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Effectiveness of preoperative screening for sickle cell disease in a population with a newborn screening program: a
James D O'Leary1, Isaac Odame, Carolyne Pehora
1Department of Anesthesia and Pain Medicine, The Hospital for Sick Children, University of Toronto, ON, Canada.
Insights
Selective preoperative screening for sickle cell disease (SCD) is ineffective in Ontario due to universal newborn screening. This approach fails to identify new SCD cases and leads to unnecessary repeat testing for sickle cell trait.
Area of Science:
- Hematology
- Pediatric Screening
- Public Health
Background:
- Clinical guidelines advocate preoperative screening for sickle cell disease (SCD) in high-prevalence ethnic groups.
- Ontario implemented universal newborn screening for sickle hemoglobinopathy in 2006.
- The study evaluates selective preoperative screening effectiveness in a universal newborn screening context.
Purpose of the Study:
- To assess the efficacy of ethnicity-based preoperative sickle cell disease screening.
- To determine if selective screening identifies additional SCD cases post-universal newborn screening.
- To analyze the impact on repeat testing and perioperative outcomes.
Main Methods:
- Retrospective review of a hospital hematology laboratory database.
- Identification of children undergoing preoperative sickle cell screening (Nov 2006 - Apr 2010).
- Analysis of perioperative outcomes for children with abnormal hemoglobin results.
Main Results:
- 710 children underwent preoperative screening; 35 had abnormal results.
- Only one new SCD diagnosis (0.14%) was identified, not caught by newborn screening.
- 33 children (4.65%) were identified with sickle cell trait, leading to potential repeat testing.
Conclusions:
- Ethnicity-based preoperative screening is ineffective for identifying additional SCD cases in Ontario's universal newborn screening population.
- The current selective screening strategy results in unnecessary repeat testing.
- An algorithm is proposed to guide physicians on appropriate preoperative sickle cell screening decisions.
Purpose:
Published clinical practice guidelines recommend that all patients from ethnic groups with a high prevalence of sickle cell disease (SCD) undergo preoperative screening for this hemoglobinopathy. Newborn Screening Ontario initiated a universal sickle hemoglobinopathy screening program in 2006 as part of its regional newborn screening program. The primary objective of this study was to determine the effectiveness of selective preoperative screening for SCD based on at-risk ethnicity in Ontario, a region that has a universal newborn sickle hemoglobinopathy screening program.
Methods:
The hematology laboratory database at our hospital was searched to identify all children who underwent preoperative sickle cell screening in the 42-month period starting in November 2006 (when the newborn sickle hemoglobinopathy screening program was introduced) and ending in April 2010. Medical records of all children testing positive on hemoglobin analysis were reviewed to determine perioperative outcomes.
Results:
Our search strategy identified 710 children who were born after the introduction of newborn screening and who underwent preoperative screening. Thirty-five of these children had abnormal sickle solubility tests - one (0.14%) was a new diagnosis (not identified by newborn screening), one had been diagnosed previously, and 33 (4.65%) were identified as having sickle cell trait. Sixty children had more than one preoperative screening test performed (range, 2-4). Six of the 35 children with abnormal sickle solubility results underwent repeated testing.
Conclusions:
Preoperative screening based on at-risk ethnicity alone is an ineffective method of identifying additional children with SCD in Ontario, a population with universal newborn sickle hemoglobinopathy screening. In an effort to avoid unnecessary repeat testing, we propose an algorithm to help physicians decide whether to initiate preoperative sickle cell screening for children.
