rates of decline in the CPT group were very similar to those in a previous study in which the patients were drawn from a similarly selected subgroup of the CF clinic population? 9 Several studies have shown short-term improvement in pulmonary function tests, and others have shown no difference. This study strongly suggests that the long-term course of pulmonary function is adversely affected when conventional physiotherapy is abandoned. It is disappointing, but perhaps not surprising, that we were unable to provide a predictive profile for patients at risk of pulmonary decline if CPT is discontinued. The patients included in this study were a select group in whom the chronic progressive respiratory symptoms of CF were not yet firmly established. The factors that trigger this progressive deterioration and contribute to its extremely variable rate are still poorly understood. It has been Suggested that because exercise programs improve the pulmonary status of some patients with CF, 3~ the routine use of physiotherapy may be abandoned. Until the role of regular exercise and fitness programs has been studied longitudinally, we believe that conventional chest physiotherapy with percussion and postural drainage should remain a standard part of CF therapy. Even in patients in whom symptoms and sputum production were minimal, there is a strong suggestion that pulmonary disease progressed more rapidly when conventiona! CPT was discontinued. REFERENCES
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