Conclusion
SCD providers consider SRH important for their female patients and largely believe these conversations should be standardized in their clinics. However, the range of conversations and contraceptive recommendations from SCD providers is broad, suggesting that this care can be improved. 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.4-7 Unintended pregnancy rates are particularly high in AYA with SCD, with up to 56% of women with SCD having at least one pregnancy by age 21.8-10 Pregnancy in SCD, whether planned or unplanned, bears a high risk of maternal and fetal morbidity and mortality.11 Thrombogenicity during pregnancy and iatrogenic pregnancy complications from medications prescribed for SCD are an additional unique concern for AYA women with SCD that require well-timed and managed pregnancies, including the use of contraception pre- and post-pregnancy.12
Providers of SCD often face challenges in advising patients about many aspects of SRH and family planning, but in particular about contraception. Hematologists must weigh the risk of unintended pregnancy with the risks of contraception. Oral contraceptive pills (OCPs) are the most commonly used hormonal method in sexually-active AYA in the US.13 Guidance for estrogen-containing combined hormonal contraceptives (CHC) use in SCD by the Centers for Disease Control and Prevention (CDC) and the World Health Organization (WHO) in the medical eligibility criteria (MEC) for contraceptive use indicates “advantages generally outweigh theoretical or proven risks,” and many women with SCD report prior use of CHC.11,14 However, theoretical risk may lead providers to advise against CHC use because of the increased separate risk of thrombotic events both in SCD and from estrogen.9,15,16 There is some data that depo-medroxyprogesterone acetate (DMPA, injectable contraception) has potential benefits for women with SCD, including reduced sickling and pain crises.17 However, loss of bone density with prolonged use is a concern for a population who may already be a risk for osteopenia.18 These examples demonstrate the complexity of prescribing contraception within this chronic disease population.
Therefore, education on family planning and SRH becomes an essential part of healthcare delivery for patients with SCD.3,12,19 Frequent clinic visits with SCD providers often lead to AYA with SCD to identify their subspecialist as their “main physician”.20,21 In this scenario, SRH counseling may occur due to SCD providers’ experience and training, the frequency of clinic visits, and potential lack of primary care providers (PCPs) within the population.21 SRH concerns such as menstruation, planning for pregnancy, contraceptive choice, and teratogenic medication use may require specific knowledge from the SCD provider. However, SCD providers’ views, attitudes, practices and consensus around SRH have not been well-studied.
This study evaluates SCD providers’ attitudes, preferences and practices regarding SRH for AYA women with SCD. We hypothesized that they recognize the importance of SRH discussions, but report infrequently discussing SRH. We also hypothesized that providers feel uncomfortable with prescription of CHC in SCD, but accept CHCs if alternative options are not available or preferred. Understanding barriers and preferences of providers of SCD can help inform future studies and interventions for education and healthcare provision to improve this aspect of care for AYA women with SCD.
METHODS
We distributed a survey to SCD providers, including physicians (MDs, DOs) and advanced practice providers, investigating their attitudes and practices regarding SRH in SCD. The survey was adapted from a study for providers of women with cystic fibrosis and piloted among hematologist colleagues.22 The survey included three sections: importance and priority of SRH topics for this population, practices around SRH discussions, attitudes toward SRH care provision, attitudes towards menstrual suppression and contraceptive use, barriers and facilitators to SRH care in the population, and demographic information, including Likert-style and multiple-choice questions (Appendix 1).
We distributed the survey via electronic mail to the 231 members of the hemoglobinopathy special interest group listserv of the American Society of Pediatric Hematology and Oncology (ASPHO) in May of 2019. An additional distribution was sent via electronic mail to 121 program directors of adult hematology and oncology fellowship programs with the request to pass to colleagues in February and April of 2020. This study was approved by Indiana University School of Medicine’s institutional review board #1906487088.
Research Electronic Data Capture (REDCap) tools hosted at the Indiana Clinical and Translational Sciences Institute were used to collect and manage study data.23 REDCap is a secure, web-based application designed to support data capture for research studies, providing 1) an intuitive interface for validated data entry; 2) audit trails for tracking data manipulation and export procedures; 3) automated export procedures for seamless data downloads to common statistical packages; and 4) procedures for importing data from external sources.
For this paper we focused specifically on providers’ attitudes and practices about SRH topics. Descriptive statistics were used to measure respondent demographics, characteristics, attitudes, and practices towards SRH in AYA with SCD. Responses for recommended contraception, acceptability of CHC as well as other methods, were compared in adult versus pediatric providers using Chi-squared, Fisher exact tests and independent sample t-tests. For this analysis, retired and combined program directors (n=2) were not included. Likert-scale ratings were measured from 1 (not at all important) to 5 (very important). Mann-Whitney tests were used to compare ordinal data between groups of pediatricians and adult providers. Missing data was excluded from analysis and ranged from 0 to 3 for various survey items. All analyses were conducted using IBM SPSS version 26.
RESULTS