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J Cyst Fibros. 2018 Dec 23. pii: S1569-1993(18)30942-1. doi: 10.1016/j.jcf.2018.12.004. [Epub ahead of print]

Measuring recovery in health-related quality of life during and after pulmonary exacerbations in patients with cystic fibrosis.

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Medical University of South Carolina, 96 Jonathan Lucas Street, 812-CSB, Charleston, SC 29425, USA. Electronic address:
Formerly of Vertex Pharmaceuticals Incorporated, 50 Northern Avenue, Boston, MA 02210, USA. Electronic address:
Quality Metric Incorporated, Now Part of Optum Insight, 1301 Atwood Avenue, Johnston, RI 02919, USA. Electronic address:
Vertex Pharmaceuticals Incorporated, 50 Northern Avenue, Boston, MA 02210, USA. Electronic address:
Nicklaus Children's Research Institute, 3100 SW 62nd Avenue, Miami, FL 33155, USA. Electronic address:



We explored the time-dependent impact of pulmonary exacerbations (PEx) on health-related quality of life (HRQoL) using Cystic Fibrosis Questionnaire-Revised (CFQ-R) data from 2 large cystic fibrosis (CF) trials.


This exploratory post-hoc analysis evaluated the impact of PEx on CFQ-R domains of functioning in 80 patients with CF (homozygous for F508del-CFTR), aged ≥14 years randomized to placebo in the TRAFFIC and TRANSPORT trials who experienced 1 PEx.


Scores on the CFQ-R were significantly lower within 1 week of PEx start in 8 out of 12 domains (Respiratory Symptoms, Physical Functioning, Emotional Functioning, Health Perceptions, Role Functioning, Social Functioning, Eating, and Vitality). Patients whose PEx was treated with hospitalization or intravenous antibiotics had greater reductions in some domains of HRQoL compared with those treated with oral antibiotics. In the immediate weeks post-PEx, improvement was seen on Emotional Functioning, Respiratory Symptoms, and Health Perceptions, while further decline was seen for Eating, Physical Functioning, Role Functioning, Vitality, and Weight. For some measures (Physical Functioning, Vitality), full recovery to pre-PEx levels took several weeks.


Pulmonary exacerbations have significant effects on multiple domains of HRQoL, and recovery across multiple domains post-PEx can take several weeks. These findings provide insight into the impact of PEx on patient HRQoL and recovery post-PEx.

CLINICAL TRIAL REGISTRATION: identifiers, NCT01807923 and NCT01807949.


Cystic fibrosis; Pulmonary exacerbations; Quality of life

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