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Background: CFTR modulators are life-changing therapy for people with CF. During preclinical studies of ivacaftor, cataracts were identified in juvenile rat pups. Subsequently, noncongenital cataracts have been reported in children receiving CFTR modulator therapy [1], so baseline and annual slit lamp eye examinations for cataract development are recommended for children with CF (aged <18). At Children’s Mercy Kansas City (CMKC), our standard practice is to conduct baseline and annual ophthalmologic examinations to assess for cataracts in children with CF prescribed CFTR modulator therapy, but there was no formal process for tracking adherence to this standard and ensuring that examinations were completed. A quality improvement project was undertaken in May 2022 to determine the rate of completion of annual eye exams and increase adherence to our annual monitoring standard.

Methods: To improve adherence to annual monitoring standards, a quality improvement taskforce including CF care team nurses and pharmacists was developed. A database was used to document baseline and follow-up eye examinations. A standardized communication process was developed and deployed that included an eye exam brochure (Figure 1), messaging to caregivers and patients through the electronic medical record (EMR), and reminders on clinic departure or discharge paperwork. Additionally, children with CF who required eye examinations were identified during preclinic huddles, and that information was communicated to the CF care team. To improve tracking of completed eye exam reports, standardized notation of the searchable term “eye exam” was included in EMR documentation.

Results: At CMKC, at least one eye examination was documented for 116 (72.5%) of 160 children with CF prescribed CFTR modulator therapy before implementation of the quality improvement project; after implementation, eye examinations were documented for 129 (81%). 64 (40%) children with CF at CMKC had eye exams completed in the year before project implementation. After project implementation, 72 (45%) PwCF had an eye exam completed per monitoring recommendations.

Conclusions: Using quality improvement methodology, the CMKC CF care team developed a tracking tool and standardized process for communicating annual eye examination education in children with CF prescribed CFTR modulators. An increase in the rate of annual eye exam completion of 5% was observed after implementation of the process. With continued education and awareness, we anticipate higher rates of annual eye examination completion.


Pediatrics | Pulmonology


Presented at the North American Cystic Fibrosis Conference, Nov 2-4, 2023: Phoenix, AZ.

Increasing annual eye exam completion for children with cystic fibrosis receiving CFTR modulators