Profile of Reproductive Issues Associated with Different Sickle Cell Disease Genotypes

Abstract

To describe the reproductive variables associated with different sickle cell disease (SCD) genotypes and the influence of contraceptive methods on acute painful episodes among the women with the homozygous hemoglobin S (HbSS) genotype. A cross-sectional study was conducted between September of 2015 and April of 2016 on 158 women afflicted with SCD admitted to a hematology center in the Northeast of Brazil. The reproduction-associated variables of different SCD genotypes were assessed using the analysis of variance (ANOVA) test to compare means, and the Kruskal-Wallis test to compare medians. The association between the contraceptive method and the acute painful episodes was evaluated by the Chi-square test. The mean age of women with SCD was 28.3 years and 86.6% were mixed or of African-American ethnicity. With respect to the genotypes, 134 women (84.8%) had HbSS genotype, 12 women (7.6%) had hemoglobin SC (HbSC) disease genotype, and 12 (7.6%) were identified with hemoglobinopathy S-beta (S-β) thalassemia. The mean age of HbSS diagnosis was lower than that of HbSC disease, the less severe form of SCD ( < 0.001). The mean age of menarche was 14.8 ± 1.8 years for HbSS and 12.7 ± 1.5 years for HbSC ( < 0.001). Among women with HbSS who used progestin-only contraception, 16.6% had more than 4 acute painful episodes per year. There was no statistically significant difference when compared with other contraceptive methods. With respect to reproduction-associated variables, only the age of the menarche showed delay in HbSS when compared with HbSC. The contraceptive method used was not associated with the frequency of acute painful episodes among the HbSS women.

Sickle Cell Disease and Pregnancy

“…These are consequences of various factors like poor nutrition, repetitive infections, blood transfusions, painful crisis, and frequent hospital admissions 5. The onset of menarche is delayed in women with SCD4,6 and is strongly associated with the HbSS phenotype compared to the HbSC phenotype 7. Women with SCD have unique risk factors that may affect their ability to conceive, including chronic inflammation, oxidative stress, transfusion-related hemochromatosis, and ovarian sickling, causing ischemia and reperfusion injury to the ovaries.…”

Provider Views on Sexual and Reproductive Health for Adolescent and Young Adult Women with Sickle Cell Disease

“…INTRODUCTION Adolescent and young adults (AYA) with sickle cell disease (SCD) have shown an increase in average life expectancy with continued advancements in therapy and management. 1 A vast majority of patients with SCD live to adulthood, and many AYA with SCD have expectations for their sexual and reproductive health (SRH) that are similar to their healthy peers. 2,3 Unfortunately, AYA women in the United States ages 15-24 years of age experience the highest rates of sexually transmitted infections and unintended pregnancy.…”