Contraceptive Attitudes and Beliefs of Women With Sickle Cell Disease: A Qualitative Study.

OBJECTIVES: We aimed to qualitatively explore factors influencing contraceptive use in women living with sickle cell disease (SCD). STUDY DESIGN: We conducted a semi-structured qualitative study using data from interviews with reproductive-aged women with SCD. The Theory of Planned Behavior, which describes an individual's attitudes, subjective norms, and perceived behavioral control as drivers of a health behavior, served as a framework for data collection and thematic analysis. RESULTS: We analyzed interviews from 20 participants. Attitudes toward contraception showed skepticism regarding hormonal contraception and concern about adverse effects of contraception related to SCD. The role of subjective norms in contraceptive choice depended on whether participants possessed trusted sources of information, such as health care providers, family, and friends, or whether they relied on themselves alone in their contraceptive decision-making. The influence of health care providers was complicated by inconsistent and sometimes conflicting counseling. Finally, with regard to participants' perceived control in their contraceptive choices, some were motivated to alleviate menstrual pain or prevent SCD-related pregnancy risks, but many reported feeling disempowered in contraceptive decision-making settings because of their demographic characteristics or pressure from health care providers. CONCLUSIONS: Women living with SCD are influenced by many factors similar to those documented for other populations when making decisions about contraception. However, SCD amplifies both the importance and risks of contraception and may also complicate individuals' autonomy and contraceptive access. Hematology and reproductive health providers should recognize the risk of coercion, maximize patients' reproductive agency, and coordinate reproductive health counseling for patients with SCD.

Outcomes After Induction of Labor Compared With Dilation and Evacuation for the Management of Rupture of Membranes in the Second Trimester.

Previable and periviable rupture of membranes is associated with significant morbidity for the pregnant patient. For those who have a choice of options and undergo active management, it is not known how the risks of induction of labor compare with those for dilation and evacuation (D&E). We performed a retrospective cohort study of patients with rupture of membranes between 14 0/7 and 23 6/7 weeks of gestation who opted for active management. Adverse events (52.2% vs 16.9%, P <.01) and time to uterine evacuation greater than 24 hours (26.7% vs 9.6%, P =.01) were more common among patients undergoing induction of labor. In a multivariable regression, induction of labor was an independent risk factor for complications (odds ratio 5.70, 95% CI, 2.35-13.82) compared with D&E. Severe complications were rare across both groups (4.4% for patients undergoing induction vs 2.6% for D&E, P =.63). Given the differing risks by termination method, access to D&E is an important treatment option for this patient population.

Trust in provider and stigma during second-trimester abortion.

OBJECTIVE: To determine whether trust in the provider and sociodemographics are associated with individual-level abortion stigma. METHODS: We performed a cross sectional and exploratory study design using secondary analysis of a randomized trial that enrolled participants undergoing second trimester abortion. We collected baseline survey data from 70 trial participants to assess stigma (Individual Level of Abortion Stigma scale, ILAS; range 0-4), trust in provider (Trust in Physician scale; range 1-5), anxiety, depression, and sociodemographics. We performed multiple linear regression, for which ILAS score was the outcome of interest. Univariate associations were used to inform the regression model. RESULTS: The mean abortion stigma score was at the low end of the ILAS at 1.21 (range 0.2-2.8, SD 0.66). Age, race, income, BMI, parity, gestational age at time of abortion, and reasons for ending the pregnancy were not significantly associated with the ILAS score. Higher trust in provider scores were (m 4.0, SD 0.49) and inversely related to the ILAS score, even after adjustment for confounders (ß -0.02, CI -0.03 to -0.004, p = 0.013). Screening positive for anxiety or depression was associated with a higher ILAS score ((ß 0.48, CI 0.10, 0.90, p = 0.015); (ß = 0.27 CI -0.097, 0.643)), while cohabitation was associated with lower ILAS score (ß -0.44, CI -0.82 to -0.57, p = 0.025). CONCLUSIONS: Trust in an abortion provider, anxiety, depression, and cohabitation are associated with abortion stigma among people seeking second trimester abortion care. Interventions that improve trust in a provider may be an area of focus for addressing abortion stigma. Future research should confirm these findings in larger populations and across diverse locations and demographics and to conduct qualitative research to understand what patients perceive as trust-promoting behaviors and words during abortion encounters.

Pregnancy-related thromboembolism in women with sickle cell disease: An analysis of National Medicaid Data.

Pregnancy and sickle cell disease (SCD) both individually carry a risk of thromboembolism (TE). Pregnancy in people with SCD may further enhance the prothrombotic effect of the underlying disease. The objectives of this study were to determine the rate and risk factors for arterial and venous thrombosis in pregnant people with SCD. Administrative claims data from the United States Centers for Medicare and Medicaid Service Analytic eXtract from 2006 to 2018 were used. The study population included people with SCD from the start of their first identified pregnancy until 1 year postpartum and a control cohort of pregnant people without SCD of similar age and race. Outcomes of interest were identified with ICD-9 or 10 codes. Logistic regression analyses were used to analyze risk factors. We identified infant deliveries in 6388 unique people with SCD and 17 110 controls. A total of 720 venous thromboembolism (11.3%) and 335 arterial TE (5.2%) were observed in people with SCD compared to 202 (1.2%) and 95 (0.6%) in controls. People with SCD had an 8-11 times higher odds of TE compared to controls (p < .001). Within the SCD cohort, age, hemoglobin SS (HbSS) genotype, hypertension, and history of thrombosis were identified as independent risk factors for pregnancy-related TE. Pregnancy-specific factors (pre-eclampsia, eclampsia, multigestational pregnancy) were not associated with TE. In conclusion, the risk of pregnancy-related TE is considerably higher in people with SCD compared with controls without SCD. Hence, people with SCD, particularly those with multiple risk factors may be candidates for thromboprophylaxis during pregnancy and the postpartum period.

Comparison of thromboembolism outcomes in patients with sickle cell disease prescribed hormonal contraception.

Patients with sickle cell disease (SCD) are at a risk of thromboembolism (TE), and use of hormonal contraception can further increase that risk. This study aims to assess patterns of hormonal contraceptive use and compare risk of contraception-related TE between combined hormonal contraceptives (CHCs) and progestin-only contraceptives (POCs). Patients with SCD aged between 12 and 44 years with a new prescription of a hormonal contraceptive in the Centers for Medicare and Medicaid Services Medicaid Analytic eXtract database (2006-2018) were followed up to 1 year. We identified 7173 new users: 44.6% initiated CHC and 55.4% initiated POC. Combined oral contraceptive pills (OCPs; 36.5%) and progestin-only depot medroxyprogesterone acetate (33.9%) were the most frequently prescribed agents. A total of 1.8% of contraception users had a new diagnosis of TE within 1 year of the first identified contraception prescription. There were no significant differences in TE event rates between CHC and POC users (17.2 and 24.7 events per 1000 person-years, respectively). In patients prescribed OCP, there were no differences in TE event rates based on estrogen dose or progestin generation. Transdermal patch had a 2.4-fold increased risk of TE as compared with that of OCP. Although limited by the retrospective study design and use of administrative claims data, this study found no significant differences in TE rates between new users of CHC and POC in patients with SCD. Careful evaluation of underlying TE risk factors should be considered for each patient with SCD before initiation of hormonal contraception.

Feasibility and acceptability of virtual group contraceptive counseling prior to abortion care.

OBJECTIVES: This study aimed to evaluate the feasibility and acceptability of virtual group contraceptive counseling in the abortion care setting. STUDY DESIGN: Patients seeking abortion care at an urban university hospital were invited to participate in this cohort study. Prior to their clinical appointments, groups of two to six patients participated in 45-minute virtual contraceptive counseling visits facilitated by study staff. Contraceptive method use, effectiveness, and side effects were reviewed according to group interest. Participant satisfaction scores were collected immediately following the sessions. After their appointments, providers estimated the time spent on contraceptive counseling during the clinical visit. RESULTS: Of 195 patients approached, 86 (44%) were enrolled. Fifty-seven (66%) enrolled patients completed a session. The most common reason for declining enrollment was concern about the time commitment. Most (93%) participants reported being "satisfied" or "very satisfied" overall, and 96% would recommend group contraceptive counseling to a friend. Providers reported that compared to typical counseling, participants required a shorter amount of time during the clinical visit than nonparticipants (time spent <5 minutes: 74% vs 54%). CONCLUSIONS: Virtual group contraceptive counseling for patients seeking abortion was feasible and acceptable in this study. Group virtual visits may reduce provider time burden, add value when virtual care delivery is desired or required, and deserve further study. IMPLICATIONS: Family planning clinics can consider incorporating virtual group counseling as a person-centered and efficient approach to contraceptive counseling at the time of abortion care.

Association between unplanned pregnancies and maternal exacerbations in cystic fibrosis.

INTRODUCTION: Following availability of the highly effective cystic fibrosis (CF) transmembrane conductance regulator modulator, elexacaftor/tezacaftor/ivacaftor, there was a near doubling of pregnancies reported in the United States (US) in people with CF. We sought to determine health impacts of planned (PP) versus unplanned pregnancies (UP). METHODS: We collected retrospective pregnancy data from January 2010-December 2020 from 11 US CF centers. After adjusting for potential confounding effects, we conducted multivariable, multilevel longitudinal regression analysis using mixed effect modeling to assess whether changes in percent predicted forced expiratory volume in one second (ppFEV1), body mass index (BMI), and pulmonary exacerbations (PEx) 1-year-pre- to 1-year-post-pregnancy were associated with pregnancy planning. RESULTS: Our analysis included 163 people with 226 pregnancies; the cohort had a mean age at conception of 29.6 years, mean pre-pregnancy ppFEV1 of 75.4 and BMI of 22.5 kg/m2. PpFEV1 declined in both PP (adjusted decline of -2.5 (95% CI: -3.8, -1.2)) and UP (adjusted decline of -3.0 (95% CI: -4.6, -1.4)) groups, they did not differ from each other (p = 0.625). We observed a difference in change in the annual number of PEx pre- to post-pregnancy (PP: 0.8 (0.7, 1.1); UP: 1.3 (1.0, 1.7); interaction effect p = 0.029). In a subset of people with available infant data, infants resulting from UP had more preterm births, lower APGAR scores, and more intensive care unit stays. CONCLUSIONS: Following UP, there is an increased trajectory for PEx and potentially for infant complications compared to PP. Clinicians should consider increased surveillance in the setting of UP.

Utility and Limitations of Human Chorionic Gonadotropin Levels for Remote Follow-up After Medical Management of Early Pregnancy Loss.

Early pregnancy loss can be treated medically with mifepristone followed by misoprostol, with ultrasonographic confirmation of pregnancy expulsion. Alternative strategies that ascertain treatment success remotely are needed. We compared percent decline in human chorionic gonadotropin (hCG) level with treatment success or failure between patients who received mifepristone pretreatment followed by misoprostol or misoprostol alone for early pregnancy loss between 5 and 12 weeks of gestation to determine a threshold decline that might predict success. Early pregnancy loss treatment success was associated with a greater percent hCG level decline compared with treatment failure, but no threshold was able to predict success. Additional research is needed to understand hCG trends after medical management of early pregnancy loss to develop reliable protocols for remote follow-up.

Sex as an Independent Risk Factor for Venous Thromboembolism in Sickle Cell Disease: A Cross-Sectional Study.

Venous thromboembolism (VTE) affects up to 25% of individuals with sickle cell disease (SCD), but risk factors are not well characterized. We sought to measure the prevalence of VTE among SCD patients in our health system and to describe the relationship between medical history, biological sex, and VTE. We performed a retrospective chart review of SCD patients who visited an outpatient hematology clinic within Penn Medicine between June 2014 and June 2019. Demographics and medical history were compared across those with and without a history of VTE. We developed a logistic regression model to describe factors independently associated with VTE. Of 597 patients with SCD who were identified, 147 (24.6%) had a history of VTE; 100 were female and 47 were male. In the regression model, female sex was independently associated with history of VTE (odds ratio 1.91, 95% confidence interval 1.26-2.91), as were pulmonary hypertension, hydroxyurea use, and history of stroke. Among females only, 49.7% were parous and 18.8% had used oral contraceptives, and these proportions did not differ by history of VTE. One-quarter of the SCD patients in our health system had a history of VTE, confirming significantly higher rates than in the general population. Females had twice the odds of VTE compared to males, highlighting an important sex disparity in SCD disease outcomes and raising questions regarding optimal pregnancy and contraceptive care for females with SCD.

Fetomaternal hemorrhage assessment in Rh-negative patients undergoing dilation and evacuation between 20 and 24 weeks' gestational age: A retrospective cohort study.

OBJECTIVE: To estimate the rate of requiring more than one 300-mcg Rh D immune globulin dose for fetomaternal hemorrhage (FMH) at the time of second-trimester dilation and evacuation (D + E). STUDY DESIGN: We performed a retrospective cohort analysis of patients at greater than 20 weeks' gestation who underwent D + E, had Rh D-negative blood type, and received FMH quantification testing. RESULTS: Of 25 eligible patients, 24 had negative quantification of FMH; one had positive quantification that did not meet the clinical threshold for additional dosing. CONCLUSIONS: The absolute risk of requiring additional Rh D immune globulin after D+E for pregnancies greater than 20 weeks' gestation was 0%.

Engaging Stakeholders in the Development of a Reproductive Goals Decision AID for Women with Cystic Fibrosis.

Objective: More people with cystic fibrosis (pwCF) are reaching adulthood and considering their reproductive futures. Unfortunately, many pwCF report gaps in their reproductive healthcare. We describe measures of stakeholder engagement in developing a reproductive goals decision aid for women with CF called MyVoice:CF. Methods: Stakeholders reviewed the content, design, and usability of the tool, which was informed by prior research related to CF family planning experiences and preferences as well as a conceptual understanding of reproductive decision making. We evaluated stakeholder engagement via process measures and outcomes of stakeholder involvement. We collected data via recorded stakeholder recommendations and surveys. Results: Fourteen stakeholders participated and the majority described their role on the project as "collaborator", "advisor", or "expert." Most felt their expectations for the project were met or exceeded, that they had contributed significantly, and that they received sufficient and frequent information about the process. All stakeholders provided recommen-dations and clarified aims for a CF-specific family planning tool, including its content and focus on facilitating shared decision making. Discussion: Utilizing meaningful stakeholder contributions, we developed MyVoice:CF, a novel web-based decision aid to help women with CF engage in shared decision-making regarding their reproductive goals. Practical Value: Our findings from working with stakeholders for MyVoice:CF indicate that disease-specific reproductive health resources can and should be designed with input from individuals in the relevant communities.

CF and male health: Sexual and reproductive health, hypogonadism, and fertility.

Over 30,000 people in the United States are diagnosed with cystic fibrosis (CF), and over 50% of those with CF are male. Men with CF face specific and sometimes difficult sexual and reproductive health (SRH) issues, including infertility and hypogonadism. Male infertility affects over 95% of men with CF, with obstructive azoospermia resulting from congenital bilateral absence of the vas deferens being the primary cause of infertility. SRH education for males with CF represents an important opportunity for clinical improvement, as the majority of men with CF know that their disease impacts their fertility, however the depth of this understanding varies significantly. Hypogonadism is a clinical syndrome that affects men with low testosterone level, a common finding among men with CF that has clinical implications and can significantly affect quality of life. Better understanding of SRH attitudes, the prevalence of hypogonadism, and management of infertility in men with CF should remain future research priorities.

Surgical uterine evacuation in patients with two cervices: a case series.

OBJECTIVES: To describe the clinical circumstances, surgical approach, intraoperative challenges, and outcomes of patients with two cervices undergoing uterine evacuation for spontaneous, incomplete, or induced abortion at our institution. STUDY DESIGN: We used diagnostic and procedure codes related to uterine anomalies and uterine evacuation to identify patients with cervical duplication who underwent uterine evacuation at Penn Medicine from January 2008 to December 2020. RESULTS: We identified 15 patients with cervical duplication in whom we performed uterine evacuations during 19 pregnancies. We describe in detail each patient's first pregnancy with uterine evacuation at our institution, of which providers performed 11 in the first trimester, and four in the second trimester. Ten patients had a known diagnosis of cervical duplication prior to pregnancy. Five patients did not have a diagnosis of cervical duplication prior to pregnancy and providers newly made this diagnosis intraoperatively either at our institution or referring clinics. Providers at outside institutions referred three patients to our institution for incomplete procedures. At our institution, one patient had a procedure notable for a major complication. Eight other cases were notable for surgical challenges including difficulty placing osmotic dilators, difficulty identifying the correct cervix for local anesthesia, and contralateral cervical and uterine horn instrumentation. CONCLUSIONS: This case series suggests surgical abortion in patients with two cervices is rare, but generally safe. The majority of patients did not have major complications, despite presenting a variety of unique procedural challenges. Surgical providers were sometimes the first to make the diagnosis of a second cervix. IMPLICATIONS: Uterine evacuation providers must be aware of the possibility of an undiagnosed cervical duplication, particularly when encountering surgical challenges in patients with congenital reproductive tract anomalies. Providers should consider procedures under ultrasound guidance regardless of gestational age for patients with any congenital reproductive tract anomaly or who had prior incomplete procedures.

Gestational and pregestational diabetes in pregnant women with cystic fibrosis.

As cystic fibrosis transmembrane regulator (CFTR) modulator therapies offer greater longevity and improved health quality, women living with cystic fibrosis (CF) are increasingly pursuing pregnancy. Maternal risks for pregnant women with CF largely depend on a woman's baseline pulmonary and pancreatic function, and the majority of CF pregnancies will successfully end in live births. Diabetes, either gestational or pre-existing cystic fibrosis-related diabetes (CFRD), is highly prevalent in women with CF, affecting 18 to 62% of pregnancies in recent CF center reports. In addition to the rising incidence of CFRD with age, gestational diabetes is also more common in women with CF due to lower insulin secretion, higher insulin resistance, and increased hepatic glucose production as compared to pregnant women without CF. Diabetes occurring during pregnancy has important implications for maternal and fetal health. It is well established in women without CF that glycemic control is directly associated with risks of fetal malformation, neonatal-perinatal mortality, cesarean delivery and need for neonatal intensive care. Small studies in women with CF suggest that pregnancies affected by diabetes have an increased risk of preterm delivery, lower gestational age, and lower fetal birth weight compared to those without diabetes. Women with CF preparing for pregnancy should be counseled on the risks of diabetes and should undergo routine screening for CFRD with oral glucose tolerance testing (OGTT) if not already completed in the past six months. Glycemic control in those with pre-gestational CFRD should be optimized prior to conception. Insulin is preferred for the management of diabetes in pregnant women with CF via multiple daily injections or insulin pump therapy, and continuous glucose monitors (CGM) can be useful in mitigating hypoglycemia risks. Women with CF face many unique challenges impacting diabetes care during pregnancy and would benefit from support by a multidisciplinary care team, including nutrition and endocrinology, to ensure healthy pregnancies.

Contraceptive use and preferences among females with sickle cell disease.

OBJECTIVES: Females with sickle cell disease now have a life expectancy that extends well into and beyond their reproductive years. Pregnancy and childbirth are accompanied by high morbidity and mortality in this population, rendering contraception a critical part of their health care. STUDY DESIGN: We approached adult female patients of the Hospital of the University of Pennsylvania hematology clinic who were of reproductive age (ages 18-45) and carried a diagnosis of sickle cell disease. We evaluated contraceptive method uptake and method characteristic preferences, as well as other reproductive history, and compared contraceptive uptake rates to that from female respondent data from the National Survey of Family Growth (2017-2019). RESULTS: Of 95 eligible patients, we completed surveys with 48 participants (response rate of 51%). Over half (n = 27, 56%) of participants were not currently using any form of contraception-double the rate of the general United States population (25%). The most common contraceptives currently used were the depot medroxyprogesterone (DMPA) injection (n = 6, 13%) and the progestin intrauterine device (IUD) (n = 6, 13%). DMPA uptake was significantly higher, and permanent contraceptive and oral contraceptive pill uptake significantly lower, among these participants with sickle cell disease compared to the general United States population. Participants' preferred contraceptive characteristics included effectiveness (n = 39, 81%), control over when to use the contraceptive (n = 39, 81%), and lack of side effects (n = 38, 79%). CONCLUSIONS: Contraceptive uptake was significantly lower and method mix different among females with sickle cell disease compared to the general United States population. Further research is needed on contraceptive safety, non-contraceptive benefits, and contraceptive decision-making for females with sickle cell disease. IMPLICATIONS: This study sheds light on the contraceptive choices and preferences of females with sickle cell disease, who are at disproportionate risk for pregnancy complications. In order to maximize the reproductive health of females with sickle cell disease, we must consider how their disease interacts with contraception and better understand how they approach contraceptive decision-making.

Pregnancy in cystic fibrosis: Review of the literature and expert recommendations.

Cystic fibrosis (CF) was historically a disease largely afflicting children. Due to therapeutic advancements, there are now more adults with CF than children. In the past decade, medications including Cystic Fibrosis Transmembrane conductance Regulator (CFTR) modulators became available that treat the underlying cause of CF and are dramatically improving lung function as well as quality and quantity of life for people with CF. As a result, more women with CF are becoming pregnant. We gathered a panel of experts in CF care, family planning, high risk obstetrics, nutrition, genetics and women with CF to review current literature on pregnancies and to provide care recommendations for this unique population.

Gynecologic health care for females with cystic fibrosis.

As females with cystic fibrosis (CF) increasingly reach their reproductive years, gynecologic issues have become an important area of clinical care and research. First, females with CF may have disease-specific gynecologic problems, including cyclic pulmonary symptoms, vaginal yeast infections, and urinary incontinence. Next, contraceptive methods are thought to be overall safe and effective, however further research is needed to confirm this and to understand the lower rates of uptake among females with CF compared to the general population. Further, females with CF have reduced fertility, although the etiology of this is unknown and under investigation. While assisted reproductive technologies may help achieve pregnancy, decision-making around parenthood remains complex. Finally, while patients and providers agree on the importance of sexual and reproductive health care, females with CF underutilize basic preventive services such as cervical cancer screening, and better approaches are needed to bridge the gap with gynecology. In this review, we discuss the current state of gynecologic care for females with CF, as well as clinical and research opportunities for improvement.

The Role of Subcutaneous Depot Medroxyprogesterone Acetate in Equitable Contraceptive Care: A Lesson From the Coronavirus Disease 2019 (COVID-19) Pandemic.

Since the beginning of the coronavirus disease 2019 (COVID-19) pandemic, health care professionals have made swift accommodations to provide consistent and safe care, including emphasizing remote access to allow physical distancing. Depot medroxyprogesterone acetate intramuscular injection (DMPA-IM) prescription is typically administered by a health care professional, whereas DMPA-subcutaneous has the potential to be safely self-injected by patients, avoiding contact with a health care professional. However, DMPA-subcutaneous is rarely prescribed despite its U.S. Food and Drug Administration approval in 2004 and widespread coverage by both state Medicaid providers and many private insurers. Depot medroxyprogesterone acetate users are disproportionately non-White, and thus the restriction in DMPA-subcutaneous prescribing may both stem from and contribute to systemic racial health disparities. We review evidence on acceptability, safety, and continuation rates of DMPA-subcutaneous, consider sources of implicit bias that may impede prescription of this contraceptive method, and provide recommendations for implementing DMPA-subcutaneous prescribing.