Comparison of patient care pathway between primary ciliary dyskinesia and cystic fibrosis

Abstract

Since the set-up of cystic Fibrosis (CF) centre in 2003, significant improvement was done in the patient care pathway. We aim to assess the care pathway in primary ciliary dyskinesia (PCD) in the light of CF experience,

Methods: CF and PCD patients (adults and children) were retrospectively compared while focusing on specialists’ consultations, number of visits and hospitalization from 2010 to 2018.

Results: We reviewed 126 CF patients with 1635 hospitalizations (122 patients) and 6519 visits (126 patients) and 74 PCD patients with 276 hospitalizations (63 patients) and 2631 visits (74 patients). The sex ratio was 0.91 and 0.95 for CF and PCD respectively. A median of 7 new CF patient and 5.5 PCD patients were noticed per year. The average number of consultations per year was 6.3 for CF and 5.1 for PCD (P=0.01) including 1.4 vs.1.8 for ENT (p=0.06), 2.2 vs 2.8 for pulmonologist (p= 0.03) and 2.1 vs. 0.7 for the physiotherapist (p<0.001). The average (median) time between appointments by specialty for CF and PCD was 10.3 (5) vs. 9.6 (5.3) for ENT, 8.1 (3.2) vs. 6.1 (4.1) for the pulmonologist (adult or paediatrician) and 9.6 (3) vs 9 (5) for physiotherapist. CF patients have a greater use of the day hospital than PCD patients (14.3 vs. 3.7 %, p<0.001) whereas PCD patients have more consultations (79.9 vs. 90.5 %, p<0.001).

Conclusion: There is significant need to improve the care pathway in PCD patients with a special focus on the use of physiotherapists and day hospitalization.