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

BACKGROUND: The present study investigated whether patients with CF who are studying or working report a better HRQoL in comparison to non-working/studying patients.

METHODS: 57 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.

RESULTS: Mean 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.

CONCLUSIONS: CF 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.