Provider Attitudes, Preferences, and Practices Regarding Sexual and Reproductive Health for Adolescents and Young Adults with Sickle Cell Disease

Abstract

Purpose

With improvements in life expectancy, adolescents and young adults (AYA) with sickle cell disease (SCD) increasingly face sexual and reproductive health (SRH) concerns. As subspecialists often serve as primary care providers for those with chronic disease, this study examines pediatric SCD providers’ practices and attitudes related to SRH of AYA women with SCD.

Methods

We developed an adapted survey to identify SCD provider attitudes and practices in addressing menses, sexual activity, contraception, and pregnancy for their female patients. We electronically distributed this survey to the American Society of Pediatric Hematology/Oncology SCD interest group. We used descriptive statistics to analyze results.

Results

Seventy-eight pediatric SCD providers completed the survey. A majority (95%) rated SRH discussions as moderately important or higher, with 89% agreeing this care should be standardized. Most respondents reported discussing SRH, such as menses (78%), teratogenic medications (61%), and contraception (90%), with their female patients with SCD at least annually. Although most refer AYA with SCD for birth control (83%), 39% endorsed a preferred method, with 33% of these favoring levonorgestrel intrauterine devices in this population and 40% injectable contraception. Approximately half of respondents (57%) reported that the use of combined hormonal contraceptives was unacceptable despite published guidelines that support potential benefits outweighing theoretical risk in AYA with SCD.

Conclusions

The range of SRH conversations and contraceptive recommendations for AYA from pediatric SCD providers is broad. SCD providers and AYA with SCD would benefit from improved evidence and educational resources related to contraception as well as coordinated SRH counseling.

Advances in therapy and management in the past decade have enabled adolescent and young adults (AYA) with sickle cell disease (SCD) to live longer. However, this population continues to face morbidity and mortality challenges as they reach adulthood and their reproductive years.^1,2 Health disparities related to systemic healthcare and socioecological factors have contributed to limited life expectancy and quality of life in SCD.^3,4 The average life expectancy of SCD patients has increased to the mid-40s to 50s, and many AYA with SCD have expectations for their sexual and reproductive health (SRH) that are similar to their non-affected peers.^5–7 Unfortunately, AYA women in the United States (US) ages 15–24 years experience the highest rates of sexually transmitted infections and unintended pregnancy.^8–11 In addition, pregnancy in SCD, whether planned or unplanned, bears a high risk of maternal and fetal morbidity and mortality, and the transmission to offspring of women with SCD must also be taken into account.¹² 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.¹³

Providers who care for individuals with 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. Contraception may provide other benefits beyond preventing pregnancy. Menses has been associated with vaso-occlusive crises and decreased quality of life in women with SCD, yet consideration of risks versus benefits to hormonal intervention must be weighed.^14,15 Oral contraceptive pills are the most commonly used hormonal method in sexually active AYA in the US.¹⁶ In contrast to conditions such as recurrent deep venous thrombosis, for which the Centers for Disease Control and Prevention (CDC) recommends estrogen-containing combined hormonal contraceptives (CHC) as an “unacceptable health risk,” guidance for CHC use in SCD by the 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.^12,17 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, though the data is unclear for AYA with SCD without history of thrombotic events.^18–20 There is some data that depo-medroxyprogesterone acetate (DMPA, injectable contraception) has potential benefits for women with SCD, including reduced sickling and pain crises.²¹ However, loss of bone density with prolonged use is a concern for a population who may already be at risk for osteopenia.²² These examples demonstrate the complexity of prescribing contraception within this chronic disease population.^6,13,23 The contraceptive prescribing patterns of providers of AYA with SCD have yet to be assessed.

Frequent clinic visits with SCD providers often lead to AYA with SCD to identify their subspecialist as their “main physician”.^24,25 In this scenario, SRH counseling may occur due to SCD providers’ experience and training, the frequency of clinic visits, and longstanding relationship with the provider.²⁵ 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’ perspectives and preferences around SRH care delivery to this population have not been well-studied.

This study evaluates pediatric 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 would report discomfort 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 physicians and advanced practice providers investigating their attitudes and practices regarding SRH in AYA with SCD. The survey was adapted from a study for providers of women with cystic fibrosis and piloted among 5 hematologist colleagues.²⁶ The survey included Likert-style and multiple choice questions regarding 1) importance and priority of SRH topics for this population; 2) practices around SRH discussions; 3) attitudes toward SRH care provision, menstrual suppression, and contraceptive use; 4) barriers and facilitators to SRH care in the population; and 5) demographics; and included areas for free-text of thoughts and comments (see Supplemental Data, Survey Questionnaire).

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. This study was approved by Indiana University School of Medicine’s institutional review board #1906487088.

Study data were collected and managed using REDCap electronic data capture tools hosted at the Indiana Clinical and Translational Sciences Institute (Indiana CTSI) funded, in part by Grant Numbers UL1TR001108, KL2TR001106, or TL1TR001107 from the National Institutes of Health, National Center for Advancing Translational Sciences, Clinical and Translational Sciences Award and at the Indiana University Pervasive Technology Institute (https://pti.iu.edu/) which supports REDCap with IT infrastructure and consulting resources.

REDCap (Research Electronic Data Capture) 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.²⁷

We utilized descriptive statistics to measure respondent demographics, characteristics, attitudes, practices, barriers, and facilitators towards SRH in AYA with SCD. We measured Likert-scale ratings from 1 (not at all important) to 5 (very important) and, for questions around attitudes and practices; we combined Likert ratings 1–2 and 4–5. We excluded missing data from analysis, which ranged from n of 0 to 3 for various survey items. We compared a subgroup analysis of years out from training, region of practice, and program size for selected primary outcomes using Chi-squared analysis. We conducted all analyses using IBM SPSS version 26.

RESULTS

Demographics

A total of 78 pediatric providers (78%, n=61 female) completed the survey. Thirty-four percent of ASPHO members (78/231) responded to the survey based on currently available membership numbers, and our sample mirrored currently available member characteristics (73% female). Almost a quarter of participants (23%, n=18) reported they were pediatric SCD program directors and one reported being a combined pediatric and adult SCD program director (Table 1).

Table 1.

Demographics.

Health Care Provider Demographics	Number (%)
Degree (n=78)
MD/DO	73 (94)
Advanced Practice Providers (NP/PA)	4 (5)
Clinical Nurse Specialist	1 (1)
Age [mean (SD)] (n=74)	41 (8)
Gender (n=78)
Female	61 (78)
Male	17 (22)
Years since Fellowship Completed (n=77)
Still in training	9 (12)
1–5	24 (31)
6–10	18 (23)
11–15	12 (16)
16–20	8 (10)
21–25	3 (4)
Over 25 years	3 (4)
Region (n=76)
Midwest	25 (33)
Northeast	18 (23)
Southeast	13 (17)
Mid-Atlantic	8 (10)
Southwest	7 (9)
West	6 (8)
Program Size (n=75)
50–150 patients	27 (36)
150–250 patients	13 (17)
250–350 patients	9 (12)
350–450 patients	9 (12)
Over 450 patients	17 (23)
SCD Teen or Transition Clinic (n=77)	37 (47)

Attitudes toward SRH Discussions with Female Patients with SCD

Ninety-five percent 95% (n=74) of respondents rated the importance of SRH discussions as somewhat or very important for AYA women with SCD and 5% (n=4) rated them as neutral. No respondents rated the importance of SRH discussions as unimportant. Over 88% of respondents (n=69) reported that SRH should be standardized for SCD patients in US care centers. Nearly half of respondents (44%, n=34) responded that the SCD care provider/team should have the primary role in discussing SRH with AYA with SCD; 28% (n=22) responded that the primary care provider (PCP) had this responsibility, 19% (n=15) responded Adolescent Medicine, and 9% (n=7) responded obstetrician/gynecologist. One respondent commented, “It is very important that we as SCD docs are open to talking about this with our teens - we see them MUCH more often than the PCP.”

Regarding the ideal age to discuss SRH topics with women with SCD, 35% (n=27) reported under age 13 years, 51% (n=40) between age 13 and 15 years, and 6% (n=5) between age 16 and 18 years. No respondents reported discussions should occur after age 18 years, and 1 respondent reported that SRH topics should not be discussed.

Nearly two-thirds of respondents (65%, n=51) answered that parents or guardians should be present during discussions about menses. Approximately one-third answered that they should be present during discussions around teratogenic medication use and future pregnancy (40%, n=31 and 36%, n=28, respectively). One of five respondents (22%, n=17) answered that parents or guardians should be present during contraceptive discussions. Only 1 respondent answered that parental presence was advised during discussions around sexual activity.

SRH Care Discussions in the SCD Care Setting

Most respondents (79%, n=62) answered that they discuss menses with AYA women with SCD. When asked about frequency of discussions about menstruation, 22% (n=17) reported never or rarely, 15% (n=12) reported annually, 63% (n=49) reported multiple times per year or every visit (see Table 2).

Table 2.

Reported frequency of Sexual and Reproductive Health (SRH) discussions.

Discussion Topic	Never/Rarely	Annually	Multiple Times Per Year or Every Visit
Menstruation (n=78)	22% (17)	15% (12)	63% (49)
Pregnancy (n=75)	51% (38)	36% (28)	12% (9)
Teratogenic Medications (n=77)	38% (30)	30% (23)	31% (24)
Contraception (n=77)	9.0% (7)	40% (31)	50% (39)

Nearly 80% of respondents (n=62) also reported discussing pregnancy with their AYA female patients. When asked about frequency of discussions about planning for pregnancy in the setting of SCD, 49% (n=38) reported discussing this never or rarely, 36% (n=28) annually, 12% (n=9) multiple times per year or every visit, and 3 did not respond.

Sixty-nine percent (n=54) reported that they discuss teratogenic medications with their patients. When asked about frequency of discussions about teratogenic medications, 38% (n=30) reported never or rarely discussing teratogenic medications, 30% (n=23) reported annual discussions, and 31% (n=24) reported multiple times per year or every visit; one did not respond.

Most respondents (92%, n=72) reported discussing contraception with their patients. When asked about frequency of discussions about contraception, 9% (n=7) reported never or rarely, 40% (n=31) reported annually, 50% (n=39) reported multiple times per year or every visit; one did not respond.

Contraceptive and Pregnancy Attitudes and Practices

Eighty-three percent (n=65) of respondents reported they refer patients to other providers for counseling and management of contraception, 13% (n=10) reported they recommend birth control to selected patients and prescribe it themselves, and 2 participants reported they recommend birth control to no one. One provider commented, “Most gynecologists have extremely poor knowledge of SCD both in contraception management with refusal to provide contraception, and in pregnancy management.”

Two-thirds of respondents (68%, n=53) reported recommending and referring for menstrual suppression, 17% (n=13) reported that they do not recommend menstrual suppression, and 14% (n=11) prescribe menstrual suppression to selected patients. Comments related to use of menstrual suppression included “Risks outweigh benefits,” and “Not enough information about benefits.” Over a third (35%, n=27) reported a preferred method for menstrual suppression, 18% (n=14) reported preferring DMPA, 10% (n=8) reported the levonorgestrel IUD, 3% (n=2) preferred progestin-only pills, 3% (n=2) preferred oral CHCs, and one preferred the contraceptive implant.

Over a third of respondents (39%, n=30) reported a preferred method of contraception (pregnancy prevention) for AYA female patients with SCD. Of this subset, 33% (n=10) preferred the levonorgestrel IUD, 40% (n=12) preferred contraceptive injection, 13% (n=4) preferred progestin-only pills, 7% (n=2) preferred the contraceptive implant, one preferred the copper IUD, and one chose oral CHCs. Over half of respondents did not deem CHC acceptable for birth control (58%, n=45) or for menstrual suppression (68%, n=53) in this population.

Regarding attitudes around CHC and pregnancy in SCD, 30% (n=23) agreed or strongly agreed that CHC should never be used in AYA with SCD (Table 2). Almost half agreed that CHC can be used if no other risk factors are present. The majority (87%, n=68) believe that pregnancy is high risk in any woman with SCD (Table 3).

Table 3.

Attitudes around combined hormonal contraception (CHC) and pregnancy in SCD.

N (%)	Strongly disagree	Disagree	Neither agree nor disagree	Agree	Strongly agree
CHC should never be used in AYA with SCD (n=77)	7 (9)	29 (38)	23 (30)	14 (18)	4 (5)
CHC can be used if no other risk factors (n=76)	3 (4)	13 (17)	22 (29)	35 (47)	3 (4)
Pregnancy is high risk in any woman with SCD (n=78)	1 (1)	3 (4)	6 (8)	32 (41)	36 (46)

CHC=Combined hormonal contraception. SCD=Sickle cell disease. AYA=Adolescents and young adults.

Subgroup Analysis

We completed a Chi-squared subgroup analysis related to the importance of SRH discussions, frequency discussions of teratogenic medications, menstrual suppression, referral and prescription of contraception, preferred contraception, and acceptability of CHC. Small programs (50–150 patients) and very large programs (>450 patients) were more likely to endorse CHC as acceptable in patients with SCD (67% (18/27) and 59% (n=10/17), respectively, vs. under half for mid-sized programs (p<.05)). No other differences were found for the aforementioned outcomes between years out from training, region of practice, or program size.

Barriers and Facilitators to SRH Care in SCD

When asked about what limits SRH discussions with their AYA female patients, 74% (n=58) of respondents cited lack of time, 50% (n=39) presence of parents in the room, 50% (n=39) cited lack of SRH knowledge, 28% (n=22) patient discomfort, and 17% (n=13) lack of rapport. One respondent commented, “This is an area that needs more emphasis and we need better training.”

When asked about facilitators to SRH discussions with their AYA female patients, 81% (n=63) endorsed training curricula or modules, 69% (n=54) guidelines from the Foundation for SCD Research (FSCDR), and 37% (n=29) small group discussions at the SCD Association of America (SCDAA) conference. Seventy-two percent (n=56) of respondents believed a standardized partnership with SRH specialists would improve this aspect of care. Related to the type of educational resources, 83% preferred online resources, 76% pamphlets or books, 72% a mobile device application with information and resources, and 40% video-based educational modules.

DISCUSSION

This survey explored the attitudes, preferences, and practices of pediatric SCD providers related to SRH of their AYA female patients. The majority of respondents agreed that SRH is an important topic for discussion with AYA women with SCD, the SCD provider should be the ones discussing SRH with their patients, and such discussions should be standard practice within pediatric SCD clinics. There were discrepancies as to the timing, content, and approach of such discussions with this population. The majority of respondents agreed that SRH discussions should happen after menarche in early adolescence. There was wide variation in terms of frequency of discussion of contraception, pregnancy, and teratogenic medications. There was also a wide variety in providers’ comfort level with use of estrogen-containing CHC, with most opting for progestin-only methods.

Patients, especially AYA, with chronic diseases including SCD, cystic fibrosis, and epilepsy depend on their providers for their SRH knowledge and access.^13,26,28,29 Ownership of SRH discussion is often lacking when a PCP is seen infrequently. Respondents felt ownership of these discussion with comments supporting ownership of SRH discussions. This supports the fact that for many with SCD, primary care may not be an available option for discussing sexual health when needed. Subspecialists often discuss issues of interest or those more medically specific to the chronic illness with the parents. Studies of providers of patients with other chronic illnesses report private discussions regarding SRH are too time-consuming or uncomfortable for the provider.^26,28,29 However, this survey indicates that subspecialists are poised to discuss SRH with their patients. Subspecialists are often the providers prescribing teratogenic medications, and most are already having some discussion around SRH. In this survey, the majority of providers reported more frequent discussions around contraception, and less frequent discussions around pregnancy. The differences in frequencies between topics is unclear; perhaps providers feel contraception is more urgent than menstrual dysfunction, pregnancy, and teratogenicity for an AYA population. Providers also commented that they would like more training, suggesting they may feel uncomfortable managing pregnancy in SCD patients. Optimizing comprehensive SRH care discussions in subspecialty care may require a larger shift to incentivize or standardize time alone with the AYA to address important concerns.

Our results demonstrate a wide discrepancy in SCD provider attitudes toward forms of contraception that are preferred and acceptable for use by AYA women. We found that providers we surveyed prefer DMPA over other methods, which is supported in the literature.^12,18,21,30 There is less evidence for use of levonorgestrel IUDs, also endorsed by many participants as a preferred option, likely due to its associated benefits of lighter menses and high efficacy without estrogen.³¹ The respondent preference for the copper IUD, which has the adverse effect of worsening dysmenorrhea and menorrhagia, likely prioritizes the avoidance of the reported risks of estrogen.³²

Current CDC recommendations, as well as the 2018 Sickle Cell Guidelines from the United Kingdom, state that CHC use with SCD is a “condition for which the advantages of using the method generally outweigh the theoretical or proven risks.”^33,34 The 2014 National Heart, Lung, and Blood Institute Guidelines reference the CDC but add that CHC data were insufficient and recognize the increased risks in this population of other contraindications such as stroke.³⁵ As such recommendations are coupled with anecdotal provider reluctance for CHC use, we asked the questions around acceptability of CHC to better understand provider recommendations and beliefs. Our findings demonstrated a fairly split distribution with about half of SCD providers responding that CHC are unacceptable in any AYA with SCD and half considering them acceptable without other contraindications. Acceptability was higher in small and large program sizes compared to mid-sized programs; it is unclear if this is due to systematic reasons. When recommending restrictions, the CDC and the WHO must balance the risk of pregnancy against the risk of CHC in SCD. This balance is challenging when there is limited data, as is the case with SCD and CHC.^17,34 Likewise it is crucial to provide contraception options that meet the desires of the patient. As CHC can suppress menses, be generally weight neutral, and provide relief for dysmenorrhea in addition to preventing pregnancy, this method may meet many patient needs. The responses by providers in this study support the need for further research in this area so that providers can make scientifically informed recommendations.

Similar to providers, women with SCD also lack clarity in contraceptive choice. While there is no one-size-fits-all approach to contraception in any population, SCD patients lack evidence-based guidance for educated, guided discussions. Finding reliable SRH information is a challenge for any AYA, and AYA women with SCD have particular gaps in knowledge in contraception, puberty, fertility, and pregnancy-related health outcomes.³⁶ Standardizing or incentivizing visits alone with AYA, such as in teen or transition clinics, can help facilitate SRH discussions and, thus, improve patient knowledge.

Strengths of this study include that it is a geographically diverse sample and novel in the SCD population. Limitations include selection bias, as providers who are interested in SRH may have been more likely to complete the survey. Though representative of the survey sample, a disproportionate number of respondents were female which may have led to gender bias. Additionally, the survey represented pediatric-trained physicians, and therefore, is not representative of all providers of SCD.

Providers and AYA with SCD would benefit from further evidence-based guidance regarding SRH, especially contraception. Future research in this area could track adverse events following different contraceptive choices in this population. Understanding these choices and patient preferences is essential for providers to adequately counsel patients and give recommendations. Additional data on the use of CHC and levonorgestrel IUDs, as well as all forms of contraception, in this population is also necessary to guide decision-making and prescribing practices. Specific evidence on the risk of contraception with VTE in patients with SCD and interaction with common medications used in SCD such as hydroxyurea is essential.

Second, these findings suggest the need for providers of SCD (with other thought leaders in SRH) to develop some type of consensus on how SRH can be addressed consistently with this population of patients. Respondents endorsed online educational modules and resources, as well as clinical partnerships with SRH providers, would improve their ability to provide SRH care for AYA with SCD. Some programs have already initiated these partnerships for other chronic diseases, and this study indicates a need for improvement and standardization.³⁷ The relationship between SCD providers and their AYA patients is a potential source of empowerment as SRH knowledge and care discussions can prepare AYA for transition to adulthood.^25,38 AYA specialists can serve as touch points and key referrals for SCD providers and patients when facing specific SRH decisions and care needs. This study may be able to help build future programs in education and interventions to improve SRH knowledge and access for patients with SCD.

Overall, this study supports that there is no standardization, wide variety, and clear barriers to SRH discussions for patients with SCD. There is a need for ongoing studies to examine the safety of various methods and training of providers to address reproductive health. Future research related to this study should include patient perspectives on SRH, and comparing their responses to provider practices. Implementation studies would help examine dissemination of existing SRH information to improve communication and SRH outcomes.

IMPLICATIONS AND CONTRIBUTION.

This survey study of providers of patients with sickle cell disease delineates the lack of research in sexual and reproductive health, the importance providers place on the topic, and the gap in provider recommendations. Specifically, results showed provider discomfort in prescription of combined hormonal contraception such as oral contraceptive pills.

Abbreviations Key:

ASPHO

American Society of Pediatric Hematology/Oncology

AYA

Adolescents and Young Adults

CDC

Centers for Disease Control and Prevention

CHC

Combined Hormonal Contraception

DMPA

Depo-medroxyprogesterone acetate

Heme/onc

Hematology/Oncology

IUC

Intrauterine device

MEC

Medical Eligibility Criteria

OCP

Oral Contraceptive Pill

PCP

Primary Care Provider

REDCap

Research Electronic Data Capture

SCD

Sickle Cell Disease

SRH

Sexual and Reproductive Health

US

United States

WHO

World Health Organization