C61-02 Importance of Establishing a Primary Ciliary Dyskinesia (PCD) Center of Excellence

Rationale Primary Ciliary Dyskinesia (PCD) is a rare lung disease that affects the motile cilia of the body and making a diagnosis can be challenging. We seek to demonstrate that the establishment of an accredited PCD Center equipped with nasal Nitric Oxide (nNO) testing impacts the PCD diagnostic process. Methods Using a retrospective chart review, we studied all patients at Monroe Carell Jr. Hospital at Vanderbilt who had nNO testing. We recorded demographic data, type of referral, time from referral to PCD consultation, and patient insurance. Patients were categorized by the time of their first PCD consultation at Monroe Carell, comparing those seen before and since the implementation of nNO testing and the establishment of our accredited PCD center in 2018. Results A total of 190 patients underwent nNO testing at Monroe Carell, which included 23 seen before 2018 and 167 seen since 2018 for suspected PCD. Among these patients, the median age for PCD referral was 7.5 years in those seen before 2018 and 8.7 years in those seen since 2018. Patients with private insurance comprised 52% of the total patients seen before 2018 and 64% of the total patients seen since 2018. Patients with public insurance comprised 39% of the total patients seen before 2018 and 30% of the total patients seen since 2018. Internal pediatric pulmonary referrals comprised 65% of total referrals before 2018 and 46% of total referrals since 2018. The median time to PCD consultation from internal pediatric pulmonary referrals did not change significantly. External referrals comprised 17% of the total patients seen before 2018 and 34% of the total patients seen since 2018. Lastly, 10 of the 23 (44%) patients seen before 2018 were diagnosed with PCD, whereas 16 of the 167 (10%) patients seen since 2018 were diagnosed with PCD. The figure illustrates the broad geographic distribution of referrals, based on patients’ county of residence. Conclusion Establishment of the PCD center resulted in an increase in external referrals from greater distances. Despite the increase in testing, the diagnosis rate declined, suggesting those referred after establishment of the center included those with more complex and broader symptoms of suppurative lung disease. Future research could investigate the diagnostic complexity of PCD, including criteria for referral and nNO testing, which may shed light on differences in testing rates for patients who are privately insured compared to those who are publicly insured.