Health related quality of life in cystic fibrosis: To work or not to work?

AbstractBackgroundThe present study investigated whether patients with CF who are studying or working report a better HRQoL in comparison to non-working/studying patients.Methods57 adult CF patients completed the Cystic Fibrosis Questionnaire—Revised, a CF-specific measure of HRQoL. Medical condition was quantified in terms of FEV1 % predicted, BMI, Pseudomonas aer. status, pancreatic status (PS), having an indwelling catheter device (PAC), CF Related Diabetes Mellitus and nutritional status.ResultsMean age was 26.7 years (SD 8.1), mean FEV1 % predicted was 65.09 (SD 22.18), mean BMI was 21.23 (SD 3.45). FEV1 % predicted was related to HRQoL domains Physical Functioning and General Health (r=0.27 and 0.38 respectively, p<0.05). A higher BMI was associated with better scores on Eating Disturbances (= fewer problems; r=0.44, p<0.01) and a better perception of Weight (r=0.43, p<0.01) and Body Image (r=0.28, p<0.05). Analysis of variance showed that specific domains of HRQoL were related to diabetes (Weight), taking caloric supplements (Body Image and Weight) and/or PAC (Physical Functioning, Treatment Burden, Role, Weight). Twenty-four patients worked/studied, these patients had a higher FEV1, and fewer had Pseudomonas aer. or a PAC or took high caloric supplements, compared to non-working/studying patients. After controlling for medical parameters, patients who were working/studying scored higher than non-working/studying patients on Physical Functioning, Role Functioning and Social Functioning.ConclusionsCF patients' HRQoL is related to medical status. The non-working/studying CF patients in this sample had greater disease severity and reported a lower quality of life than their working/studying peers, even after controlling for relevant medical parameters. The decision to stop work/study for CF patients is difficult and affects patients' personal, social and financial well-being

Communication of information about reproductive and sexual health in cystic fibrosis. Patients, parents and caregivers' experience

AbstractBackgroundThis review evaluated research concerning communication and information about reproductive and sexual health (RSH) in cystic fibrosis (CF).MethodsPapers in the English language reporting RSH issues in CF, published between January 2000 and December 2010, were included. The review focused on (a) the content of information given to parents and patients, (b) the timing of information, (c) the sources of information, (d) attitudes and emotional reactions, (e) chronic illness and sexual behavior, (f) methodological and cultural considerations and (g) ethical considerations.ResultsEleven papers were identified originating from Australia, the United Kingdom and Poland. Patients and parents expressed the need for up-to-date, CF-related verbal and written information, provided by the CF team and infertility specialists. Patients were often embarrassed to initiate a discussion. Health care providers expressed the need for training to counsel patients in RSH.ConclusionA pro-active discussion of RSH issues is proposed as a standard part of the care-pathway. A list of recommendations is given to initiate this process

Assessing gastro-intestinal related quality of life in cystic fibrosis: Validation of PedsQL GI in children and their parents

Background: Most patients with cystic fibrosis (CF) suffer from pancreatic insufficiency, leading to fat malabsorption, malnutrition and abdominal discomfort. Until recently, no specific tool was available for assessing gastro-intestinal related quality of life (GI QOL) in patients with CF. As the Horizon2020 project MyCyFAPP aims to improve GI QOL by using a newly designed mobile application, a sensitive and reliable outcome measure was needed. We aimed to study the applicability of the existing child-specific Pediatric Quality of Life Inventory, Gastrointestinal Symptoms Scales and Module (PedsQL GI) in children with CF. Methods: A multicenter, prospective observational study was performed in 6 European centers to validate the PedsQL GI in children with CF during 3 months. Results: In total, 248 children and their parents were included. Within-patient variability of PedsQL GI was low (24.11), and there was reasonable agreement between children and parents (ICC 0.681). Nine of 14 subscales were informative (no ceiling effect). The PedsQL GI and the median scores for 4 subscales were significantly lower in patients compared to healthy controls. Positive associations were found between PedsQL GI and age (OR = 1.044, p = 0.004) and between PedsQL GI and BMI z-score (OR = 1.127, p = 0.036). PedsQL GI correlated with most CFQ-R subscales (r 0.268 to 0.623) and with a Visual Analogue Scale (r = 0.20). Conclusions: PedsQL GI is a valid and applicable instrument to assess GI QOL in children with CF. Future research efforts should examine the responsiveness of the CF PedsQL GI to change in the context of clinical interventions and trials

European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre

A significant increase in life expectancy in successive birth cohorts of people with cystic fibrosis (CF) is a result of more effective treatment for the disease. It is also now widely recognized that outcomes for patients cared for in specialist CF Centres are better than for those who are not. Key to the effectiveness of the specialist CF Centre is the multidisciplinary team (MDT), which should include consultants, clinical nurse specialist, microbiologist, physiotherapist, dietitian, pharmacist, clinical psychologist, social worker, clinical geneticist and allied healthcare professionals, all of whom should be experienced in CF care. Members of the MDT are also expected to keep up to date with developments in CF through continued professional development, attendance at conferences, auditing and involvement in research. Specialists CF Centres should also network with other Centres both nationally and internationally, and feed Centre data to registries in order to further the understanding of the disease. This paper provides a framework for the specialist CF Centre, including the organisation of the Centre and the individual roles of MDT members, as well as highlighting the value of CF organisations and disease registries

Breaking bad news, the diagnosis of cystic fibrosis in childhood

The day parents are told their child has cystic fibrosis (CF) is imprinted in their memory. Parents often show strong emotions (e.g. shock, anxiety); they need to cope with bad news and restructure their lives taking into account CF.publisher: Elsevier articletitle: Breaking bad news, the diagnosis of cystic fibrosis in childhood journaltitle: Journal of Cystic Fibrosis articlelink: http://dx.doi.org/10.1016/j.jcf.2014.12.005 content_type: article copyright: Copyright © 2014 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.status: publishe

Belgian siblings of children with a chronic illness: Is their quality of life different from their peers?

To assess Belgian siblings' self-reported quality of life (QoL) and the impact of illness on four different paediatric illnesses. Healthy siblings (n = 131) of children with type 1 diabetes, cancer, congenital heart disease (CHD) and cystic fibrosis (CF) completed the Child Health Questionnaire and the Sibling Perception Questionnaire. Results were compared to those of a matched group of siblings of healthy children. Siblings reported a good QoL, similar to controls, with the exception that siblings reported better on the QoL domain pain (p < .01). QoL was not related to time since diagnosis but the impact of illness was higher nearer to the time of diagnosis (r = -.39, p < .001). QoL of siblings of children with CHD or cancer was lower than QoL in the CF or type 1 diabetes group whilst impact of illness was highest for the CHD group. QoL of siblings of a child with a chronic illness is similar to the QoL of peers. Studies investigating siblings' QoL or the impact of illness on siblings should include the day-to-day demands of the illness as well as less obvious illness-related issues like 'hidden stress' and 'sense of control'.status: publishe

The impact of the COVID-19 pandemic on the emotional well-being and home treatment of Belgian patients with cystic fibrosis, including transplanted patients and paediatric patients.

BACKGROUND: Little is known about the impact of COVID-19 on patients with cystic fibrosis (CF), despite being considered a high-risk group. This study explored the early impact of COVID-19 on the emotional well-being of patients and self-reported changes in their home therapy since the start of the pandemic. METHODS: Adult patients with CF, lung-transplanted (LTX) CF patients and parents of children with CF completed an online questionnaire, securely linked to their medical files. The questionnaire covered the emotional impact of the pandemic, changes in CF and LTX treatment, changes in health-protecting behaviours and CF-related concerns, and their perception of their COVID-19 status. RESULTS: The response rate was 63% (80 CF, 66 LTX and 73 parents). A wide range of illness severity was included. None of the respondents had contracted COVID-19 and all strictly followed the social distancing rules. There was evident psychological impact, with many reporting increased stress, fear and worry about CF and the future. Changes in treatment were positive, including more physiotherapy for adults and better-quality nebulizing. Changes in routine were reported, such as different treatment timing. Adult patients and parents had cancelled their CF appointments more often since the start of the pandemic. CONCLUSIONS: The initial psychological impact of COVID-19 was evident. The impact on home treatment was reassuringly small. Psychological care is needed for patients suffering prolonged psychological impact, and CF teams need to contextualize the information that patients and parents receive from the media and support them to balance the perceived risk with true risk.status: publishe