Self-Reported Physical and Psychological Symptom Burden in Adults with Cystic Fibrosis

Abstract

Symptom burden is a key component of health-related quality of life in patients with cystic fibrosis (CF). To examine symptom prevalence and characteristics of adults with CF, we administered the Memorial Symptom Assessment Scale (MSAS), a previously validated measure of symptom burden, to CF patients enrolled in the Project on Adult Care in CF. The mean age of the 303 respondents (response rate 91%) was 32.8 years (range, 19–64); 58% were female, and their mean baseline pulmonary function (FEV₁ % predicted) was 69% (SD 28%). The median number of symptoms reported was 10, and there was no difference in the number of symptoms reported based on age, gender, or FEV₁. The most prevalent symptoms were cough (94%), shortness of breath (77%), and lack of energy (77%). Lack of energy and irritability caused the highest level of distress. MSAS symptom subscales were only moderately correlated with symptom status domains from existing CF health-related quality of life measures. Factor analysis led to the development of three distinct MSAS CF-symptom subscales, each with high internal validity. These findings show that adults with CF have a high symptom burden, particularly with respiratory and psychological symptoms, and that the new MSAS CF-specific subscales are a reliable measure of symptom distress in the CF population.