Family planning needs of young adults with sickle cell disease

Abstract Sexual and reproductive healthcare standards for adolescents and young adults with sickle cell disease (SCD) are not established. A total of 50 young adults entering adult SCD care completed a Family Planning Survey assessing sexual and reproductive health needs from March 2019 to July 2020. Clinical data were abstracted from respondents’ electronic medical records. Linear and logistic regression was applied to explore associations between clinical characteristics and survey results. Few respondents (8%) wished to be pregnant in the coming year, and 46% answered yes to at least one of four needs assessment questions. Those who were not employed full time were more likely to endorse needing help with getting sickle cell trait testing for a partner (ORadj = 9.59, p‐value = 0.05). Contraceptive use was associated with having an obstetrician–gynecologist (OR = 6.8, p‐value = 0.01). Young adults with SCD entering adult care have diverse reproductive health needs, highlighting opportunities to provide multidisciplinary, SCD‐specific reproductive healthcare.

F I G U R E 1 Young adults with sickle cell disease family planning survey responses. The Family Planning Survey gathered information on respondents' sexual and reproductive health history, awareness, and intentions. Additionally, it assessed the extent of four areas of potential healthcare need. Per-question response rates varied between 100% (Questions 1 and 4) and 48% (Question 9). people in pediatric care are automatically transitioned to adult care [11]. SCD treatments might positively affect reproductive health by reducing priapism, decreasing painful crises with menstruation, improving overall organ function to improve pregnancy outcomes, or by enhancing overall well-being, reducing social isolation and thus enabling the development of supportive relationships [12,13].
Treatments and cures may also raise reproductive health concerns, including infertility and teratogenicity [12,14]. AYA with SCD in pediatric care want-and lack-reproductive health knowledge and care [15,16].
Approaches to systematically address these complex and wideranging SCD-specific sexual and reproductive health concerns for AYA in the pediatric and adult care systems are emerging. Recently, the American Society of Hematology's SCD Transition Summary was updated to include prompts about SCD-related sexual and reproductive health concerns [17], and an empirically designed pediatric counseling tool addressing gonadal health now exists [1]. Standardized approaches, research, and clinical resources to provide this multidisciplinary care continues to evolve.
In 2019, we established a Young Adult Clinic at our SCD Center for Adults to provide subspecialty, developmentally appropriate SCD care to AYA integrating into the adult healthcare system. Patients' initial clinic visit includes a reproductive health assessment performed using our Family Planning Survey ( Figure 1) and with physician-led, standard interview questions. The purpose of this study was to describe survey results from the first 50 respondents. We hypothesized that using a survey to assess the sexual and reproductive health needs of AYA with SCD upon entry to adult care would reveal unmet reproductive healthcare needs among this population.

METHODS
This study was deemed exempt from formal review by the Institutional Review Board of a large northeastern academic hospital. We surveyed AYA (ages [18][19][20][21][22][23][24][25][26][27][28][29][30] with SCD when they established care at the Young Adult Clinic between March 1, 2019, and July 15, 2020. The Family Planning Survey ( Figure 1) consists of 10 yes/no questions written at a Flesch-Kincaid Grade Level of 6.0. Six survey questions addressed respondents' sexual and reproductive history, and four addressed respondents' sexual healthcare needs. The needs assessment includes "One Key Question," a validated screening tool to assess pregnancy intention in the next year [14]. Additionally, we conducted chart reviews documenting participants' clinical, socioeconomic, and reproductive health characteristics.
The surveys were either self-administered on paper during patients' first visit or, starting at the outset of the COVID-19 pandemic, electronically via a REDCap questionnaire sent to patients' email addresses. Those who returned a Survey with five or more questions answered were considered "respondents," and all others were considered "non-respondents." Because the survey was implemented as a clinical care standard, no incentives were offered for participation.
Data were analyzed using Stata/IC 16.1 [15]. Descriptive statistics summarizing survey responses were compared by patient characteristics. Mann-Whitney U-test compared respondents and nonrespondents by age, sex, and primary insurer. Fisher's exact tests and exact logistic regression models were fit to explore potential associations between patients' survey responses and sociodemographic variables.
Statistical significance was defined as p ≤ 0.05; no corrections were made for multiple hypothesis testing.

RESULTS
Seventy patients established care during the study period. Fifty completed surveys were included in this analysis. Respondents (N = 50) and nonrespondents (N = 20) did not differ by age, sex, or primary insurer (data not shown).  Among men who reported current contraceptive use (N = 8), most (N = 6/8) reported using condoms, and two reported that their sexual partner used another contraceptive method.

Survey results
Survey responses are shown in Figure 1. Response rates varied as 8 of 10 questions were completed by most respondents (>90%), and 2 questions, about pregnancy interest in the next year and preimplantation genetic testing, had lower response rates (48%).
Forty-six percent of respondents (N = 23) answered "yes" to at least one of the four needs assessment questions, specifically help with seeking contraception information (N = 13), getting a partner tested for sickle cell trait (SCT) (N = 9), learning about ways to prevent SCD (N = 9), and accessing genetic counseling (N = 6). After controlling for sex, age, and primary insurer, respondents who were not employed full time were more likely to request assistance with SCT partner testing than those who were employed full time (OR adj = 9.59, p-value = 0.05).   [18,19]. Assessing these needs as AYA establishes themselves in adult care is feasible and identifies clinical care needs; a validate tool to ensure systematic implementation of is needed [20].
Sexually active young people with SCD lack disease-specific counseling interventions for considering pregnancy and for preventing pregnancy. Most subjects in this study used disease-modifying therapy, and 52% of young women took hydroxyurea. Almost a decade ago, the NHLBI identified that contraception should be offered alongside hydroxyurea therapy for people with ovaries and SCD [21]; whether this guideline is widely implemented is unclear. The mismatch between pregnancy intention and contraceptive use among sexually active TA B L E 1 Sickle cell disease young adult family planning survey respondent demographic and reproductive health characteristics Abbreviations: IQR, interquartile range; SCD, sickle cell disease; SS, genotype SS; S 0 , genotype sickle beta zero thalassemia. a Sex-specific SCD complications: Priapism for males; Sickle cell pain associated with menstruation for females.

F I G U R E 2
Contraception and disease-modifying therapy use among sexually active and inactive young adults and adolescents living with sickle cell disease (SCD). Hydroxyurea use is an indication for contraceptive counseling in people with SCD. The reasons for not using contraception among people with SCD need study.
respondents in this survey is common to studies of contraception and unintended pregnancy in people with SCD in the United States [22,23].
Here, respondents engaged in sexual activity at rates roughly on-par with their age-peers in the general US population [24,25]. In diverse populations both with and without chronic conditions, "anticipatory guidance" in the form of individualized counseling addressing pregnancy intention and life goals improves the uptake of highly reliable contraception [19,26]. Hill). Gynecology care is integrated into bleeding disorder clinics, but gynecology is not yet routinely integrated into comprehensive SCD care [27]. This clinic redesign, supported by recent recommendations for building comprehensive SCD centers, removes a structural barrier to contraception use in SCD [28].
Other emerging markers of high-quality SCD care are suggested here. No respondent transitioning from a high-quality pediatric SCD center used an estrogen-containing form of contraception. This raises the possibility that AYA cared for at SCD centers are told, or their contraceptive prescribers elect, to give preference to progesterone-only contraceptive methods over estrogen-containing methods in women and girls with SCD. This may be due to concerns for exacerbating SCD-associated thrombotic risks [6]. In this cohort, 33% of female participants had a history of thrombosis, and the respondent with a provoked thrombotic event attributed to combined hormonal contraception was not cared for at a comprehensive pediatric SCD center.
Possibly, there is a practice difference in contraception prescribing between SCD care received at high-quality, comprehensive SCD centers compared to care outside of such centers, at least in the geographic region studied.
The sexual and reproductive healthcare needs of boys and men with SCD are often overlooked despite significant risks for erectile dysfunction, infertility, and associated mood disorders [3,4,29]. Indeed, almost half of male survey respondents reported priapism. The overrepresentation of young men with priapism in this sample raises the possibility of selection bias, but also that priapism is a protective factor for successfully transitioning to adult care. Delivering sex-specific education and care to boys and men with SCD is critical, and these long-standing issues are now well over 50-year old [30]; a recent study identifies ongoing needs and challenges integrating sexual and reproductive healthcare into pediatric SCD care [31,32]. Other nuances in providing this care need consideration. Fewer men than women responded to this survey, and most were privately insured (11/17) and graduated from high school or college (15/17). Future research must also include those who are less well educated and/or publicly insured.
In the United States, most people with SCD are African American; thus, there are intersectional care needs for boys and men with SCD, and leveraging the expertise of researchers and clinicians focused on Black boys and men may be important [33].
This study's limitations include its cross-sectional design, small sample size, and restriction to AYA with SCD who successfully transition to adult SCD care. This patient population is distinguished by prior and likely future access to expert disease-specific medical care that is not universally available [34]. Our survey's findings may not extend to this Nordisk.

CONFLICT OF INTEREST STATEMENT
The authors report no conflict of interests in the design, conduct, analysis, or presentation of this research. We disclose the following

DATA AVAILABILITY STATEMENT
Author elects not to share data.

ETHICS STATEMENT
We received ethics approval from the Johns Hopkins Institutional Review Board.