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Clinical Investigations: CYSTIC FIBROSIS |

Impact of Recent Pulmonary Exacerbations on Quality of Life in Patients With Cystic Fibrosis* FREE TO VIEW

Maria T. Britto, MD, MPH; Uma R. Kotagal, MBBS, MSc; Richard W. Hornung, DrPH; Harry D. Atherton, MS; Joel Tsevat, MD, MPH; Robert W. Wilmott, MD, FCCP
Author and Funding Information

*From the Division of Adolescent Medicine (Dr. Britto), Health Policy and Clinical Effectiveness (Dr. Kotagal and Mr. Atherton), and Pulmonary Medicine, Allergy & Clinical Immunology (Dr. Wilmott), Children’s Hospital Medical Center, Cincinnati; Institute For Health Policy and Health Services Research (Dr. Hornung), and Division of General Internal Medicine (Dr. Tsevat), University of Cincinnati, Cincinnati, OH.

Correspondence to: Maria T. Britto, MD, MPH, Children’s Hospital Medical Center, Division of Adolescent Medicine, 3333 Burnet Ave, Cincinnati, OH; e-mail: maria.britto@chmcc.org



Chest. 2002;121(1):64-72. doi:10.1378/chest.121.1.64
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Objective: To compare the health-related quality of life (HRQOL) of people with cystic fibrosis (CF) to the general population, and to determine the relationship between HRQOL and clinical and demographic factors.

Design: Cross-sectional analysis of observational cohort.

Setting: Outpatient clinics of a Midwestern CF center.

Subjects: One hundred sixty-two subjects with CF aged 5 to 45 years.

Main outcome measures: Physical and psychosocial summary scores and individual scale scores for the Child Health Questionnaire and Short Form-36.

Results: Compared with the general population, people with CF reported similar scores for most psychosocial measures, but lower scores for most physical measures, with the lowest scores on the general health perceptions scale. In multivariable analyses, pulmonary exacerbations in the past 6 months were strongly associated with the physical (p = 0.001) and psychosocial (p = 0.0003) scores. The physical score fell, on average, 6 points per exacerbation and the psychosocial score fell 3 points. Lung function, nutrition, 6-min walk distance, age, gender, and insurance status were not significantly associated with HRQOL in this study population. Those who declined to participate had significantly lower FEV1 percent predicted and nutritional indexes. Our findings may not be generalizable to the entire CF population.

Conclusion: Recent pulmonary exacerbations have a profound negative impact on HRQOL that is not explained by differences in lung function, nutritional status, or demographic factors.

Figures in this Article

As treatment advances have improved the life expectancy of people with cystic fibrosis (CF), understanding the impact of the disease and its treatment on everyday function and well-being has assumed greater importance. Since 1987, the National Heart, Lung, and Blood Institute has recommended increased development and use of health-related quality of life (HRQOL) measures in CF care and research.1The World Health Organization has defined health as “a state of complete physical, mental and social well being and not merely the absence of disease or infirmity.”2 In this framework, five aspects of health are generally accepted as necessary for comprehensive measurement of HRQOL: physical health, mental health, social functioning, role functioning, and general health perceptions. Comprehensive HRQOL measures assess the individual’s ability to function in each area, and the individual’s evaluation of their functioning.

Measures of HRQOL that are valid and suited to the CF population are needed for research, for policy making, and for improving clinical care. HRQOL measures can serve as outcomes in clinical trials and studies of health-care effectiveness. They can also be used to determine the cost-effectiveness of new treatments. These latter analyses, which are based partly on patients’ assessment of HRQOL, may impact policy decisions regarding allocation of resources for different CF treatments, or for treatments for CF vs other conditions. HRQOL measures are especially important for interventions that may improve the patient’s well-being or ability to function, but that do not result in changes in FEV1 percent predicted or other traditional clinical outcomes. They also may be useful in assessing disease progression and monitoring the clinical course of individual patients. Finally, HRQOL measures can describe health outcomes in ways that are meaningful to patients and families as well as to health professionals.3

Among adults with a variety of chronic conditions, self-reported HRQOL has been found to be a powerful predictor of future health and social outcomes, such as hospital days, mortality, total health costs, and employment status.4In older adolescents and adults with CF, scores on the Nottingham Health Profile correlated with FEV1 percent predicted, breathlessness, and time receiving home antibiotic therapy.5 The RAND functional status index (RAND Health Insurance Study) by itself significantly predicted mortality in adults with CF over the following 5 years.6The quality of well-being scale correlated well with measures of pulmonary function and exercise tolerance in cross-sectional7and longitudinal8studies including both children and adults. A larger study of 199 pediatric patients with CF, however, found only low-to-moderate correlations between the quality of well-being scale and other measures of physical and psychosocial health.9 One consistent finding in all these earlier studies is that the correlation between FEV1 percent predicted and HRQOL, while usually significant, is typically weak.,3 These previous studies may have limited applicability to children and adolescents, since none of the measures used were developed for a pediatric population. In addition, most earlier studies used convenience samples that may not be representative of the underlying CF population. New HRQOL measures, specifically designed for patients with CF, have recently been developed for adults and adolescents10and for children.11 These measures may correlate more highly with physiologic outcomes and may be particularly suited to CF clinical trials. Generic HRQOL measures, however, will be important for comparing outcomes across diseases and for determining the relative cost-effectiveness of different treatments.

This study sought to build on previous work by using recently developed age-specific HRQOL measures and by attempting to include the entire population of a large center, rather than a convenience sample. The specific aims of this study were to determine the impact of CF on HRQOL, to compare HRQOL of a population with CF to the general population, and to determine the relationship between HRQOL and clinical and demographic factors. We hypothesized that CF would have a greater impact on physical than psychosocial functioning, and that physical functioning would be significantly associated with FEV1 percent predicted and with exercise capacity as measured by 6-min walk distance.

Study Population

Subjects were recruited from the pediatric and adult CF clinics of a midwestern medical school. All patients who were ≥ 5 years old and who had not undergone lung transplantation were invited to participate. Patients were enrolled during routine quarterly clinic visits. For those who had no clinic visits during the 27-month enrollment period or who were missed by study personnel, attempts were made to enroll by mail or telephone. Overall, 162 of 191 eligible patients (83.9%) participated. Three of these patients were enrolled by telephone. The remainder enrolled in the clinic. The participants, compared with those who declined, were significantly better nourished (body mass index [BMI] percentage of ideal, 98.5 vs 92.3; weight z score, − 0.5 vs − 1.2; height z score,− 0.6 vs − 1.2), had a higher FEV1 percent predicted (72.8 vs 58.6), and were more likely to have commercial insurance (81.6% vs 61.5%). They did not differ significantly with respect to age, gender, rates of colonization with Pseudomonas aeruginosa, or presence of diabetes mellitus.

Quality-of-Life Measures

The HRQOL measures used in this study were based on the World Health Organization conceptual model of health, which incorporates physical, mental, and social well-being. In addition to being derived from the same conceptual framework, the measures share similar construction and identical scaling of summary scores. Subjects < 18 years old completed the Child Health Questionnaire (CHQ).12 The Parent Form-50 (PF-50) of the CHQ consists of 50 items comprising 11 multi-item scales: physical function, role function as limited by physical problems, general health perceptions, bodily pain, role function as limited by social/emotional/behavioral problems, self esteem, mental health, behavior, family activities, parental impact-emotional, and parental impact-time. The parental-impact scales capture the amount of emotional distress and time limitation experienced by the parent due to the child’s physical health, emotional well-being, attention/learning abilities, ability to get along with others, and general behavior.12) In addition, there are single-item measures of family cohesion, global health, global behavior, and change in health perceptions. The Child Form-87 (CF-87) of the CHQ, designed for those aged 10 to 17 years, consists of 87 questions and contains the same scales as the PF-50, except that it omits the parental impact scales. For patients ≥ 18 years old, we administered the Short Form-36 (SF-36), which contains eight scales: physical functioning, role-physical, bodily pain, general health, vitality, social functioning, role-emotional, and mental health. For all three measures, the reporting frame for all items is the past month, except for the change in health item and general health perceptions, for which the reporting frame is the past year. Individual scale scores range from 0 to 100, with higher scores representing better HRQOL. For the PF-50 and the SF-36, physical and psychosocial summary scores have been created using factor analytic techniques.1213 All three measures have been found to be valid and reliable.12

Clinical and Demographic Measures

Height and weight were obtained by clinic personnel using a stadiometer and digital scale, and were recorded on clinic intake sheets. Height and weight z scores and BMI were calculated using Epi-Info 2000 from the Centers for Disease Control and Prevention.15The BMI percentage of median expected for age was derived from the 1998 standards proposed by Rosner and colleagues.16As has been commonly done in CF research, for those aged ≥ 18 years, BMI percentage was calculated using standards for 18 year olds. Pulmonary function was determined using spirometry, and total body plethysmography according to American Thoracic Society Standards.17Pulmonary function test data were excluded if they did not meet these standards (n = 30); these were from mostly young children. Demographic data and other clinical indicators (such as pulmonary exacerbations) were obtained from medical record review and hospital databases. Pulmonary exacerbations were defined using the criteria suggested by Fuchs et al.18 Patients with four or more of the following signs or symptoms were considered to have an exacerbation and were treated with antibiotics: (1) increased cough; (2) increased dyspnea (without reactive airways component); (3) increased sputum production/change in sputum appearance; (4) new/increased hemoptysis; (5) temperature> 38°F; (6) loss of appetite or weight loss (unexplained by stool pattern); (7) malaise/fatigue/lethargy; (8) new finding on chest examination; (9) new infiltrate on chest radiograph; (10) decline in pulmonary function FVC or FEV1 percent predicted> 10% from previous measurement; (11) sinus pain/tenderness. No patients at this center received routine tri-monthly antibiotics.

Exercise tolerance was determined by timed walking distance. In children with CF and other lung diseases, walking distance has correlated well with more complicated measures of functional capacity, such as peak oxygen uptake and physical work capacity.1920 Baseline oxygen saturation and vital signs were obtained. A portable pulse oximeter was attached to the patient and carried by study personnel. The patient was instructed to walk as quickly as comfortably possible on a 41-m long, level hospital corridor for 6 min. Each patient completed the protocol one time. Supplemental oxygen was administered as needed to keep the patient’s oxygen saturation ≥ 88%. Owing to infection control concerns, patients known to have Burkholderia cepacia or methicillin-resistant Staphylococcus aureus colonization did not complete the walk test. A few additional patients declined the walk test, leaving a total of 121 patients with walk test data.

Data Collection Procedures

The study was approved by the institutional review boards at the children’s and university hospitals. Families were informed of the study by mail. At the time of registration for a routine quarterly visit, families were invited formally to participate. Informed assent or consent was obtained from the patient, and from the parent or guardian for those patients < 18 years of age. A parent completed the PF-50 for children < 18 years old, and children 10 to 17 years old completed the CF-87. Those ≥ 18 years old completed the SF-36. The SF-36 could be completed in 5 to 7 min, and the CHQ could be completed in 15 to 20 min. HRQOL measures were completed prior to obtaining any clinical information (such as weight) from the patient. Pulmonary function testing was done as part of routine clinical care. The 6-min walk test was completed during the visit. For patients who missed more than two quarterly visits, the study team attempted to reschedule a visit. If a visit could not be scheduled, the HRQOL measure was administered by telephone using standardized interview protocols developed by the CHQ and SF-36 developers.1213

Data Management

Data were double entered into Microsoft Access (Microsoft; Redmond, WA). Standardized software provided by the CHQ and SF-36 developers were used to compute scale and summary scores. For missing scale items, the developers recommend that scale scores be calculated if more than half of the items within the scale have been answered, substituting the mean score for that scale for the missing value. If fewer than half of the items in a scale were completed, then the scale was coded as missing.

Power Calculation

Although there is no “gold standard,” a ≥ 5-point difference in scale scores is a conservative estimate of clinically and socially important differences. For example, a difference of 5.5 points on the physical function scale of the CHQ PF-50 discriminated children with asthma in the 75th percentile of function from those in the 50th percentile.12 Power was calculated in two ways: (1) comparing the study population with a national population norm, and (2) comparing patients with FEV1 percent predicted of> 70% predicted or < 70% predicted. SDs for CHQ scores were obtained from children with asthma.,12) FEV1 percent predicted means and SDs were obtained from the clinical database for the prospective study population. With a projected sample of 165, we had 80% power to detect 2-point differences in the physical or psychosocial summary scores between the study population and published norms. For the individual scale scores, we had 80% power to detect a 3- to 4- point difference between the study population and published norms. For the high vs low FEV1 percent predicted groups, we had 80% power to detect a difference of 3 to 4 points on the summary scores and 5 points on an individual scale score.

Analysis

Pairwise comparisons between the means of each scale and summary score for the SF-36 and PF-50 and the published norms for the general US population12) were conducted using two-tailed t tests. Published normative data are summary in nature. Hence, the study patients could not be individually matched but only compared at the group level. Detailed sociodemographic data for the normative samples were also not available, so comparison could not be made on these variables. Since the mean age of our SF-36 completers was 27 years, we compared their scores with both the 18- to 24-year-old and 25- to 34-year-old norms. The results were the same, so only the comparisons with the 18- to 24-year-old norm are presented. National norms and summary scores are not yet available for the CF-87. The physical and psychosocial summary scores for the SF-36 and PF-50 are scaled to have a mean of 50 and a SD of 10 in the general population. In order to determine whether the SF-36 and PF-50 scores could be combined in subsequent analyses, we first tabulated them separately and tested the homogeneity of the SF-36 summary scales vs the PF-50 summary scales by stratifying on other suspected predictors of difference in scores, such as FEV1 percent predicted and number of exacerbations. The means and the variances of the summary scores were not significantly different after stratifying by these factors. They were therefore combined in subsequent analyses.

In preliminary analyses, bivariate associations between physical and psychosocial summary scores and the independent variables were determined using t tests for continuous variables andχ 2 tests for categorical variables. Multiple linear regression was then used to identify variables independently associated with summary scores. The two a priori predictors of differences in scores were FEV1 percent predicted and 6-min walk distance. The other independent variables (age, gender, height, and weight z scores, percent predicted BMI, number of pulmonary exacerbations, presence of P aeruginosa in the sputum, presence of diabetes mellitus, and insurance status) were added to the model and retained if significant or if their inclusion caused a significant change in the coefficients of the model (confounding). These variables were also examined for significant interactions with the measures of disease severity (effect modification). The number of pulmonary exacerbations was not initially included as a key predictor, but preliminary analyses demonstrated a significant association between HRQOL and exacerbations. Thus additional post hoc analyses were conducted to determine if there were any significant interaction terms involving this variable. Those with no exacerbations in the preceding 6 months were coded as having 183 days (6 months) since last exacerbation.

Table 1 summarizes the demographic and clinical characteristics of the participants based on the questionnaire they completed (SF-36, PF-50, or CF-87). Sixty-three children aged 10 to 17 years completed the CF-87. One hundred fourteen parents (84% mothers) of children aged 5 to 17 years completed the PF-50. Tables 234 present the mean, SD, range, quartiles, and percent at the floor and ceiling for each scale and for the summary scores. The highest mean scores (93.5) were for the role function-behavioral and role function-physical scales of the CF-87. The lowest mean scores on all three questionnaires were for general health perceptions, which ranged from 47.5 on the SF-36 to 60.9 on the CF-87. Overall, there were very few scales for which the respondents reported the worst possible function (floor effect). Only the role-physical and role-emotional scales of the SF-36 had > 3% of respondents reporting the worst possible score. However, all three questionnaires demonstrated problems with ceiling effects, particularly for the role functioning scales. From 54.2 to 79.7% of respondents reported functioning at the top of these scales. In addition, over half of the PF-50 respondents reported the highest possible score for physical function.

Figures 1 , 2 compare the summary and individual scale scores of the study participants with normative values for the general US population. On the PF-50, participants were similar with respect to the psychosocial summary score, role function-emotional/behavior, mental health, behavior, and parental impact-time scales. The self-esteem scale score was higher than that of the general population, while scores were lower for the physical summary score, physical function, role function-physical, general health perceptions, bodily pain, and parental impact-emotional. On the SF-36, participants’ scores were similar to the general young adult population on the mental health summary score, and the bodily pain, vitality, social function, role function-emotional, and mental health scales. Participants reported lower scores for the physical summary score and the physical function, role function-physical, and general health perceptions scales.

Table 5 shows the bivariate relationships between the independent variables and the physical and psychosocial summary scores. In both the bivariate and multivariate analyses, age, FEV1 percent predicted, percent expected BMI, and days since last exacerbation were analyzed both as continuous and categorical variables. The results of the statistical testing were similar; they are presented as categorical variables for clarity. In the bivariable analyses, the number of exacerbations in the past 6 months and the days since last exacerbation were both strongly associated with the physical and psychosocial summary score. Although there were trends in the expected directions (scores fell with decreasing FEV1 percent predicted), no other factors were significantly associated with the summary scores.

In the multivariate modeling, the only significant predictor of both the physical and psychosocial summary scores was the number of exacerbations. Those with no exacerbations had a mean physical summary score of 49.8, similar to the general population mean. For those with one exacerbation, the mean was 42.1. With two exacerbations, the mean fell to 39.6; with three or more exacerbations, the mean dropped to 32.0, almost 2 SDs below the general population. Although significant on its own, the number of days since last exacerbation was not significant when number of exacerbations was also in the model. The two were highly correlated with a coefficient of − 0.72. Because the FEV1 percent predicted was a hypothesized predictor and because there was a trend in the bivariate analysis, we forced FEV1 percent predicted into the models. It remained nonsignificant in both the physical (p = 0.499) and psychosocial models (p = 0.497). No other term entered any model with the exception of P aeruginosa colonization (p = 0.03) in the psychosocial summary model that had only exacerbations. However, in the model with FEV1 percent predicted added, P aeruginosa colonization was no longer significant. All two-way interactions with exacerbations and with P aeruginosa were examined, and none was significant.

Due to the unexpected lack of significant association between the summary scores and FEV1 percent predicted, walk distance, or the nutritional indexes, we performed further analyses to examine the associations between these variables and the individual scale scores. We thought that the overall nature of the summary scores may obscure real differences between groups that could be apparent comparing the more specific scale scores. There were a number of significant bivariate associations, especially for the global health scale. When multivariable modeling was conducted, however, the only significant independent predictors were those related to exacerbations (data not shown).

Pulmonary exacerbations of CF had a profound negative impact on physical and psychosocial HRQOL. Other traditional severity measures such as FEV1 percent predicted and nutritional indexes were not associated with significant differences in HRQOL, although most trends were in the expected direction. On average, those with FEV1 percent predicted ≤ 70 had physical scores 3 points lower than those with higher FEV1 percent predicted. We had 80% statistical power to detect a 3- to 4-point difference between groups, so it is possible that we failed to detect a significant difference by chance. Nonetheless, the modest magnitude of the difference (3 points) suggests that the impact of FEV1 percent predicted on HRQOL is relatively small, especially compared to the impact of exacerbations. Our findings are similar to some other studies of patients with CF3 and other pulmonary disease21 that demonstrated only moderate correlations between physiologic parameters and HRQOL. The powerful association of HRQOL with exacerbations, and the weaker association with FEV1 percent predicted may imply that for patients and families, HRQOL has less to do with how severe one’s underlying disease is, and more to do with the disruptive effect of exacerbations. In this analysis, the available data on exacerbations was limited to the number and timing of protocol-defined exacerbations. Thus, we are unable to fully explore how exacerbations cause their detrimental effect on HRQOL. For example, do different treatment strategies have the same impact? Prospective longitudinal information regarding the association between FEV1 percent predicted, HRQOL, and exacerbations will help to clarify these issues.

The lack of association between HRQOL and 6-min walk distance was surprising. We had expected this measure, which is related to functional exercise capacity,1920 to be reflected in the physical HRQOL scores. One possible explanation for the lack of association may be the relatively long distances walked by our patients: 507 m or 84 m/min on average. In the study of severely ill patients by Nixon et al,19 the mean distance was only 407 m. Some of our most physically limited patients declined to undertake the walk test. It may have been that in order to reach submaximal exercise capacity, our participating patients would have needed to run rather than walk quickly. This explanation seems less likely, though, because the HRQOL scores were similar for even the lowest tertile of walkers whose maximum distance was only 440 m. Thus, it may be that exercise tolerance is not closely related to HRQOL in this relatively healthy population. Other studies of patients with CF have not assessed the relationship of HRQOL to exercise tolerance.

The CHQ and SF-36 overall demonstrated wide variability in this population except for the role function scales, where most participants scored at the highest possible level. Ceiling problems with the role function scales have been a common problem with generic HRQOL measures. Studies for which role function is of particular interest should use more detailed role function measures that explore a broader range of function.

In the psychosocial domains, the participants’ scores were similar to the overall population. Our findings are in agreement with other studies2223 demonstrating the reasonably good psychosocial function of patients with CF. One important caveat here is that our participants were healthier than our nonparticipants. They were also more likely to have commercial, rather than public, health insurance, which likely reflects higher socioeconomic status. Thus, we cannot conclude from this work that CF patients, in general, experience psychosocial HRQOL similar to the overall population.

As expected, the majority of the physical functioning scores were worse than the general population, although the magnitude of the difference was modest. Differences of the largest magnitude were seen in the general health perceptions scale. Items in that scale require respondents to compare explicitly their state of health to that of their peers. The relatively higher scores on the other scales likely reflect CF patients’ and families’ long-term acclimatization to living with CF.

The issue of systematic differences between those who participate and those who decline is a significant one in behavioral and HRQOL studies in CF and other pediatric chronic illnesses. Relatively small population sizes preclude random sample techniques used in general population studies. Thus, most studies of psychosocial adaptation or HRQOL in patients with CF have relied on convenience samples. It is likely that these samples overestimate the true health of the population. With an extended 27-month recruitment period and outreach efforts, we were able to recruit 83% of the population of our center. Nonetheless, those who declined were sicker and may well have had a substantially lower HRQOL than participants. Populations in other geographic areas or at other centers may have different characteristics and subsequently different HRQOL than our population.

Recent pulmonary exacerbations are the most important factor determining physical and psychosocial HRQOL in the patients with CF who we studied. Other traditional severity measures had less impact, at least in this cross-sectional analysis. Overall, the CHQ and the SF-36 appear to be well suited for use in the CF population, except for the role-function scales, where ceiling effects limit their usefulness. Generic HRQOL measures, such as these, will complement the CF-specific measures now being developed. They will be especially crucial for studies investigating health utilities or cost-effectiveness of treatments. Finally, the key role of exacerbations in determining HRQOL needs to be further understood. Longitudinal follow-up of this cohort and more detailed data regarding pulmonary exacerbations will allow us to determine how exacerbations and other changes in disease severity impact HRQOL over time.

Abbreviations: BMI = body mass index; CF = cystic fibrosis; CF-87 = Child Form-87; CHQ = Child Health Questionnaire; HRQOL = health-related quality of life; PF-50 = Parent Form-50; SF-36 = Short Form-36

Supported by grant BRITTO98AO from the Cystic Fibrosis Foundation.

Presented in part at the 1999 North American CF Conference, Seattle, WA, October 9, 1999; and Pediatric Academic Societies and American Academy of Pediatrics Joint Meeting, Boston, MA, May 12–15, 2000.

Table Graphic Jump Location
Table 1. Demographic and Clinical Characteristics by HRQOL Measure Completed*
* 

Data are presented as mean (SD) unless otherwise indicated.

Table Graphic Jump Location
Table 2. CHQ PF-50 Scale Scores and Distribution*
* 

Floor % = those reporting the worst possible score; ceiling % = those reporting the best possible score.

 

Physical functioning = physical limitation due to health-related problems; Role function-physical = limitation in activities with friends and at school due to physical health problems; General health = subjective assessment of overall health and illness; Bodily pain = intensity and frequency of general pain and discomfort; Role function-emotional, behavioral = limitations in school work or activities with friends due to emotional or behavioral difficulties; Self-esteem = satisfaction with school and athletic ability, looks/appearance, ability to get along and life overall; Mental health = anxiety, depression and positive affect; Behavior = aggression, delinquency, hyperactivity/impulsivity and social withdrawal; Family cohesion (single item) = how well family gets along with one another; Family activity = frequency of disruption, “usual” family activities; Parental impact-emotional = distress experienced due to child’s physical health, emotional well being, attention or learning abilities, ability to get along and general behavior; Parental impact-time = limitation in parents’ personal time due to same child factors as parental impact-emotional scale.

Table Graphic Jump Location
Table 3. SF-36 Scale Scores and Distributions*
* 

See Table 2 for scale explanations and abbreviations.

Table Graphic Jump Location
Table 4. CHQ CF-87 Scale Scores and Distribution*
* 

See Table 2 for scale explanations and abbreviations.

Figure Jump LinkFigure 1. SF-36. Comparison with the general population.Grahic Jump Location
Figure Jump LinkFigure 2. PF-50. Comparison with the general population.Grahic Jump Location
Table Graphic Jump Location
Table 5. Bivariate Associations Between Clinical Parameters and Summary Scores*
* 

Data are presented as mean (SD).

 

p < 0.01.

We thank the patients and clinical staff of Children’s Hospital Medical Center and University of Cincinnati CF Centers for participating; Debbie Stewart, Angie Duggins, Jennifer Fende, Jennifer Westrich, and Vikki Kociela for assistance with data collection; and Carol Muir for article preparation.

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Figures

Figure Jump LinkFigure 1. SF-36. Comparison with the general population.Grahic Jump Location
Figure Jump LinkFigure 2. PF-50. Comparison with the general population.Grahic Jump Location

Tables

Table Graphic Jump Location
Table 1. Demographic and Clinical Characteristics by HRQOL Measure Completed*
* 

Data are presented as mean (SD) unless otherwise indicated.

Table Graphic Jump Location
Table 2. CHQ PF-50 Scale Scores and Distribution*
* 

Floor % = those reporting the worst possible score; ceiling % = those reporting the best possible score.

 

Physical functioning = physical limitation due to health-related problems; Role function-physical = limitation in activities with friends and at school due to physical health problems; General health = subjective assessment of overall health and illness; Bodily pain = intensity and frequency of general pain and discomfort; Role function-emotional, behavioral = limitations in school work or activities with friends due to emotional or behavioral difficulties; Self-esteem = satisfaction with school and athletic ability, looks/appearance, ability to get along and life overall; Mental health = anxiety, depression and positive affect; Behavior = aggression, delinquency, hyperactivity/impulsivity and social withdrawal; Family cohesion (single item) = how well family gets along with one another; Family activity = frequency of disruption, “usual” family activities; Parental impact-emotional = distress experienced due to child’s physical health, emotional well being, attention or learning abilities, ability to get along and general behavior; Parental impact-time = limitation in parents’ personal time due to same child factors as parental impact-emotional scale.

Table Graphic Jump Location
Table 3. SF-36 Scale Scores and Distributions*
* 

See Table 2 for scale explanations and abbreviations.

Table Graphic Jump Location
Table 4. CHQ CF-87 Scale Scores and Distribution*
* 

See Table 2 for scale explanations and abbreviations.

Table Graphic Jump Location
Table 5. Bivariate Associations Between Clinical Parameters and Summary Scores*
* 

Data are presented as mean (SD).

 

p < 0.01.

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