Importance The interplay between sickle cell disease (SCD) and reproductive health remains understudied. Menstruation is particularly complex in individuals with SCD, who may have delayed puberty, early menopause, abnormal uterine bleeding, and overlapping SCD-related and menstruation-related pain. Objective To assess menstrual patterns in female patients with SCD, these patients’ access to and use of contraceptive therapy, and clinician practices associated with reproductive health. Design, Setting, and Participants This cross-sectional survey study was conducted in 13 outpatient SCD centers across the US. Female patients with SCD who had reached menarche 12 or more months prior to enrollment between March 1, 2022, and May 31, 2024, and were not pregnant or post menopausal were included. Clinicians involved in the care of enrolled participants were also surveyed on a voluntary basis. Main Outcomes and Measures Age-appropriate menstrual bleeding questionnaires were used to assess menstrual patterns, and global health was evaluated using the Patient-Reported Outcomes Measurement Information System mental and physical health scales. Newly designed patient-facing surveys were used to assessed SCD health and contraceptive use. Clinician-facing surveys assessed participants’ clinical history and care plans after reviewing the patient surveys. Results A total of 211 participants (mean SD age 23.7 10.1 years) were enrolled. All patient-facing surveys with a corresponding clinician survey were completed for 183 participants (86.7%). Abnormal uterine bleeding was noted in 52 participants (24.6%) and was associated with a higher rate of hospitalizations (59.1% hospitalized in this group vs 34.5% in the non–abnormal uterine bleeding group). Most participants (134 of 208 64.4%) endorsed sickle cell pain with menses. Among these participants, 42 (36.5%) had no emergency department (ED) visits, 51 (44.3%) had 1 to 3 ED visits, and 22 (19.1%) had 4 or more ED visits in the 6 months prior to enrollment compared with those who did not endorse sickle cell pain associated with menstruation (38 58.5%, 24 36.9%, and 3 4.6%, respectively). In addition, among the participants who endorsed sickle cell pain, 57 (49.6%) had 0 hospitalizations, 48 (41.7%) had 1 to 3 hospitalizations, and 10 (8.7%) had 4 or more hospitalizations compared with those who did not endorse sickle cell pain associated with menstruation (50 76.9%, 14 21.5%, and 1 1.5%, respectively). There was minimal use of hormonal contraception (40 of 208 participants 19.2%), and 46 participants (22.1%) reported that they had never heard of these medications. After reviewing the patient survey, clinicians reported several intended steps to improve reproductive health for their patients, including reproductive health education (131 participants 72.4%), referral to a reproductive health specialist (90 participants 49.7%), or workup for iron deficiency (29 participants 16.0%) or an underlying bleeding disorder (21 participants 11.6%). Three participating sites established new multidisciplinary clinics for reproductive health and hematology during the enrollment period. Conclusions and Relevance In this multicenter survey study of female individuals with SCD, sickle cell pain with menses was prevalent, and hormonal contraceptive use was low. The findings suggest that brief assessments may facilitate dialogue between female patients with SCD and their sickle cell clinicians and lay the groundwork for improved reproductive health and medical management.
Rush et al. (Tue,) studied this question.