Children with bronchiectasis have poorer lung function than those with cystic fibrosis (CF), likely because of the differing levels of care given to children with these conditions, an Australian study suggests.
The study, “Children with bronchiectasis have poorer lung function than those with cystic fibrosis and do not receive the same standard of care,” was published in the journal Pediatric Pulmonology.
Bronchiectasis and CF both can seriously affect the lungs’ ability to work; in fact, people with CF can develop bronchiectasis as a secondary complication.
Detailed guidelines for how best to provide medical care for children with CF are available. Typically, these involve a multidisciplinary team, and hospital visits to a specialist doctor every few months. Care guidelines for non-CF childhood bronchiectasis tend to be less clear.
Researchers at Sydney Children’s Hospital wondered if children with non-CF bronchiectasis were receiving lesser medical care than their counterparts with CF, which could lead to worse clinical outcomes.
They reviewed medical records at their pediatric hospital, and identified 22 children (average age of 11) with bronchiectasis who were treated between 2010 and 2015. Records of 22 children with CF, matched for age and sex, were also analyzed.
Results showed that those with CF had three to 12 hospital visits with a lung specialist each year, which was significantly more than among children with bronchiectasis (none to three visits). Similar differences were seen for physiotherapy interventions given to the two groups — CF patients had a median of 3.5 annual outpatient physiotherapy interventions, and bronchiectasis a median of 0.
Significant differences also existed in terms of lung health. At the most recent exam, CF children had significantly higher forced vital capacity (FVC) than did those with bronchiectasis (a mean FVC of 100.7 vs. 86.6).
For forced expiratory volume in one second (FEV1), the amount of air that can be exhaled in one second after a deep breath, the best calendar year test recorded 78.7 for bronchiectasis patients, and 105 for those with CF.
“This study has identified a difference of 20% predicted in the FEV1 between the CF and bronchiectasis groups, a significant lung function deficit in children with bronchiectasis,” the researchers wrote, noting that FEV1 in pediatric bronchiectasis patients is “an important clinical outcome marker that predicts morbidity, quality of life, and mortality.”
Children with bronchiectasis also were evaluated for lung function significantly less often — a median of one measure taken each year — than did those with CF (a median of six times each year).
No significant differences were found in terms of weight, height, and body-mass index (BMI) between the two groups.
“Our study has shown that children at our institution with bronchiectasis have significantly poorer lung function compared to age- and sex-matched CF counterparts, and are seen much less frequently by respiratory physicians and physiotherapists,” the researchers wrote.
This was a single-center study with a small number of participants, which limits the strength of the findings, they noted. Nonetheless, “Undertaking this project has highlighted the inconsistency in care and investigations performed, potentially resulting in a discrepancy in the clinical outcomes of children with bronchiectasis within our center.”
Since, however, “a new model of care, similar to that available for patients with CF, was introduced [in their center] to enable high‐quality, multidisciplinary, patient‐centered management to be provided to children with bronchiectasis.”
Future research is needed to determine whether a multidisciplinary respiratory clinic approach can improve the clinical outcomes and quality of life for these children.
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