Literature DB >> 8984707

Quality of life in adults with cystic fibrosis.

J Congleton1, M E Hodson, F Duncan-Skingle.   

Abstract

BACKGROUND: Cystic fibrosis is an inherited condition with a high mortality and morbidity. The aims of this study were to assess quality of life in a population of adults with cystic fibrosis, to compare quality of life with published scores from a healthy population and other patient groups, and to examine the relation between quality of life and other measured clinical variables.
METHODS: Patients over 16 years of age attending an adult cystic fibrosis outpatient clinic were surveyed at a time when they were clinically stable. A self-complete questionnaire was administered which comprised the Nottingham Health Profile (NHP) together with six additional questions related to cystic fibrosis.
RESULTS: Completed questionnaires were obtained from 240 subjects (100 women) of median age 26 years (range 16-56). Mean (SD) forced expiratory volume in one second (FEV1) was 49 (26)% predicted, forced vital capacity (FVC) was 68 (26)% predicted, and the FEV1:FVC ratio was 59 (16)%. In this cross sectional study different patterns of perceived quality of life were seen in men and women. In part 1 of the NHP there was an age related trend compared with norms in men, with more distress/disability in the dimensions of emotion, sleep, and social isolation in the older age groups. In women there was no age related trend in the degree of distress/disability compared with norms. The mean score was different from norms in the dimensions of pain, emotion and sleep. For the patients with cystic fibrosis as a whole the scores in part 1 were comparable with published scores of patients with minor non-acute conditions. Scores in part 2 of the NHP for men were different from norms in six of the seven areas of daily living (all except home life). For women the scores were different from norms in the areas of looking after the home, social life, hobbies, and holidays. There were correlations between several of the quality of life dimensions and other measured variables such as FEV1, breathlessness score, and the time spent on home treatment.
CONCLUSIONS: Men and women with cystic fibrosis have different patterns of perceived quality of life, and there is an age related trend of perceived quality of life in men in some dimensions. Quality of life scores in this group, as assessed by the NHP, are similar to those reported in subjects with minor non-acute conditions.

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Year:  1996        PMID: 8984707      PMCID: PMC472619          DOI: 10.1136/thx.51.9.936

Source DB:  PubMed          Journal:  Thorax        ISSN: 0040-6376            Impact factor:   9.139


  5 in total

1.  Heart-lung transplantation for cystic fibrosis and subsequent domino heart transplantation.

Authors:  M H Yacoub; N R Banner; A Khaghani; M Fitzgerald; B Madden; V Tsang; R Radley-Smith; M Hodson
Journal:  J Heart Transplant       Date:  1990 Sep-Oct

2.  Survival and quality of life of cystic fibrosis patients before and after heart-lung transplantation.

Authors:  N Caine; L D Sharples; R Smyth; J Scott; T Hathaway; T W Higenbottam; J Wallwork
Journal:  Transplant Proc       Date:  1991-02       Impact factor: 1.066

Review 3.  Cystic fibrosis.

Authors:  M E Hodson
Journal:  Postgrad Med J       Date:  1984-03       Impact factor: 2.401

4.  Perceived health: age and sex comparisons in a community.

Authors:  S M Hunt; J McEwen; S P McKenna
Journal:  J Epidemiol Community Health       Date:  1984-06       Impact factor: 3.710

5.  A cost description of an adult cystic fibrosis unit and cost analyses of different categories of patients.

Authors:  M Robson; J Abbott; K Webb; M Dodd; J Walsworth-Bell
Journal:  Thorax       Date:  1992-09       Impact factor: 9.139

  5 in total
  11 in total

1.  Comparison of three generic questionnaires measuring quality of life in adolescents and adults with cystic fibrosis: the 36-item short form health survey, the quality of life profile for chronic diseases, and the questions on life satisfaction.

Authors:  L Goldbeck; T G Schmitz
Journal:  Qual Life Res       Date:  2001       Impact factor: 4.147

2.  The revised German Cystic Fibrosis Questionnaire: validation of a disease-specific health-related quality of life instrument.

Authors:  Kerstin Wenninger; Pierre Aussage; Ulrich Wahn; Doris Staab
Journal:  Qual Life Res       Date:  2003-02       Impact factor: 4.147

3.  Development of the Cystic Fibrosis Questionnaire (CFQ) for assessing quality of life in pediatric and adult patients.

Authors:  Bernadette Henry; Pierre Aussage; Cécile Grosskopf; Jean-Marie Goehrs
Journal:  Qual Life Res       Date:  2003-02       Impact factor: 4.147

4.  Quality of life in patients with cystic fibrosis and their parents: what is important besides disease severity?

Authors:  D Staab; K Wenninger; N Gebert; K Rupprath; S Bisson; M Trettin; K D Paul; K M Keller; U Wahn
Journal:  Thorax       Date:  1998-09       Impact factor: 9.139

Review 5.  Quality of life in children and adolescents with cystic fibrosis: implications for optimizing treatments and clinical trial design.

Authors:  Janice Abbott; Louise Gee
Journal:  Paediatr Drugs       Date:  2003       Impact factor: 3.022

Review 6.  Sleep disorders in cystic fibrosis: A systematic review and meta-analysis.

Authors:  Joel Reiter; Alex Gileles-Hillel; Malena Cohen-Cymberknoh; Dennis Rosen; Eitan Kerem; David Gozal; Erick Forno
Journal:  Sleep Med Rev       Date:  2020-02-19       Impact factor: 11.609

7.  Do patient-reported outcomes have a role in the management of patients with cystic fibrosis?

Authors:  M Sam Salek; S Jones; M Rezaie; C Davies; R Mills; R I Ketchell
Journal:  Front Pharmacol       Date:  2012-03-12       Impact factor: 5.810

8.  Gender differences in health-related quality of life of adolescents with cystic fibrosis.

Authors:  Renata Arrington-Sanders; Michael S Yi; Joel Tsevat; Robert W Wilmott; Joseph M Mrus; Maria T Britto
Journal:  Health Qual Life Outcomes       Date:  2006-01-24       Impact factor: 3.186

9.  The cost-effectiveness of neonatal screening for cystic fibrosis: an analysis of alternative scenarios using a decision model.

Authors:  Neil Simpson; Rob Anderson; Franco Sassi; Alexandra Pitman; Peter Lewis; Karen Tu; Heather Lannin
Journal:  Cost Eff Resour Alloc       Date:  2005-08-09

10.  Health-related quality of life in children with cystic fibrosis: validation of the German CFQ-R.

Authors:  Anne Schmidt; Kerstin Wenninger; Nadja Niemann; Ulrich Wahn; Doris Staab
Journal:  Health Qual Life Outcomes       Date:  2009-12-02       Impact factor: 3.186

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