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

Abstract

Background-Cystic fibrosis is the most common inherited disease with a fatal outcome in industrialised nations. With the improvement in life expectancy, supporting patients and their families in adapting to life with this chronic progressive disease has become increasingly important. The aim of the present study was to investigate the relationship between health related quality of life (HRQOL) in this population, severity of disease, and cognitive/behavioural factors such as subjective health perception and ways of coping. Methods-A sample of 89 adolescent and adult patients with cystic fibrosis and 125 parents of younger patients with cystic fibrosis completed questionnaires on health related quality of life and on ways of coping with the illness. Parents were asked to fill out the questionnaires regarding their own quality of life and coping. Multiple regression analyses were performed to examine the relationship between diVerent predictor variables and quality of life. Results-After accounting for the impact of disease severity and hours of treatment per day, the subjective health perception of patients significantly explained variance in their quality of life. Ways of coping were also significantly correlated with HRQOL. In parents the most important factor in explaining variance of HRQOL seems to be the coping style, whereas disease severity of the child and subjective health perception did not show any influence. Conclusions-The findings support the important role of cognitive and behavioural factors in specific subjective health perception and ways of coping in the adaptation to this severe chronic disease, both in patients themselves and in parents. The results call for a careful assessment of issues of coping and professional support for families of patients with cystic fibrosis in the early course of disease. (Thorax 1998;53:727-731)

Avaliação da qualidade de vida de pacientes com fibrose cística por meio do Cystic Fibrosis Questionnaire

“…(6,19,25) Pulmonary impairment has been described in the literature as an important factor for the onset of emotional stress in individuals with CF, since it correlates with higher morbidity and mortality, requiring treatments that are even more demanding. (21,25) Our results show that children ≥ 6 and < 14 years of age have a satisfactory QoL, regardless of FEV 1 . In contrast, adolescents and adults with greater pulmonary impairment had worse scores in the respiratory, social role, body image, and physical domains.…”

Anxiety, depression, and life satisfaction in parents caring for children with cystic fibrosis

“…As expected, anxious and depressive symptoms were negatively correlated with General and Health-related Life Satisfaction and with higher levels of symptom severity, a finding that converges with others on quality of life in patients with CF and their families. 49 …”

Linguistic validation of cystic fibrosis quality of life questionnaires

“…Validação lingüística dos questionários de qualidade de vida em fibrose cística Tatiana Rozov 1 , Maristela T. Cunha 2 , Oliver Nascimento 3 , Alexandra L. Quittner 4 , José R. Jardim 5 …”

“…Recentemente, tem sido enfatizado que a avaliação da QV constitui uma medida clínica adicional muito importante nos estudos clínicos da FC e na monitorização individual 3 . Vários instrumentos e questionários doença-específicos que avaliam a QV de FC foram desenvolvidos nos últimos 20 anos, tanto para crianças como para adultos [4][5][6][7][8][9] . Alguns desses questionários foram validados para as diversas línguas nesta última década [10][11][12] .…”