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. 2018 Oct 18:73:e427.
doi: 10.6061/clinics/2017/e427.

Quality of life amongst adolescents and young adults with cystic fibrosis: correlations with clinical outcomes

Daniela W Gancz  1 , Maristela T Cunha  2 , Claudio Leone  3 , Joaquim C Rodrigues  1 , Fabíola V Adde  1
Affiliations

Quality of life amongst adolescents and young adults with cystic fibrosis: correlations with clinical outcomes

Daniela W Gancz et al. Clinics (Sao Paulo). .

Abstract

Objectives: The current study sought to evaluate the quality of life of young patients with cystic fibrosis and correlate these results with the clinical parameters indicative of disease severity.

Methods: This cross-sectional study applied the validated Portuguese version of a cystic fibrosis specific quality of life questionnaire to clinically stable patients aged 14 to 21 years old. The correlations between the questionnaire domain scores and forced expiratory volume in one second (FEV1) values, the Shwachman-Kulczycki score, and body mass index were assessed, and correlations were considered as significant when p<0.05.

Results: A total of 31 patients (11 females; 16.4±2.3 years old) were evaluated, and the median scores on the questionnaire domains ranged from 66.7 to 100. A significant correlation was found between body mass index and the weight (r=0.43, p=0.016) and the eating questionnaire domains (r=0.44, p=0.013); between FEV1 and the physical (r=0.53, p=0.002) and treatment burden (r=0.41, p=0.023) domains; and between the Shwachman-Kulczycki score and the physical (r=0.39, p=0.03), health (r=0.41, p=0.023), and role (r=0.37, p=0.041) domains. A significant difference was found amongst patients with FEV1 values above or below 60% of the predicted value with regard to the role and health domains. No differences in the scores were found according to gender.

Conclusions: The current cystic fibrosis specific quality of life questionnaire scores exhibited wide variability across all domains; however, they indicated a relatively satisfactory quality of life amongst the patients studied. Certain domains exhibited significant correlations with clinical parameters; thus, this instrument has consistent associations with clinical outcomes.

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Conflict of interest statement

No potential conflict of interest was reported.

Figures

Figure 1
Figure 1
A) The correlation between the weight domain and body mass index (BMI); B) the correlation between the eating domain and BMI; C) the correlation between the physical domain and FEV1; and D) the correlation between the treatment burden domain and FEV1.
Figure 2
Figure 2
A) The correlation between the health perceptions domain and the SKS; B) the correlation between the physical functioning domain and the SKS; C) the correlation between the role domain and the SKS.
Figure 3
Figure 3
The CFQ-R domain scores for FEV1 values above and below 60% of the predicted value.
See this image and copyright information in PMC

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