Psychological and family functioning and quality of life in adolescents with cystic fibrosis

Szyndler, Janina; Towns, Susan; Asperen, Peter P Van; McKay, Karen

doi:10.1016/j.jcf.2005.02.004

Cited by 74 publications

(75 citation statements)

References 32 publications

(27 reference statements)

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“…This analysis included FEV 1 and FEV 1 /FVC data collected closest to the dates of completed HRQOL measures. We used data representing patients' baseline pulmonary has been measured in short-term CF-related intervention studies [5][6][7][8][9] and cross-sectional psychosocial research, [10][11][12][13] the relationship between NBS and HRQOL in children and adolescents has not been thoroughly evaluated. Thus far, the RCT has consistently revealed better nutritional status among presymptomatic patients with CF diagnosed through NBS (early diagnosis group) compared with those with CF diagnosed traditionally by symptom identifi cation (control group).…”

Section: Methodsmentioning

confidence: 99%

Relationships Among Health-Related Quality of Life, Pulmonary Health, and Newborn Screening for Cystic Fibrosis

Tluczek

Becker

Laxova

et al. 2011

Chest

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Medical advances have signifi cantly improved the longevity of patients with cystic fi brosis (CF). The median survival age is approximately 37 years, and many patients live well into middle age. 1 Newborn screening (NBS) is an innovation that provides early intervention for affected infants. Although neo natal diagnosis offers nutritional advantages, over time, patients experience complications that require increasingly complex treatments 2-4 for which they and their families typically shoulder most of the burden. The progressive nature of CF and treatment side effects can adversely affect every aspect of life. Thus, there has been a growing interest in understanding how this chronic illness and related life-long care affect health-related quality of life (HRQOL) for persons with CF.This study examined relationships between pulmonary health and HRQOL in patients with CF who participated in the Wisconsin CF Neonatal Screening randomized clinical trial (RCT). 2 Although HRQOL Background: The objective of this study was to examine relationships between pulmonary health and health-related quality of life (HRQOL) in patients with cystic fi brosis (CF) evaluated longitudinally in the Wisconsin Newborn Screening Project. Methods: Patients aged 8 to 18 years (mean Ϯ SD, 13.5 Ϯ 2.8) in early diagnosis (n 5 45) and control (n 5 50) groups completed Cystic Fibrosis Questionnaires (CFQs) to measure HRQOL at three data points over a 2-year period. Pulmonary health was evaluated concurrently by the Wisconsin chest x-ray scoring system (WCXR) and pulmonary function tests (PFTs). Results: WCXR showed signifi cant group differences ( P Յ .023), with the early diagnosis group showing more-severe lung disease. When adjusted for group differences in mucoid Pseudomonas aeruginosa status and pancreatic status, however, WCXR differences and PFT data were not signifi cant. Most patients (74%) had FEV 1 values Ն 80% predicted (within normal range). For patients aged , 14 years, as WCXR scores worsened CFQ respiratory and physical domain scores decreased (both P Յ .007). FEV 1 /FVC showed a positive relationship with the respiratory and physical domains (both P Յ .006). WCXR scores for patients aged Ն 14 years were associated with CFQ weight, respiratory, and health domains (all P Յ .011). FEV 1 was associated with CFQ weight, respiratory, health, and physical domains (all P Յ .003). Changes in pulmonary health were not associated with changes in CFQ over time. Signifi cant group differences on the CFQ-Child social functioning domain favored the control group. Conclusions: To our knowledge, this study is the fi rst to compare pulmonary outcomes with HRQOL indicators assessed by serial, standardized, patient-reported outcome measures for patients with CF identifi ed either through newborn screening or diagnosed by use of traditional methods. This study found no benefi ts of newborn screening for pulmonary health or HRQOL after controlling for risk factors. Using WCXR and PFT data collectively helped to identify associations between pulmo...

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Section: Methodsmentioning

confidence: 99%

Relationships Among Health-Related Quality of Life, Pulmonary Health, and Newborn Screening for Cystic Fibrosis

Tluczek

Becker

Laxova

et al. 2011

Chest

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“…Limited daily functioning, poor adherence to treatment, low self-esteem, short stature, and impaired psychosocial outcomes have been reported. [10][11][12][13][14][15] What remains less well known is how young patients cope with the symptoms, prognostic uncertainty, and treatment burden of CF. In-depth insights into people' s beliefs and attitudes can be gained by qualitative research, and synthesis of multiple qualitative studies can provide a broader scope of data across different health care contexts and generate new and more comprehensive understandings of social phenomena.…”

mentioning

confidence: 99%

Children's Experiences of Cystic Fibrosis: A Systematic Review of Qualitative Studies

Jamieson¹,

Fitzgerald

Singh‐Grewal

et al. 2014

PEDIATRICS

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“…Several studies have described psychological problems in patients with CF21 but only three, to our knowledge, have explored the relationship with HRQoL (4,5,22). Szyndler et al (22) found that Australian adolescents with CF exhibited psychopathology levels lower than their non-diseased peers (12.5%); however, increased psychopathology was associated with lower ratings on several quality of life scores.…”

Section: Discussionmentioning

confidence: 99%

“…Szyndler et al (22) found that Australian adolescents with CF exhibited psychopathology levels lower than their non-diseased peers (12.5%); however, increased psychopathology was associated with lower ratings on several quality of life scores. Riekert et al (4) found a 30% prevalence of depressive symptoms among adult patients, a figure similar to ours; in all HRQoL domains, patients with both depressive symptoms and poor lung function reported significantly worse scores than those without depressive symptoms, regardless of lung function status.…”

Section: Discussionmentioning

confidence: 99%

“…Most parents appear to be supportive of NBS, in the belief that early detection and intervention will improve the health of their children and reduce the stress of a delayed diagnosis (30,31). This positive parental attitude might affect patients' beliefs and the likelihood of developing psychological distress, as suggested by Szyndler et al (22) who attributed the low levels of psychopathology found in adolescents to the large screening uptake in Australia. Early psycho-social care in a dedicated structure can also be an explanation.…”

Section: Discussionmentioning

confidence: 99%

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Health-Related Quality of Life and Psychological Distress in Italian Patients With Cystic Fibrosis

et al. 2015

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Objective -The aim of this paper is to describe the health related quality of life (HRQoL) in an unselected hospital-based cohort of patients with Cystic fibrosis (CF), and to explore the association between HRQoL and psychological status. Study design -115 patients with CF (47 children, 26 adolescents and 42 adults) attending the Bambino Gesù Pediatric Hospital in Rome, Italy, were recruited. HRQoL was measured through the Cystic Fibrosis Questionnaire (CFQ), child and adult versions. The Youth Self Report Form and the General Health Questionnaire were used to screen for psychological distress in patients aged below and above 18 respectively. Multivariable logistic regression analysis was performed to explore the relationship between CFQ scores and psychological distress, adjusting for socio-demographic and clinical variables. Results -HRQoL was high, except for Social Limitations, Energy/Wellbeing and Treatment Constraints. Twenty-eight patients (24.4%) scored positive for psychological distress. HRQoL scores below median for Symptoms, Body Image and Social Limitations, and unemployment were significantly associated with the presence of psychological distress, while age, screening-based diagnosis, gender and medical factors were not. Conclusions -Screening for psychological distress should be part of routine management of patients with CF. Opportunities for age-appropriate socialization, work and leisure time should be allowed and encouraged.

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Psychological and family functioning and quality of life in adolescents with cystic fibrosis

Cited by 74 publications

References 32 publications

Relationships Among Health-Related Quality of Life, Pulmonary Health, and Newborn Screening for Cystic Fibrosis

Relationships Among Health-Related Quality of Life, Pulmonary Health, and Newborn Screening for Cystic Fibrosis

Children's Experiences of Cystic Fibrosis: A Systematic Review of Qualitative Studies

Health-Related Quality of Life and Psychological Distress in Italian Patients With Cystic Fibrosis

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