Sickle Cell Disease and Pregnancy: Does Outcome Depend on Genotype or Phenotype?

Abstract

Objective: Women with Sickle Cell Disease (SCD) who become pregnant are at risk for serious maternal and fetal complications. Our objective was to determine if pregnancy outcome is dependent on phenotype. Methods: Retrospective cohort study of pregnant women with SCD, including hemoglobin (Hb) SS, Hb SC, and Hb Sβ-thalassemia, between January 1999 and December 2008). Antenatal and neonatal outcomes were compared between pregnancies with painful episodes and those without. The primary outcome was preterm birth (PTB) <37 weeks. Secondary outcomes included maternal medical complications, antenatal complications, delivery outcomes, and neonatal outcomes. Results: 31 women were included (18 (58%) with painful episodes, 13 (42%) without painful episodes). The median number of painful episodes was 2.5 (1 - 19) and these women required a median of 13 total days (1 - 59) of inpatient treatment. At delivery, women who had experienced painful episodes had lower Hb levels and were more likely to be taking chronic narcotic pain medications. The overall incidence of PTB <37wks was 55% and was not significantly different between groups (11 [61%] with painful episodes versus 6 [46%] without painful episodes; p = 0.485). Secondary outcomes were also not significantly different between groups. There was one maternal death. Conclusion: Adverse obstetrical out-comes were more common among women with sickle cell disease who experienced painful crises however, in this small sample, the difference were not statistically significant

The accuracy of using ICD‐9‐CM codes to determine genotype and fever status of patients with sickle cell disease

“…To the Editor: ICD‐9‐CM codes are commonly used by health services researchers and administrators investigating quality metrics to identify patients, but no study has determined their accuracy for classifying patients with sickle cell disease (SCD) by genotype and fever status . We conducted a retrospective chart review to compare ICD‐9‐CM codes to manual chart review for patients 3 months to 21 years old with SCD who presented to the Emergency Department (ED) at a Midwest urban children's hospital between January 1, 2004 and December 31, 2012.…”