Table 1.

Summary of existing research in girls and women with sickle cell disease with attention to date, topic, conclusions, and opportunities for additional study

Category	In-text reference	Summary of published findings	Opportunities for future investigation
Menstruation: dysmenorrhea and acute vaso-occlusive pain	Citation 5	Acute vaso-occlusive pain is temporally associated with menstruation in a subset of women with SCD Dysmenorrhea is associated with having acute vaso-occlusive pain associated with menstruation	Pathophysiology of acute vaso-occlusive pain that occurs in association with menstruation Optimal hormonal contraception for menses-associated pain
Contraception	Citation 9, 10, 11, 79, 80	Use of low reliability barrier methods of contraception is common in women with SCD Unintended pregnancy is common in women with SCD	Safety of contraception use in women with SCD Barriers to treatment acceptance Care structures to prevent unplanned pregnancies
Cross-sex hormone therapies	Citation 83	Some individuals with SCD are transgender and will request hormone therapy	Safety of exogenous oestrogen and testosterone
Pregnancy	Citation 52, 53, 59,	Maternal mortality is increased in women with SCD Pregnant women with SCD are at risk for hypertensive disorders of pregnancy, adverse fetal outcomes (intrauterine growth restriction, fetal demise, & malformations), miscarriage and pre-term delivery Transfusion reduces acute pain and acute chest episodes during pregnancy	Optimal treatment for SCD during pregnancy Optimal supportive care during pregnancy Determine teratogenicity of SCD-specific therapies Implementation & cost-effectiveness of comprehensive care systems
Breastfeeding	Citation 60	Women without SCD have low levels hydroxyurea in breast milk after a single dose	Preferences and practices relating to breastfeeding in women with SCD Feasibility of pump and dump with hydroxyurea therapy during lactation
Ovarian reserve	Citation 19, 21, 22	Adult women with SCD have accelerated decline in ovarian reserve Diminished ovarian reserve occurs in a subset of girls and women with SCD	Extent to which SCD and its treatments affect ovarian reserve Correlation of ovarian reserve testing with pregnancy outcomes
Fertility preservation	Citation 25, 33	Girls and women with SCD have used ovarian tissue cryopreservation and oocyte and embryo preservation as fertility preservation strategies	Optimal timing of fertility preservation interventions Indications for fertility preservation Optimal health policies to guarantee access to care
In Vitro Fertilisation with Preimplantation Genetic Testing	Citation 67, 68	Parents of children with SCD are interested in IVF + PGT IVF + PGT has been used successfully in couples affected by sickle cell trait and SCD	Outcomes from large contemporary data sets Cost benefit analysis and education, particularly for IVG + PGT to prevent SCD and produce HLA-matched siblings
Menopause	Citation 15	Brazilian women with SCD enter menopause in their late 40’s, and sooner with hydroxyurea	Epidemiology and risk factors for early menopause Interaction of menopausal and SCD-related symptoms