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