source: American Society of Hematology

year: 2016

authors: Faith S. Raider, Susan Paulukonis, Ward Hagar, Marsha J Treadwell, Mary Hulihan

Maternal mortality results among women with sickle cell disease (SCD) from recent population-based studies using US hospital discharge data range from 72 (Villers, 2008) to 160 per 100,000 (Alayed, 2014). Researchers use hospital discharge or death certificate data to examine maternal death, as no national SCD surveillance system exists. We analyze California’s surveillance data to describe the in-hospital maternal mortality rate among women meeting a stringent case definition for SCD, compare that rate to rates for all women and for Black women, and describe cases of SCD maternal demise.

The CDC has developed the Sickle Cell Data Collection (SCDC) program to conduct state level surveillance in this disease. SCDC uses a validated case definition: confirmed SCD with known genotype (via newborn screening or clinical case reports) or three or more healthcare encounters in administrative or claims data with SCD ICD-9 codes. California SCDC collected hospitalization data for years 2004-2014 on 1,829 women with SCD. We queried hospitalization data for women ages 15-45 at time of admission for ICD-9 codes for delivery (V27.X) with disposition codes indicating death during the same admission. We used the same query for all women and for Black women to calculate comparable in-hospital maternal mortality rates. We reviewed death records for ICD-10-CM underlying cause of death (COD) codes, and prior ED and inpatient records, and describe the history of the women with SCD who died.

We found 636 delivery hospitalizations among 441 of the 1,829 eligible women with SCD during the 11-year period. The maternal death rate for SCD was 629 per 100,000 (n = 4 of 636 deliveries), compared to 6 per 100,000 deliveries in the general population and 12 per 100,000 among Black women. There was an additional death (#5) among the women with SCD that occurred shortly after discharge; we include this death in the case descriptions, but not in the mortality rates. All of the women with SCD who died were Black. All births were live, singleton deliveries by cesarean surgery.

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