A real world evaluation of the long-term efficacy of strategies to prevent chronic Pseudomonas aeruginosa pulmonary infection in children with cystic fibrosis

Abstract

BackgroundChildren with cystic fibrosis (CF) are susceptible to chronic Pseudomonas aeruginosa (PA) infection. Early eradication of PA has proven short-term efficacy. No studies have evaluated the long- term impact of early eradication for CF patients, particularly those diagnosed by newborn screening (NBS). Our objective was to quantify the long-term impact of early PA eradication on the risk of chronic PA infection in children (0–18 years old) with CF prior to and following the introduction of a province-wide NBS program. MethodsThis 20-year retrospective cohort study compared 94 patients eligible for treatment with inhaled tobramycin at first PA isolation (“recent cohort”) with 27 historical controls (“historical cohort”). ResultsA smaller proportion of patients in the recent cohort developed chronic PA (24% versus 78%; P<0.001); the adjusted risk of chronic infection was 2.90 (95%CI 1.47, 5.76; P=0.002) in the historical vs recent cohort. However, NBS was not independently associated with the risk of chronic PA infection after its introduction. ConclusionsEarly eradication of PA, irrespective of early diagnosis, is associated with reduced risk of chronic PA. However, concomitant improvements in medical care since the introduction of early eradication protocols may have contributed to these long-term observed benefits.