Louise Gee

Ways of coping with cystic fibrosis: implications for treatment adherence

Purpose: How individuals cope with aspects of cystic fibrosis (CF) has the potential to influence their self management and the course of their disease. To evaluate how individuals cope with CF, a disease specific coping scale was developed and validated. A second objective of the work was to examine the relationship between coping styles and treatment adherence. Methods: The development of the coping scale constituted a longitudinal design. A cross-sectional questionnaire design was used to examine the coping-adherence relationship. The development and validation of the coping scale comprised three phases: (1) Initially, 60 patients were interviewed to identify CF concerns. From this information a list of 23 concerns were recorded; (2) Eighty-three patients were interviewed to identify CF coping responses. For each concern, they were asked what they did or thought to ease the worry. A list of 24 coping strategies were recorded that formed a comprehensive set of items as to how people with CF act, feel and think about aspects of their disease; and (3) Further development and testing of the questionnaire involved 174 patients completing the measure. Four distinct ways of coping with CF were identified by factor analysis. These were termed optimistic acceptance, hopefulness, distraction and avoidance. The cronbach alpha coefficients were 0.74 (optimistic acceptance), 0.69 (hopefulness), 0.71 (distraction) and 0.76 (avoidance). To evaluate the relationship between coping and treatment adherence 60 patients completed the CF Coping Questionnaire and the Manchester Adult Cystic Fibrosis Compliance Questionnaire. Results: Compared with patients who were non-adherent, those who were adherent scored higher on the optimistic acceptance scale (physiotherapy p < 0.05, enzymes p < 0.003, vitamins p < 0.05) and hopefulness scale (physiotherapy p < 0.002, enzymes p < 0.001). Those who were partially adherent reported using distraction as a way of coping to a greater extent than adherent or non-adherent patients (all ps 0.05). Nonadherent patients used avoidance strategies to a greater extent than those who were adherent (physiotherapy p < 0.05, enzymes p < 0.04), although interestingly, adherence with exercise was associated with avoidant coping (p < 0.004). Conclusions: The degree of adherence to treatments was influenced by a persons style of coping. The identification of effective coping strategies to aid both long-term psychological and clinical well-being should improve the management of nonadherence.PURPOSE How individuals cope with aspects of cystic fibrosis (CF) has the potential to influence their self management and the course of their disease. To evaluate how individuals cope with CF, a disease specific coping scale was developed and validated. A second objective of the work was to examine the relationship between coping styles and treatment adherence. METHODS The development of the coping scale constituted a longitudinal design. A cross-sectional questionnaire design was used to examine the coping-adherence relationship. The development and validation of the coping scale comprised three phases: (1) Initially, 60 patients were interviewed to identify CF concerns. From this information a list of 23 concerns were recorded; (2) Eighty-three patients were interviewed to identify CF coping responses. For each concern, they were asked what they did or thought to ease the worry. A list of 24 coping strategies were recorded that formed a comprehensive set of items as to how people with CF act, feel and think about aspects of their disease; and (3) Further development and testing of the questionnaire involved 174 patients completing the measure. Four distinct ways of coping with CF were identified by factor analysis. These were termed optimistic acceptance, hopefulness, distraction and avoidance. The cronbach alpha coefficients were 0.74 (optimistic acceptance), 0.69 (hopefulness). 0.71 (distraction) and 0.76 (avoidance). To evaluate the relationship between coping and treatment adherence 60 patients completed the CF Coping Questionnaire and the Manchester Adult Cystic Fibrosis Compliance Questionnaire. RESULTS Compared with patients who were non-adherent, those who were adherent scored higher on the optimistic acceptance scale (physiotherapy p < 0.05, enzymes p < 0.003, vitamins p < 0.05) and hopefulness scale (physiotherapy p < 0.002, enzymes p < 0.001). Those who were partially adherent reported using distraction as a way of coping to a greater extent than adherent or non-adherent patients (all ps < 0.05). Non-adherent patients used avoidance strategies to a greater extent than those who were adherent (physiotherapy p < 0.05, enzymes p < 0.04), although interestingly, adherence with exercise was associated with avoidant coping (p < 0.004). CONCLUSIONS The degree of adherence to treatments was influenced by a persons style of coping. The identification of effective coping strategies to aid both long-term psychological and clinical well-being should improve the management of non-adherence.

Contemporary psychosocial issues in cystic fibrosis: treatment adherence and quality of life

This review focuses on the two major psychosocial areas currently topical in cystic fibrosis research: treatment adherence and quality of life. First, the paper discusses the importance of evaluating adherence to treatments and medical advice, its measurement, predictors of adherence and non-adherence and whether the data should be used to inform clinical practice and policy decisions. Second, the purpose of evaluating quality of life in cystic fibrosis and an overview of the data are discussed. Both areas have suffered from problems with terminology and definition, inappropriate methodologies and hence difficulties with data interpretation. Future directions for research are advocated.

Health-related quality of life in adults with cystic fibrosis: The role of coping

OBJECTIVE This study examined the role of coping in explaining health-related quality of life (HRQoL) in cystic fibrosis (CF). METHODS Coping, quality of life, and clinical variables were assessed. The CF Coping Scale measures four ways of coping: optimistic acceptance, hopefulness, distraction, and avoidance. The Cystic Fibrosis Quality of Life Questionnaire (CFQoL) comprises 9 domains: physical, social, treatment, chest symptoms, emotional, body image, relationships, career, and future. Step-wise multiple regression and path analysis were used to model the associations between coping and CFQoL domains, taking into account important demographic and clinical factors. RESULTS One hundred and sixteen people with CF were recruited to the study. Their mean FEV(1)% predicted and BMI were 59.3% and 21.2 kg/m(2), respectively. Mean scores were highest for optimism and lowest for distraction coping. Coping had an inequitable influence across the CFQoL domains: it had negligible influence on domains that incorporate symptoms and aspects of physical functioning but considerable influence on psychosocial domains. Optimism and distraction were strongly associated with emotional responses, social functioning, and interpersonal relationships. A high level of optimism was associated with a better HRQoL and high levels of distraction with a poorer HRQoL. For some domains, optimism and distraction had a counterbalancing effect so the difference between them could be an important predictor of HRQoL. CONCLUSION Coping has emerged as an important factor in explaining some quality-of-life domains but not others. This has important implications especially when employing HRQoL as an outcome measure in clinical trials.

Validation of the SF-36 for the assessment of quality of life in adolescents and adults with cystic fibrosis

BACKGROUND Generic health-related quality of life measures are often applied to disease groups without assessment of their psychometric properties. The current work assesses the properties of the Short Form 36-item (SF-36) questionnaire in a British sample of adolescents and adults with cystic fibrosis (CF). METHODS Two hundred and twenty-three adolescents and adults with CF completed the SF-36 with a further 185 approached and not responding by non-completion of the questionnaire. The structure and internal reliability of the instrument was assessed by principal components analysis, Cronbach alpha coefficients and item to domain correlations. Differences between disease severity groups were assessed by analysis of variance. RESULTS Factor analysis of the SF-36 scores broadly confirmed domain structures for the SF-36. Cronbach alpha coefficients were high (range 0.82-0.91) and item-to-same domain correlations were stronger than item-to-unrelated domain correlations. Examination of differences between mild, moderate and severe disease states revealed four significant main effects for: physical functioning, role limitation due to physical functioning, general health perceptions and energy and vitality. The analysis also revealed the presence of numerous ceiling effects across domains. CONCLUSIONS The domain structure of the SF-36 was demonstrated to be robust. However, the discriminatory ability of the measure was disappointing. The presence of ceiling effects and the low frequency of differences between intermediate disease severity groups indicated that the SF-36 was not discriminatory with respect to mild disease states or progression of illness.

Perceived Body Image and Eating Behavior in Young Adults with Cystic Fibrosis and Their Healthy Peers

Treatment aimed at achieving an ideal nutritional status is an integral part of the management of patients with cystic fibrosis (CF). Emphasis is continually placed upon dietary intake and weight. The effects of this on eating behavior and self-perceptions are unclear. This work compared male and female CF adults with a healthy male and female control population with regard to (a) clinical variables, (b) actual, perceived, and desired body shape/body mass index (BMI), and (c) body satisfaction, eating behaviors and attitudes, and self-esteem. Clinical data were recorded for 221 adults with CF and 148 healthy controls. All subjects completed BMI Charts (perception of body weight/BMI), the Eating Attitudes Test, and scales of body satisfaction and self-esteem. CF patients had poorer lung function and nutritional status than controls. Control males accurately perceived their body shape/BMI and were content with it, whereas CF males viewed their BMI as greater than it actually was and desired to be much heavier. Control females viewed their body shape/BMI as less than it actually was and desired to be even slimmer, in comparison with CF females, who perceived their BMI as less than it actually was but were happy with their perceived shape/weight. Control subjects, especially females, dieted to a greater extent and were more preoccupied with food (with binge eating and intended vomiting) than CF patients. Conversely, those with CF reported greater pressure from others to eat than did controls. More problems with food/eating behavior were associated with less body satisfaction and reduced self-esteem. In comparison with a healthy control population, the perceptions and behaviors of CF adults relating to eating, weight, and body image are not abnormal. Indeed, females with CF have fewer problems than their healthy peers.

Quality of Life in Children and Adolescents with Cystic Fibrosis

Health related quality of life (QOL) as an outcome measure in clinical trials is becoming increasingly important. Trials should not only be able to demonstrate the pharmacologic activity of a therapy, but of equal importance, they should demonstrate clinical effectiveness that is of significant benefit to the patient. QOL measurement provides a way of incorporating the child/parent’s perspective of how cystic fibrosis (CF) and its therapies impact on their lives. Several validated generic instruments have been employed to measure QOL in adolescents and adults. QOL assessment is more difficult in children and, therefore, has been employed less often in children with CF.Difficulties arise with the issue of whether children can report their own experiences directly, or whether a parent or clinician should report on behalf of the child. A child-centered approach is imperative since the literature indicates that children are able to report on their own QOL. An additional complication has been the use of adult measures with children. These are often inappropriate in their complexity, use of language, response scales, and time frame.The evaluation of pharmacologic therapies can profit from QOL measurement. The effectiveness of a drug and any adverse effects that impact on daily life can be assessed from the child/parent’s viewpoint. Home therapy versus hospital therapy and drug delivery systems, are additional areas where QOL as an outcome measure is valuable. There have been relatively few appropriately powered trials in CF, and only a minority of these have evaluated QOL as an outcome measure.This review highlights areas where QOL measurement is appropriate. It focuses on the pharmacologic trials that have employed QOL assessment for antibiotic, mucociliary clearance, anti-inflammatory, and nutritional therapies.Methodological issues of incorporating QOL assessment into trials center on cross-cultural and data interpretation issues. QOL measurement in CF has been patchy and largely unreliable. The notion that improved symptoms equate with improved QOL is erroneous. Measurement of how symptoms impact on QOL is essential. Currently, the development and validation of CF specific measures (across the CF age range) provides optimism for appropriate QOL measurement in clinical trials, and for future meta-analysis and systematic reviews.

Cross cultural differences in health related quality of life in adolescents with cystic fibrosis

Purpose : Quality of Life (QoL) is an important outcome measure in health care and pharmacological trials. The trend towards multinational clinical trials may be problematic because it is unclear as to whether, or to what extent, QoL measures are comparable across cultures. This study compared QoL between English and German adolescents with Cystic Fibrosis (CF) and their healthy peers. Method : The study comprised a cross-sectional design. The English subjects formed two groups; 58 adolescents with CF and 49 healthy controls. The German subjects consisted of 26 adolescents with CF and 75 healthy controls. Quality of life was measured using the English and German versions of the SF-36. Demographic (age and gender) and clinical data (FEV 1 % predicted and BMI) were also recorded. Results : The English and German CF groups had similar age-adjusted lung function and body mass index. Both the English CF and control groups reported a poorer quality of life than their respective German counterparts across several domains of the SF-36. These were limitations in activities due to physical health problems and emotional difficulties, social functioning, energy and vitality and pain. Conclusions : The differences in quality of life between English and German adolescents with CF appear to be either culturally determined or due to idiosyncrasies in the translations of the SF-36, rather than a consequence of their disease or its management.PURPOSE Quality of Life (QoL) is an important outcome measure in health care and pharmacological trials. The trend towards multinational clinical trials may be problematic because it is unclear as to whether, or to what extent, QoL-measures are comparable across cultures. This study compared QoL between English and German adolescents with Cystic Fibrosis (CF) and their healthy peers. METHOD The study comprised a cross-sectional design. The English subjects formed two groups; 58 adolescents with CF and 49 healthy controls. The German subjects consisted of 26 adolescents with CF and 75 healthy controls. Quality of life was measured using the English and German versions of the SF-36. Demographic (age and gender) and clinical data (FEV1% predicted and BMI) were also recorded. RESULTS The English and German CF groups had similar age-adjusted lung function and body mass index. Both the English CF and control groups reported a poorer quality of life than their respective German counterparts across several domains of the SF-36. These were limitations in activities due to physical health problems and emotional difficulties, social functioning, energy and vitality and pain. CONCLUSIONS The differences in quality of life between English and German adolescents with CF appear to be either culturally determined or due to idiosyncrasies in the translations of the SF-36. rather than a consequence of their disease or its management.

Quality of life in adults with cystic fibrosis: the impact of gender and disease severity

Background: Disease progression in cystic fibrosis (CF) is marked by deterioration across a number of physiological systems. In addition, there is evidence that females have a worse prognosis than males. The current work assesses the impact of both these factors on health related quality of life (HRQoL). Methods: Two hundred and twenty-three adolescents and adults completed the cystic fibrosis quality of life (CFQoL) questionnaire with a further 185 approached and not responding by non-completion of the questionnaire. The CFQoL is divided into nine domains: physical, social, treatment, chest symptoms, emotional functioning, concerns for the future, relationships, body image, and career. Measurement of objective clinical status included, body mass index (BMI), and percentage of predicted forced expiratory volume in one second (FEV1). General health perceptions (GHP) were also measured. Results: Patients were sub-divided by gender and disease severity (mild >70% FEV1, moderate 40–69% and severe <40%). Factorial analysis of variance indicated significant main effects for FEV1 (F=587.98, P≤0.001) and BMI (F=17.29, P≤0.001) as a function of disease severity. Post hoc tests revealed significant two-group differences for FEV1 and BMI between disease severity groups. No differences were observed for gender across FEV1 or BMI. Differences emerged across most CFQoL domains for disease severity, with the exception of concerns for the future, which was consistently low throughout. Gender differences emerged for chest symptoms, emotional functioning, concerns for the future, body image and career. With the exception of body image, females exhibited poorer HRQoL. Pearson correlations indicated that females’ perception of health was more closely related to clinical status than males. Conclusions: Disease severity has an impact on HRQoL in adolescents and adults with CF. Some differences emerged between males and females, with females generally reporting poorer HRQoL. Evidence indicated that males and females perceived their health status differently, with females having a more accurate perception of objective clinical health status.