Review: Quality of Life in Children with Non-cystic Fibrosis Bronchiectasis

Abstract

Non-cystic fibrosis bronchiectasis (NCFB) has gained renewed interest, due to its increasing health-care burden. Annual mortality statistics in England and Wales showed that under 1,000 people die from bronchiectasis each year, and this number is increasing by 3% yearly. Unfortunately, there is a severe lack of well-powered, randomized controlled trials to guide clinicians how to manage NCFB effectively. Quality-of-life (QOL) measures in NCFB are an important aspect of clinical care that has not been studied well. Commonly used disease-specific questionnaires in children with NCFB are the St George’s Respiratory Questionnaire, Short Form-36, the Leicester Cough Questionnaire, and the Parent Cough-Specific Quality of Life questionnaire (PC-QOL). Of these, only the PC-QOL can be used in young children, as it is a parent-proxy questionnaire. We reviewed pediatric studies looking at QOL in children with NCFB and cystic fibrosis. All types of airway clearance techniques appear to be safe and have no significant benefit over each other. Number of exacerbations and hospitalizations correlated with QOL scores, while symptom subscales correlated with lung function, worse QOL, frequent antibiotic requirements, and duration of regular follow-up in only one study. There was a correlation between QOL and age of diagnosis in children with primary ciliary dyskinesia. Other studies have shown no relationship between QOL scores and etiology of NCFB as well as CT changes. As for treatments, oral azithromycin and yoga have demonstrated some improvement in QOL scores. In conclusion, more studies are required to accurately determine important factors contributing to QOL.

Exacerbations and Pseudomonas aeruginosa colonization are associated with altered lung structure and function in primary ciliary dyskinesia

“…The exacerbations in PCD, defined as an acute increase in respiratory symptoms [14], are predictors of severity of disease and poor quality of life [10] and negatively impact on lung function, as bacterial infections are associated with morbidity and mortality in these patients [13]. It was reported that about 25% of children with PCD fail to recover to baseline lung function within 3 months following a pulmonary exacerbation treated with intravenous antibiotics and may never regain pre-exacerbation spirometry [15].…”

“…Respiratory infections are major determinants of morbidity and mortality in patients with PCD, as they are correlated to a poor quality of life [10] and to functional decline [11]. Haemophilus influentiae and Streptococcus pneumoniae appear to be the most common pathogens isolated from patients with PCD during early childhood [4,12].…”

Paediatric and adult bronchiectasis: Monitoring, cross‐infection, role of multidisciplinary teams and self‐management plans

“…CC‐QoL questionnaire for older children able to self‐report . Relationship between QoL measures and other bronchiectasis markers of severity…”

“…Given the difficulties of direct reporting for children of preschool age, parent‐proxy reporting is often the practice of choice. Parent‐proxy cough‐specific QoL (PC‐QoL) is the commonly used tool in paediatric studies . For older children (>7 years), a child self‐reporting instrument, chronic cough‐specific QoL (CC‐QoL) questionnaire is a more reliable and sensitive HR‐QoL from the child's perspective …”

Non-CF Bronchiectasis as a Possible Indicator of a Primary Immunodeficiency: Diagnosis, Clinical Course, and Quality of Life in a Pediatric Cohort

“…NCBE are associated with comorbidities such as anxiety, depression, and fatigue [25]. Reduced quality of life due to respiratory symptoms and decreasing ability to participate in activities of daily living have been reported in NCBE [13,25,29].…”