Flares in patients with systemic lupus erythematosus.

OBJECTIVE: SLE is characterized by relapses and remissions. We aimed to describe the frequency, type and time to flare in a cohort of SLE patients. METHODS: SLE patients with one or more 'A' or 'B' BILAG-2004 systems meeting flare criteria ('new' or 'worse' items) and requiring an increase in immunosuppression were recruited from nine UK centres and assessed at baseline and monthly for 9 months. Subsequent flares were defined as: severe (any 'A' irrespective of number of 'B' flares), moderate (two or more 'B' without any 'A' flares) and mild (one 'B'). RESULTS: Of the 100 patients, 94% were female, 61% White Caucasians, mean age (s.d.) was 40.7 years (12.7) and mean disease duration (s.d.) was 9.3 years (8.1). A total of 195 flares re-occurred in 76 patients over 781 monthly assessments (flare rate of 0.25/patient-month). There were 37 severe flares, 32 moderate flares and 126 mild flares. By 1 month, 22% had a mild/moderate/severe flare and 22% had a severe flare by 7 months. The median time to any 'A' or 'B' flare was 4 months. Severe/moderate flares tended to be in the system(s) affected at baseline, whereas mild flares could affect any system. CONCLUSION: . In a population with active SLE we observed an ongoing rate of flares from early in the follow-up period with moderate-severe flares being due to an inability to fully control the disease. This real-world population study demonstrates the limitations of current treatments and provides a useful reference population from which to inform future clinical trial design.

An educational intervention to prevent overweight in pre-school years: a cluster randomised trial with a focus on disadvantaged families.

BACKGROUND: Early prevention is a promising strategy for reducing obesity in childhood, and Early Years settings are ideal venues for interventions. This work evaluated an educational intervention with the primary aim of preventing overweight and obesity in pre-school children. METHODS: A pragmatic, cluster randomised trial with a parallel, matched-pair design was undertaken. Interventions were targeted at both the cluster (Early Years' Centres, matched by geographical area) and individual participant level (families: mother and 2-year old child). At the cluster level, a staff training intervention used the educational resource Be Active, Eat Healthy. Policies and provision for healthy eating and physical activity were evaluated at baseline and 12-months. The intervention at participant level was the Healthy Heroes Activity Pack: delivered over 6 months by Centre staff to promote healthy eating and physical activity in a fun, interactive way. Child and parent height and weight were measured at four time-points over 2 years. The trial primary outcome was the change in BMI z-score of the child between ages 2 and 4 years. Secondary outcomes consisted of parent-reported measures administered at baseline and two-year follow-up. RESULTS: Five pairs of Early Years' Centres were recruited. Four pairs were analysed as one Centre withdrew (47 intervention families; 34 control families). At the cluster level, improvement in Centre policies and practices was similar for both groups (p = 0.830). At the participant level, the intervention group reduced their mean BMI z-score between age 2 and 4 years (p = 0.002; change difference 0.49; 95% CI 0.17 to 0.80) whereas the control group showed increasing BMI z-score throughout. Changes in parent-reported outcomes and parent BMI (p = 0.582) were similar in both groups. CONCLUSIONS: The Healthy Heroes educational resource deterred excess weight gain in pre-school children from poor socioeconomic areas. With training, Early Years' staff can implement the Healthy Heroes programme. TRIAL REGISTRATION: ISRCTN22620137 Registered 21st December 2016.

Reciprocal Prospective Relationships Between Loneliness and Weight Status in Late Childhood and Early Adolescence.

Adolescents who do not conform to weight ideals are vulnerable to disapproval and victimization from peers in school. But, missing from the literature is a prospective examination of weight status and feelings of loneliness that might come from those experiences. Using data from the Québec Longitudinal Study of Child Development, we filled that gap by examining the prospective associations between loneliness and weight status when the sample was aged 10-13 years. At ages 10, 12, and 13 years, 1042 youth (572 females; 92% from French speaking homes) reported on their loneliness and were weighed and measured. Family income sufficiency was included in our analyses given its relationship with weight status, but also its possible link with loneliness during early adolescence. The findings showed that (1) weight status and loneliness were not associated concurrently; (2) weight status predicted increases in loneliness from ages 12 to 13 years; and (3) loneliness predicted increases in weight from ages 12 to 13 years among female adolescents, but weight loss among male adolescents. The fact that loneliness was involved in weight gain for females suggests that interventions focused on reducing loneliness and increasing connection with peers during early adolescence could help in reducing obesity.

International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety.

Studies measuring psychological distress in individuals with cystic fibrosis (CF) have found high rates of both depression and anxiety. Psychological symptoms in both individuals with CF and parent caregivers have been associated with decreased lung function, lower body mass index, worse adherence, worse health-related quality of life, more frequent hospitalisations and increased healthcare costs. To identify and treat depression and anxiety in CF, the CF Foundation and the European CF Society invited a panel of experts, including physicians, psychologists, psychiatrists, nurses, social workers, a pharmacist, parents and an individual with CF, to develop consensus recommendations for clinical care. Over 18 months, this 22-member committee was divided into four workgroups: Screening; Psychological Interventions; Pharmacological Treatments and Implementation and Future Research, and used the Population, Intervention, Comparison, Outcome methodology to develop questions for literature search and review. Searches were conducted in PubMed, PsychINFO, ScienceDirect, Google Scholar, Psychiatry online and ABDATA by a methodologist at Dartmouth. The committee reviewed 344 articles, drafted statements and set an 80% acceptance for each recommendation statement as a consensus threshold prior to an anonymous voting process. Fifteen guideline recommendation statements for screening and treatment of depression and anxiety in individuals with CF and parent caregivers were finalised by vote. As these recommendations are implemented in CF centres internationally, the process of dissemination, implementation and resource provision should be closely monitored to assess barriers and concerns, validity and use.

Mapping the disease-specific LupusQoL to the SF-6D.

PURPOSE: To derive a mapping algorithm to predict SF-6D utility scores from the non-preference-based LupusQoL and test the performance of the developed algorithm on a separate independent validation data set. METHOD: LupusQoL and SF-6D data were collected from 320 patients with systemic lupus erythematosus (SLE) attending routine rheumatology outpatient appointments at seven centres in the UK. Ordinary least squares (OLS) regression was used to estimate models of increasing complexity in order to predict individuals' SF-6D utility scores from their responses to the LupusQoL questionnaire. Model performance was judged on predictive ability through the size and pattern of prediction errors generated. The performance of the selected model was externally validated on an independent data set containing 113 female SLE patients who had again completed both the LupusQoL and SF-36 questionnaires. RESULTS: Four of the eight LupusQoL domains (physical health, pain, emotional health, and fatigue) were selected as dependent variables in the final model. Overall model fit was good, with R(2) 0.7219, MAE 0.0557, and RMSE 0.0706 when applied to the estimation data set, and R(2) 0.7431, MAE 0.0528, and RMSE 0.0663 when applied to the validation sample. CONCLUSION: This study provides a method by which health state utility values can be estimated from patient responses to the non-preference-based LupusQoL, generalisable beyond the data set upon which it was estimated. Despite concerns over the use of OLS to develop mapping algorithms, we find this method to be suitable in this case due to the normality of the SF-6D data.

Developmental associations between victimization and body mass index from 3 to 10 years in a population sample.

In the current prospective study, we investigated (1) whether high and low BMI in early childhood puts a child at risk of victimization by their peers, and (2) whether being victimized increases BMI over the short- and long-term, independent of the effect of BMI on victimization. We also examined whether gender moderated these prospective associations. Participants were 1,344 children who were assessed yearly from ages 3 to 10 years as part of the Québec Longitudinal Study of Child Development (QLSCD). BMI predicted annual increases in victimization for girls aged 6 years and over; for boys aged 7 and 8 years of age, higher BMI reduced victimization over the school year. Further, victimization predicted annual increases in BMI for girls after age 6 years. When these short-term effects were held constant, victimization was also shown to have a three and 5-year influence on annual BMI changes for girls from age 3 years. These short- and long-term cross-lagged effects were evident when the effects of family adversity were controlled. The findings support those from previous prospective research showing a link between higher BMI and victimization, but only for girls. Further, being victimized increased the likelihood that girls would put on weight over time, which then increased future victimization. The implications of these prospective findings for interventions are considered. Aggr. Behav. 42:109-122, 2015. © 2015 Wiley Periodicals, Inc.

Prevalence of depression and anxiety in patients with cystic fibrosis and parent caregivers: results of The International Depression Epidemiological Study across nine countries.

BACKGROUND: Individuals with chronic diseases and parent caregivers are at increased risk for symptoms of depression and anxiety. Prevalence of psychological symptoms was evaluated in adolescents and adults with cystic fibrosis (CF) and parent caregivers across nine countries. METHODS: Patients with CF, ages 12 years and older, and caregivers of children with CF, birth to18 years of age, completed measures of depression and anxiety across 154 CF centres in Europe and the USA. Psychological symptoms were compared across countries using χ(2). Logistic regression examined extent of comorbid symptoms, predictors of depression and anxiety, and concordance between parent and adolescent symptomatology. RESULTS: Psychological symptoms were reported by 6088 patients with CF and 4102 parents. Elevated symptoms of depression were found in 10% of adolescents, 19% of adults, 37% of mothers and 31% of fathers. Elevations in anxiety were found in 22% of adolescents, 32% of adults, 48% of mothers and 36% of fathers. Overall, elevations were 2-3 times those of community samples. Participants reporting elevated anxiety were more likely to report depression (ORs: adolescents=14.97, adults=13.64, mothers=15.52, fathers=9.20). Significant differences in reports of depression and anxiety were found by patient age and parent respondent. Concordance between 1122 parent-teen dyads indicated that adolescents whose parents reported depression were more likely to be elevated on depression (OR=2.32). Similarly, adolescents whose parents reported anxiety were more likely to score in the elevated range on the anxiety measure (OR=2.22). CONCLUSIONS: Symptoms of depression and anxiety were elevated in both patients with CF and parents across several European countries and the USA. Annual screening of psychological symptoms is recommended for both patients and parents.

Development and validation of the Liverpool infant bronchiolitis severity score: a research protocol.

AIM: To develop and validate a bronchiolitis severity scoring instrument for use by nurses and other healthcare professions. BACKGROUND: Bronchiolitis is a viral lower respiratory tract infection of infancy. In industrialized countries, admission rates have increased over the last decade with up to 3% of all infants born being admitted to hospital. A small number of these hospitalized infants will require admission to critical care for either invasive or non-invasive ventilation. During the seasonal epidemic, the number of unplanned admissions to critical care with bronchiolitis substantially increases. DESIGN: We will use a mixed methods study design. METHODS: We will use scale development and psychometric methods to develop a scoring instrument and to test the instrument for content, construct and criterion validity and reliability in several different clinical locations. This study protocol has been reviewed and approved by the NHS National Research Ethics Service, January 2011. DISCUSSION: There is an urgent need to develop a valid and reliable severity scoring instrument sensitive to clinical changes in the infant, to facilitate clinical decision-making and help standardize patient care. Furthermore, a valid and reliable scoring instrument could also be used as a proxy patient-reported outcome measure to evaluate the efficacy of clinical interventions in randomized controlled trials.

Longitudinal association between lung function and health-related quality of life in cystic fibrosis.

BACKGROUND: Lung function is an important indicator of cystic fibrosis disease status and those with better forced expiratory volume in 1 s (FEV(1))% predicted have tended to report a better health-related quality of life (HRQoL) in cross-sectional studies. The relationship between lung function and HRQoL over time is unknown. This work assesses the natural progression of HRQoL reporting over many years and compares assessments across a whole decade and evaluates the relationship between lung function and HRQoL longitudinally. METHODS: Demographic (age, gender), clinical (FEV(1)% predicted, body mass index, diabetes, Burkholderia cepacia complex, intravenous access device and nutritional status) and HRQoL (Cystic Fibrosis Quality of Life Questionnaire) variables were obtained every 2 years over a 12-year period (seven time points from 1998 to 2010). RESULTS: HRQoL and lung function declined slowly over time and significant decade changes were observed for FEV(1)% predicted and the nine domains of the Cystic Fibrosis Quality of Life Questionnaire. The results of random coefficient modelling indicated that, at the population level, decreasing FEV(1)% predicted was associated with decreasing HRQoL after adjusting for confounding variables. However, the percentage of patients for whom a decrease in lung function was associated with a decrease in HRQoL differed according to the quality of life domain. CONCLUSIONS: HRQoL and FEV(1)% predicted decline slowly; nevertheless, a decrease in lung function predicted a decrease in HRQoL over time.

Comparison of the psychometric properties of health-related quality of life measures used in adults with systemic lupus erythematosus: a review of the literature.

OBJECTIVE: A review of the literature was undertaken to evaluate the development and psychometric properties of health-related quality of life (HRQoL) measures used in adults with SLE. This information will help clinicians make an informed choice about the measures most appropriate for research and clinical practice. METHODS: Using the key words lupus and quality of life, full original papers in English were identified from six databases: OVID MEDLINE, EMBASE, Allied and Complementary Medicine, Psychinfo, Web of Science and Health and Psychosocial Instruments. Only studies describing the validation of HRQoL measures in adult SLE patients were retrieved. RESULTS: Thirteen papers were relevant; five evaluated generic instruments [QOLS-S (n = 1), EQ-5D/SF-6D (n = 1), SF-36 (n = 3)] and eight evaluated disease-specific measures [L-QOL (n = 1), LupusQoL (UK) (n = 1), LupusQoL (US) (n = 1), SSC (n = 2), SLEQOL (n = 3)]. For the generic measures, there is moderate evidence of good content validity and internal consistency, whereas there is strong evidence for both these psychometric properties in disease-specific measures. There is limited to moderate evidence to support the construct validity and test-retest reliability for the disease-specific measures. Responsiveness and floor/ceiling effects have not been adequately investigated in any of the measures. CONCLUSIONS: Direct comparison of the psychometric properties was difficult because of the different methodologies employed in the development and evaluation of the different HRQoL measures. However, there is supportive evidence that multidimensional disease-specific measures are the most suitable in terms of content and internal reliability for use in studies of adult patients with SLE.

Ways of coping and survival in Cystic Fibrosis: a 20-year longitudinal study.

BACKGROUND: The relationship between ways of coping and health outcomes has been a focus of interest for decades. There is increasing recognition that positive psychological functioning can influence health outcomes beneficially. This work investigated the role of coping in predicting survival in CF. METHODS: A longitudinal observational cohort study with a 20-year follow-up period was undertaken. At entry to the study, demographic and clinical variables were recorded, and ways of coping were assessed using the Cystic Fibrosis Coping Scale which measures four distinct ways of coping: optimism, hopefulness, distraction and avoidance. Survival outcome was measured as time in days from the date of recruitment to exit from the study, where exit was either death, loss to follow-up or the end of the follow-up period. RESULTS: Survival time was modelled using Cox's proportional hazards model. At baseline, 116 people with CF were recruited. By the census date, 54 people had died (14 men had died during 248,565 person-days of observation and 40 women had died during 358,372 person-days of observation). Optimism was the only way of coping that showed any beneficial effect on survival (RR=0.984, p=0.040) after adjustments for age, gender, ppFEV1 and the three other coping variables measured at baseline. CONCLUSION: This work suggests that optimistic coping serves as a prognostic measure of survival in CF beyond key clinical and demographic variables. Ways of coping are modifiable, providing a target for clinical intervention; to improve quality of life and clinical outcomes and potentially increase longevity.

Negotiating safety and sexual risk reduction with clients in unsanctioned safer indoor sex work environments: a qualitative study.

OBJECTIVES: We examined how unique, low-barrier, supportive housing programs for women who are functioning as unsanctioned indoor sex work environments in a Canadian urban setting influence risk negotiation with clients in sex work transactions. METHODS: We conducted 39 semistructured qualitative interviews and 6 focus groups with women who live in low-barrier, supportive housing for marginalized sex workers with substance use issues. All interviews were transcribed verbatim and thematically analyzed. RESULTS: Women's accounts indicated that unsanctioned indoor sex work environments promoted increased control over negotiating sex work transactions, including the capacity to refuse unwanted services, negotiate condom use, and avoid violent perpetrators. Despite the lack of formal legal and policy support for indoor sex work venues in Canada, the environmental-structural supports afforded by these unsanctioned indoor sex work environments, including surveillance cameras and support from staff or police in removing violent clients, were linked to improved police relationships and facilitated the institution of informal peer-safety mechanisms. CONCLUSIONS: This study has drawn attention to the potential role of safer indoor sex work environments as venues for public health and violence prevention interventions and has indicated the critical importance of removing the sociolegal barriers preventing the formal implementation of such programs.

Mothers' process of decision making for gastrostomy placement.

In this article we present the findings of an exploration of mothers' discourses on decision making for gastrostomy placement for their child. Exploring in-depth interviews of a purposive sample, we analyzed the mothers' discourses of the decision-making process to understand how their experiences of the process influenced their subsequent constructions of decision making. Mothers negotiated decision making by reflecting on their personal experiences of feeding their child, either orally or via a tube, and interwove their background experiences with the communications from members of the health care team until a decision was reached. Decision making was often fraught with difficulty, resulting in anxiety and guilt. Experiences of decision making ranged from perceived coercion to true choice, which encompasses a truly child-centered decision. The resulting impact of the decision-making process on the mothers was profound. We conclude with an exploration of the implications for clinical practice and describe how health care professionals can support mothers to ensure that decision-making processes for gastrostomy placement in children are significantly improved.

Understanding the sleep-aggression relationship in a forensic mental health sample.

The contribution of cognition to the sleep-aggression relationship is explored via three connected studies, involving adult male forensic patients detained in a high secure hospital. Study 1 included 31 patients, interviewed to examine their experiences of specific sleep problems. In Study 2, 42 patients completed a series of measures examining sleep dysfunction, aggression, and cognition, while Study 3 was designed to impact on sleep via a cognitive approach. In the latter, 48 patients were randomly assigned as part of a feasibility trial to one of three conditions: mindfulness (cognitive approach), sleep education, and treatment as usual. Collectively, the studies demonstrated the multifaceted nature of cognition in the sleep-aggression relationship, with a need to account fully for cognitive factors. A preliminary conceptual model is outlined - the Cognitive Sleep Model for Aggression and Self Harm (CoSMASH), as a direction for future research to consider.

Treatment Preference Among People With Cystic Fibrosis: The Importance of Reducing Treatment Burden.

BACKGROUND: There is a growing consensus that the perspective of the patient should be considered in the evaluation of novel interventions. RESEARCH QUESTION: What treatment outcomes matter to people with cystic fibrosis (CF), and what trade-offs would they make to realize these outcomes? STUDY DESIGN AND METHODS: Adults attending a specialist CF center were invited to complete an online discrete choice experiment (DCE). The DCE required participants to evaluate hypothetical CF treatment profiles, defined by impact on lung function, pulmonary exacerbations, abdominal symptoms, life expectancy, quality of life, inhaled medicine usage, and physiotherapy requirement. Choice data were analyzed, using multinomial logit and latent class models. RESULTS: One hundred and three people with CF completed the survey (median age, 35 years; range, 18-76 years); 52% were female; mean FEV1 % predicted, 69% [SD, 22%]). On average, an improvement in life expectancy by 10 years or more had the greatest impact on treatment preference, followed by a 15% increase in lung function. However, it was shown that people would trade substantial reductions in these key outcomes to reduce treatment time or burden. Preference profiles were not uniform across the sample: three distinct subgroups were identified, each placing markedly different importance on the relative importance of both life expectancy and lung function compared with other attributes. INTERPRETATION: The relative importance of treatment burden to people with CF, compared with life expectancy and lung function, suggests it should be routinely captured in clinical trials as an important secondary outcome measure. When considering the patient perspective, it is important that decision-makers recognize that the values of people with CF are not homogeneous.

Cystic fibrosis-related diabetes (CFRD) and cognitive function in adults with cystic fibrosis.

BACKGROUND: Being able to function cognitively is imperative for successful achievement in school, working life, and disease self-management. Diabetes is known to cause changes in brain structure and long-term cognitive dysfunction. This work investigated cystic fibrosis-related diabetes (CFRD) as a mechanism for cognitive impairment in people with CF. It was hypothesised that cognition would be poorer in adults with CFRD than in those with CF without diabetes (CFND) or in healthy controls. METHODS: Cognitive performance was assessed using the Cambridge Neuropsychological Test Automated Battery which provides a comprehensive cognitive assessment with tests mapping onto specific brain regions. Demographic, clinical and self-reported health data were documented for all participants. CF specific clinical variables were recorded for the two CF groups. RESULTS: Ninety-eight people with CF (49CFRD,49CFND) and 49 healthy controls were recruited. People with CF demonstrated deficits in aspects of verbal and spatial memory, processing speed and cognitive flexibility compared with healthy controls, with all areas of the brain implicated. Those with CFRD had additional difficulties with higher-level processes known collectively as 'executive function', which demand greater cognitive load and recruit the prefrontal cortex. Compared with healthy controls, those with CFND and CFRD had an estimated 20% and up to 40% reduction in processing speed respectively. CONCLUSION: Managing CF requires higher order executive function. Impairments may be sufficient to interfere with self-care and the ability to perform everyday tasks efficiently. At which point in the CF disease trajectory these difficulties begin, and what may attenuate them, has yet to be determined.

Exploring the nature of perceived treatment burden: a study to compare treatment burden measures in adults with cystic fibrosis [version 1; peer review: 2 approved].

Background: Despite the importance of reducing treatment burden for people with cystic fibrosis (CF), it has not been fully understood as a concept. This study aims to quantify the treatment burden perceived by CF adults and explore the association between different validated treatment burden measures. Methods: This is a cross-sectional observational study of CF adults attending a single large UK adult center. Participants completed an online survey that contained three different treatment burden scales; CF Questionnaire-Revised (CFQ-R) subscale, CF Quality of Life (CFQoL) subscale, and the generic multimorbidity treatment burden questionnaire (MTBQ). Results: Among 101 participants, the median reported treatment burden by the CFQ-R subscale was 55.5 (IQR 33.3 - 66.6), the CFQoL subscale was 66.6 (IQR 46.6 - 86.6), and the MTBQ reversed global score was 84.6 (IQR 73.1 - 92.3). No correlation was found between respondents' demographic or clinical variables and treatment burden measured via any of the three measures. All treatment burden measures showed correlations against each other. More treatments were associated with high treatment burden as measured by the CFQ-R, CFQoL subscales, and the MTBQ. However, longer treatment time and more complex treatment plans were correlated with high treatment burden as measured by the CFQ-R and CFQoL subscales, but not with the MTBQ. Conclusions: Treatment burden is a substantial issue in CF. Currently, the only available way to evaluate it is with the CF-specific quality of life measure treatment burden subscales (CFQ-R and CFQoL); both indicated that treatment burden increases with more treatments, longer treatment time, and more complex treatments.

Impact of COVID-19 on mental health: Effects on screening, care delivery, and people with cystic fibrosis.

BACKGROUND: Depression and anxiety are two to four times more prevalent in people with CF (pwCF) than the general population. COVID-19 may exacerbate mental health challenges, increasing demand for psychological services, while decreasing their availability. We assessed the impact of the pandemic on depression and anxiety in pwCF, including how COVID-19 affected the frequency of mental health screening and the types of services provided. METHODS: A 38-item internet survey, completed in June 2020, assessed how COVID-19 affected: 1) the mental health clinician's role and screening processes; 2) barriers to screening and resource needs; 3) impact of COVID-19 on depression and anxiety, and 4) positive outcomes and confidence in sustaining mental health screening and treatment, including telehealth services, after the pandemic. RESULTS: Responses were obtained from 131 of the 289 US CF programs. Overall, 60% of programs (n=79) continued mental health screening and treatment, although less frequently; 50% provided individual tele-mental health interventions, and 9% provided telehealth group therapy. Clinically elevated depression symptoms (PHQ-9≥10; moderate to severe), were found in 12% of 785 pwCF, with 3.1% endorsing suicidal ideation. Similarly, elevated anxiety (moderate to severe; GAD-7≥10) was found in 13% of pwCF (n=779). CONCLUSIONS: The COVID-19 pandemic created an opportunity to implement innovative solutions to disruptions in mental health screening and treatment in CF programs. We found that pwCF had increased access to psychological interventions during the pandemic via telehealth, supporting the continued integration of tele-mental health screening and treatment into CF care.

Can health-related quality of life predict survival in adults with cystic fibrosis?

RATIONALE: Advances in the management of cystic fibrosis have led to a significant improvement in survival, although marked differences between individuals are still observed. The value of patient-reported health-related quality of life scores in predicting survival in adults with cystic fibrosis is unknown. OBJECTIVES: To evaluate whether patient-reported health-related quality of life could predict survival in cystic fibrosis. METHODS: From 1996 to 1997 a consecutive series of 223 patients were recruited to evaluate the Cystic Fibrosis Quality of Life Questionnaire. Demographic (age, sex), clinical (FEV(1)% predicted, body mass index, diabetes, B. Cepacia complex, intravenous access device, nutritional and lung transplant status) and health-related quality of life variables were recorded (Cystic Fibrosis Quality of Life Questionnaire and the SF-36). These data were used as baseline measures to explore the prognostic association of health-related quality of life and subsequent survival. MEASUREMENTS AND MAIN RESULTS: At the census date (December 31, 2006) 154 (69.1%) adults were alive, 66 (29.6%) had died, and three (1.3%) were lost to follow-up. Cox proportional hazards models and bootstrapping procedures examined if health-related quality of life domains predicted survival after adjusting for the demographic and clinical factors. The physical functioning domain of the Cystic Fibrosis Quality of Life Questionnaire and the pain domain of the Short Form-36 had the strongest statistical associations with survival. CONCLUSIONS: Aspects of patient-reported quality of life serve as prognostic measures of survival beyond a number of previously known factors in cystic fibrosis. This needs to be investigated further in a larger longitudinal study.