Reproductive Health Surveys Administered in Sickle Cell Clinics Can Inform Personalized Care and Multidisciplinary Collaboration

Background: Females with sickle cell disease (SCD) aged 10-39 years old experience 26% more acute pain episodes than their male counterparts. An association between acute SCD pain and the menstrual cycle has been established. Hormonal contraception may decrease the frequency of menstrual-associated acute SCD pain. In this study, we aimed to assess menstrual patterns, awareness, education, and access to hormonal contraception. In addition, we aimed to determine changes in practice based on patient survey responses.

Methods: We conducted a cross-sectional survey study of 212 individuals with a uterus (IWU) with SCD (70 adolescent and 142 adult), and their providers at 13 SCD centers across the US between March 2022 and May 2024. Patient and provider surveys inquiring about quality of life, healthcare utilization and medications, and exposure to reproductive health education were created in collaboration with members of the Foundation for Women and Girls with Blood Disorders Learning Action Network. In addition, a validated menstrual bleeding questionnaire (MBQ) was used to screen for abnormal uterine bleeding. MBQ and patient surveys were completed during routine sickle cell visits. Providers were asked to complete an optional survey for each patient survey completed. Preliminary data from these measures were presented at the ASH 2023 annual meeting. After accrual was complete, participating sites were queried regarding SCD-focused reproductive care clinics at their institution.

Results: The mean MBQ scores for adult (18.1, SD 11.5) and adolescent (16.3, SD 7.3) participants with SCD were not significantly different from the mean validated MBQ scores in the general population (adult: 10.8, SD 8.8; adolescent:19.6, SD 7.6). However, 134 participants (65%) reported sickle cell pain around menstruation. Only 37 participants (17%) reported being on hormonal contraception. Surprisingly, 45 participants (21%) reported not having heard of birth control/hormonal contraception, including 25 adult participants.

For the 212 patients enrolled, 185 provider surveys were completed (87%). After review of the MBQ and patient survey, providers selected verbal reproductive health education as a next step of their care 127 times, further evaluation for iron deficiency and/or bleeding disorder workup 42 times, and referral to specialty care for female reproductive health 76 times. In 94 instances, the provider endorsed “I have no concerns for this patient based on their MBQ and survey responses.”

A final survey was sent to participating sites after the end of the enrollment period. This revealed 3 of 13 sites (23%) had a combined hematology and gynecology/adolescent medicine clinic established before joining this study. One additional site had a similar clinic on hold with the intention to restart. During the study (Mar 2022 to May 2024), 3 additional sites (an additional 23%), implemented new multidisciplinary specialty clinics, and one of the existing multidisciplinary clinics added a new SCD-specific clinic. One hundred fifteen IWU with SCD have participated in these new clinics to date. Most of these clinics serve adolescents and young adults while only one clinic serves all ages of IWU. Multidisciplinary care so far includes combinations of maternal-fetal medicine, social work, psychology, genetic counselors, physical therapy, and nutrition in addition to hematology and gynecology/adolescent medicine.

Conclusion: Sickle cell pain around menstruation is a prevalent issue unique to individuals with a uterus (IWU) with SCD. Our study highlights a gap in the awareness and utilization of hormonal contraception among IWU with SCD, despite the overwhelming report of menstrual-associated SCD pain. Menstrual bleeding assessments during routine sickle cell visits can be implemented to open a dialogue between patients and their sickle cell providers and facilitate multidisciplinary reproductive health care for our patients. Multidisciplinary reproductive clinics tailored for IWU with SCD may be helpful in improving health outcomes and need to be studied longitudinally.