Province-Wide Biliary Atresia Home Screening Program in British Columbia: Evaluation of First Two Years

Background: Biliary atresia (BA), a rare newborn liver disease, is the leading cause of liver-related death in children. Early disease recognition and timely surgical Kasai hepatoportoenterostomy (KP) offers long-term survival without liver transplant. Universal BA screening in Taiwan using infant stool color cards (ISCCs) has proven effectiveness. We report our experience with ISCC BA screening in a province-wide program in British Columbia (BC). Objectives: To assess program performance and cost from launch April 1, 2014-March 31, 2016. Methods: ISCCs distributed to families upon maternity ward discharge. Parents were instructed to monitor their infant's stool color for one month and contacted the screening center with concerns. The number of live births, ISCC distribution, BA cases, and costs were recorded. Cases with Program screen success had both acholic stool recognition (ISCC screen success) and timely referral for BA. Results: All 126 maternity units received ISCCs. Of 87,583 live births there were 6 BA cases. Of the five cases with ISCC Screen Success three had Program Screen Success. The median KP age in the program screen success and failure groups was 49 (42–52) and 116 (49–184) days respectively. Program sensitivity was 50%, specificity 99%, PPV 4% and NPV 99%. A random sample of 1054 charts at BC Children's Hospital found an ISCC distribution rate of 94%. After a phase-in period, the annual program cost was $30,033.82, and the ISCC cost per birth was $0.68. Conclusions: The screening program has high specificity and distribution with low cost. Successful program case identification had earlier age at KP. Program modifications aim to improve sensitivity. Longer-term studies will determine program impact on health outcomes. Address correspondence and reprint requests to Dr. Richard A. Schreiber, Room K4-200, BC Children's Hospital, 4480 Oak Street, Vancouver, BC, Canada, V6H3V4 (e-mail: rschreiber@cw.bc.ca). Received 9 September, 2017 Accepted 14 February, 2018 List of Authors and his/her Respective Roles: Jessica P. Woolfson: Acquisition, analysis and interpretation of data for the work; Drafting and revising the work for important intellectual content, Final approval of the version to be published; Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. Richard A. Schreiber: Substantial contributions to the conception or design of the work; analysis, or interpretation of data for the work; Drafting the work or revising it critically for important intellectual content; Final approval of the version to be published; Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. Alison E. Butler: Acquisition and analysis of data for the work; Drafting the work or revising it critically for important intellectual content; Final approval of the version to be published; Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved Interpretation of data for the work. Julie MacFarlane: Acquisition of data for the work; Drafting the work or revising it critically for important intellectual content; Final approval of the version to be published; Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. Janusz Kaczorowski: Substantial contributions to the conception or design of the work; Drafting the work or revising it critically for important intellectual content; Final approval of the version to be published; Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. Lisa Masucci: Substantial contributions to the conception or design of the work; Drafting the work or revising it critically for important intellectual content; Final approval of the version to be published; Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. JP Collet: Substantial contributions to the conception or design of the work; Drafting the work or revising it critically for important intellectual content; Final approval of the version to be published; Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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