Rationale:
Chronic infection with Pseudomonas aeruginosa (PsA) negatively impacts
lung disease in patients with
primary ciliary dyskinesia (PCD). There is currently limited evidence regarding the efficacy of PsA
antibiotic eradication
therapy (
AET) in children with PCD. Objectives: To assess the effectiveness of
AET of early PsA
infection in children with PCD. Methods: This retrospective study included pediatric patients with a confirmed PCD diagnosis according to the American Thoracic Society guidelines at the Hospital for Sick Children between 2010 and 2022. Children with newly acquired PsA
infection underwent
AET using a stepwise protocol. The protocol included the following steps: step 1, 28 days of
tobramycin inhalation
solution (TIS); step 2, repeat TIS if culture positive after step 1; and step 3, 14 days of intravenous
antibiotics followed by 28 days of TIS if culture positive after step 2. Step 3 was also used for patients who presented with pulmonary exacerbation symptoms. The main outcome was a PsA-negative culture result based on the microbiological results of the first culture after completion of each step of treatment. Results: During the study period, 31 children had a new PsA
infection and underwent
AET. Of the 27 children who had been asymptomatic at the time of the PsA
infection, negative PsA culture results were achieved in 20 (74%) of 27, 1 (14%) of 7, and 5 (83%) of 6 after steps 1, 2, and 3 of
AET, respectively. All four symptomatic patients who initially were treated with step 3 had successful clearance of PsA. The overall cumulative success rate of the protocol for negative culture results after
AET was 97% (30 of 31). For patients in whom
AET was successful, the probability of staying PsA free for at least 1 year was 70%. Conclusions:
AET for early PsA
infection is highly effective in PCD, with sustained efficacy in most individuals. These data suggest that
AET should be considered in all children with PCD who have early PsA
infection.