Cystic fibrosis

Children with bronchiectasis deserve MDT model of care clinics


Dr Bernadette Prentice

Children with bronchiectasis should have access to dedicated multidisciplinary team (MDT) clinics similar to those routinely provided for children with cystic fibrosis (CF), NSW clinicians say.

A study of care provided for 22 children with bronchiectasis at a tertiary centre in Sydney found they were seen far less frequently by respiratory physicians and physiotherapists and had significantly worse lung function compared to a matched cohort of children with CF managed in the same centre.

While almost all children with CF had regular three monthly MDT reviews to monitor their lung function and have microbiology sampling, the children with bronchiectasis were generally seen only once a year for a physician appointment.

The study, led by Dr Bernadette Prentice, a respiratory physician at Sydney Children’s Hospital, examined the hospital records for 22 children with bronchiectasis, with an average age of 11 years. The most common aetiology was postinfective (6/22) but was unknown for many (8/22).

For the primary outcome of lung function the children with bronchiectasis showed significantly worse differences in FEV1 (most recent test 78.6 vs 94.5) compared to 22 matched children with CF. Similar clinically important differences were seen in most recent FVC  (86.5 vs 100.3).

Rates of lung function testing were lower for children with bronchiectasis compared to children with CF (1 vs 6), and 77% children with bronchiectasis had only one test in the previous year. Likewise, respiratory tract sputum or swab microbiology sampling rates were lower for children with bronchiectasis: 10 had no respiratory samples collected in the previous year whereas all children with CF had at least one sample analysed.

In terms of respiratory physician appointments, children with CF had an average of 5.5 visits in the previous year whereas children with bronchiectasis had only one. Children with CF also had more physiotherapy interventions compared to children with bronchiectasis (3.5 vs 0).

Writing in Paediatric Pulmonology, Dr Prentice and colleagues said the 20% difference in FEV1 between children with bronchiectasis and those with CF was likely due to the lack of regular review and early treatment of exacerbations guided by microbiology sampling  of the respiratory tract.

Children with bronchiectasis therefore deserved the same model of care as those with CF, to provide early intervention to slow the rate of lung function decline that will otherwise worsen into adult life, they said.

“If lung function deficits are not corrected in childhood or ongoing damage is not mitigated, children with bronchiectasis are likely to have ongoing lung function decline and grow into adults with significant respiratory morbidity with potential for early mortality,” they wrote.

However the current Australia and NZ guidelines for bronc recommend only six monthly review for children, which may not be enough to manage the burden of bronchiectasis.

To address the discrepancy the hospital has introduced  a new Bronchiectasis Clinic with a multidisciplinary model of care based on that already provided or children with CF.

“The goals of the bronchiectasis clinic include early identification, investigation, and monitoring of bronchiectasis, preservation and stabilisation of lung function, optimisation of airway clearance, normalisation of growth and improved quality of life,” they wrote.

“We also hope to minimise the number of patients with bronchiectasis being misdiagnosed and mismanaged and increase the profile and awareness of bronchiectasis within our institution.”

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