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J Pediatr. 2019 Dec;215:164-171.e2. doi: 10.1016/j.jpeds.2019.08.038.

Caregiver Burden Due to Pulmonary Exacerbations in Patients with Cystic Fibrosis.

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Vertex Pharmaceuticals Incorporated, Boston, MA.
Department of Pediatrics, Pediatric Pulmonology, Jena University Hospital, Jena, Germany; Pediatric Pulmonology and Cystic Fibrosis, Brandenburg Medical School, University Hospital, Brandenburg, Germany.
Respiratory Department, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland.
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH; Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
AMF Consulting, Los Angeles, CA.
Division of Pulmonary Medicine, Boston Children's Hospital, Boston, MA.
Division of Pulmonary and Sleep Medicine, Seattle Children's Hospital, Seattle, WA. Electronic address:



To describe the poorly understood burden of pulmonary exacerbations experienced by primary caregivers of children (aged 2-17 years) with cystic fibrosis (CF), who frequently require prolonged hospitalizations for treatment of pulmonary exacerbations with intravenous (IV) antibiotics.


In this prospective observational study, 88 caregivers in Germany, Ireland, the United Kingdom, and the US completed a survey during pulmonary exacerbation-related hospitalizations (T1) and after return to a "well state" of health (T2). The impact of pulmonary exacerbations on caregiver-reported productivity, mental/physical health, and social/family/emotional functioning was quantified.


Primary caregivers of children with CF reported significantly increased burden during pulmonary exacerbations, as measured by the 12-item Short-Form Health Survey mental health component and the Work Productivity and Activity Impairment: Specific Health Problem absenteeism, presenteeism, work productivity loss, and activity impairment component scores. Compared to the "well state," during pulmonary exacerbations-related hospitalization caregivers reported lower physical health scores on the Child Health Questionnaire-Parent Form 28. Quality-of-life scores on the Caregiver Quality of Life Cystic Fibrosis scale and total support score on the Multidimensional Scale of Perceived Social Support did not differ significantly between T1 and T2. More caregivers reported a negative impact on family/social/emotional functioning during pulmonary exacerbations than during the "well state."


Pulmonary exacerbations necessitating hospitalization impose a significant burden on primary caregivers of children with CF. Preventing pulmonary exacerbations may substantially reduce this burden.


chronic disease; hospitalization; intravenous antibiotics; parent; pediatric; prospective study; quality of life; work productivity loss productivity

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