Patient Care Pathways for Pregnancy in Rare and Complex Rheumatic Diseases: Results From an International Survey.

OBJECTIVE: To map existing organizational care pathways in clinical centers of expertise that care for pregnant women affected by rare and complex connective tissue diseases (rcCTDs). METHODS: An international working group composed of experts in the field of pregnancy in rcCTDs co-designed a survey focused on organizational aspects related to the patient's pathway before, during, and after pregnancy. The survey was distributed to subject experts through referral sampling. RESULTS: Answers were collected from 69 centers in 21 countries. Patients with systemic lupus erythematosus and/or antiphospholipid syndrome were followed by more than 90% of centers, whereas those with disorders such as IgG4-related diseases were rarely covered. In the majority of centers, a multidisciplinary team was involved, including an obstetrician/gynecologist in 91.3% of cases and other healthcare professionals less frequently. Respondents indicated that 96% of the centers provided routine pre-pregnancy care, whereas the number of patient visits during pregnancy varied across centers. A formalized care pathway was described in 49.2% of centers, and 20.3% of centers had a predefined protocol for the monitoring of pregnant patients. Access to therapies during pregnancy also was heterogeneous among different centers. CONCLUSION: In international referral centers, a high level of care is provided to patients with rcCTDs before, during, and after pregnancy. No significant discrepancies were found between European and non-European countries. However, this work highlights a potential benefit to streamlining the care approaches across countries to optimize pregnancy and perinatal outcomes among patients with rcCTDs.

A Framework for Femtech: Guiding Principles for Developing Digital Reproductive Health Tools in the United States.

The United States has abysmal reproductive health indices that, in part, reflect stark inequities experienced by people of color and those with preexisting medical conditions. The growth of "femtech," or technology-based solutions to women's health issues, in the public and private sectors is promising, yet these solutions are often geared toward health-literate, socioeconomically privileged, and/or relatively healthy white cis-women. In this viewpoint, we propose a set of guiding principles for building technologies that proactively identify and address these critical gaps in health care for people from socially and economically marginalized populations that are capable of pregnancy, as well as people with serious chronic medical conditions. These guiding principles require that such technologies: (1) include community stakeholders in the design, development, and deployment of the technology; (2) are grounded in person-centered frameworks; and (3) address health disparities as a strategy to advance health equity and improve health outcomes.

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.

Redefining Primum Non Nocere to Include Reproductive Autonomy: A New Paradigm in Subspecialty Medicine.

People with chronic medical illnesses are at particularly high risk for adverse pregnancy outcomes, yet current clinical approaches largely fail to identify and support their individualized reproductive and pregnancy goals. Instead, the predominant approach to pregnancy in subspecialty medicine is disease centered rather than patient centered. To better meet the individual needs and preferences of people with childbearing potential who have chronic medical conditions, we advocate in this article for a paradigm shift in subspecialty care that honors individuals' reproductive autonomy and human right of reproduction.

Optimizing reproductive health management in lupus and Sjogren's syndrome.

PURPOSE OF REVIEW: People with childbearing capacity who are diagnosed with systemic lupus erythematosus (SLE) and Sjogren's syndrome (SS) have specific and important reproductive health considerations. RECENT FINDINGS: Recommendations from the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) provide rheumatologists and other clinicians with guidance for reproductive health management of patients with rheumatic diseases. Patient-centered reproductive health counseling can help clinicians to operationalize the EULAR and ACR guidelines and enhance patient care. SUMMARY: Disease activity monitoring, risk factor stratification, and prescription of pregnancy-compatible medications during pregnancy help to anticipate complications and enhance pregnancy outcomes in SLE and SS. Assisted reproductive technologies are also safe among people with well-controlled disease. Safe and effective contraceptive methods are available for patients with SLE and SS, and pregnancy termination appears to be safe among these patients.

Tough Choices: Exploring Medication Decision-Making During Pregnancy and Lactation Among Women With Inflammatory Arthritis.

OBJECTIVE: This study explored how women's beliefs about drug safety and interactions with their health care providers influenced their decisions to continue arthritis medications during pregnancy and lactation. METHODS: We collaborated with ArthritisPower, a patient-powered research network, and CreakyJoints, its partner online community, to develop and disseminate a survey among members with inflammatory arthritis who had at least one pregnancy after diagnosis. Participants' free-text responses were evaluated by using thematic analysis. RESULTS: Women in the sample were 40 years old on average (N = 66). Nineteen of their pregnancies had ended in fetal loss. Fifteen percent of all pregnancies were exposed to methotrexate. Among women who used safe arthritis medications, up to 80% discontinued treatment either in preparation for pregnancy or during pregnancy or lactation. Women's decisions to continue medications during pregnancy were influenced by their perceptions of safety and advisement from health care providers, although they often described that advice about medication safety was inconsistent between providers. CONCLUSION: Women often chose to endure active inflammatory arthritis rather than to use disease-modifying antirheumatic drugs because of concerns about medication safety during pregnancy and lactation. Conflicting medical advice from health care providers undermined patients' trust in their providers and in the safety of their medications. The high rate of peripartum exposure to methotrexate, a fetotoxic drug, underscores the need for better family planning care for women with childbearing potential.

Exploring the Preferences of Women Regarding Sexual and Reproductive Health Care in the Context of Rheumatology: A Qualitative Study.

OBJECTIVE: To explore the sexual and reproductive health (SRH) care and counseling needs of young women with rheumatic diseases in the context of their rheumatology care. METHODS: Semistructured qualitative telephone interviews were conducted with female patients with rheumatic diseases ages 18-45 years (n = 30). Women were recruited from outpatient rheumatology clinics in western Pennsylvania. Interviews were audiorecorded and transcribed verbatim. A codebook was inductively developed based on the interview transcripts, and the finalized coding was used to conduct a thematic analysis. RESULTS: Four themes emerged from interviews: 1) women want rheumatologists to initiate conversations about SRH and to revisit the conversation over time; 2) women desire clear and complete information regarding fetal, pregnancy, and infertility risks associated with their diseases and disease-modifying antirheumatic drugs (DMARDs); 3) women want to be treated holistically, with SRH addressed in the context of their life circumstances and personal values in addition to their rheumatic diseases; 4) women generally feel that they are intermediaries between their rheumatologists and obstetrician-gynecologists (OB/GYNs), but preferred for providers to communicate directly with one another about their SRH. CONCLUSION: Patients strongly desired rheumatologists to play an active role in their SRH, by initiating family planning conversations, providing SRH education in the context of their diseases and DMARDs, and directly coordinating SRH care with OB/GYNs. To meet patients' SRH needs, further work is needed to clarify the specific role of rheumatologists in providing SRH care and to identify ways to better facilitate communication between rheumatologists and reproductive health care providers.

Contraception decision-making and care among reproductive-aged women with autoimmune diseases.

OBJECTIVE: In this qualitative study, we sought to understand better the contraception-related experiences and decision-making of women with autoimmune diseases, a group at high risk for adverse pregnancy and perinatal outcomes. STUDY DESIGN: We conducted semi-structured telephone interviews with women ages 18 to 45 who had at least one autoimmune disease diagnosis. Our team recruited from outpatient rheumatology clinics in Pittsburgh, Pennsylvania. We analyzed our data for content and themes, using inductive and deductive techniques and the constant comparison approach. RESULTS: The 30 women in the sample ranged from 25 to 44 years old; most were white (73%), married (52%), and had at least one child (53%). Four themes emerged from our interviews: (1) Women's decisions to use contraception were strongly influenced by their diseases and perceptions of their health; (2) Women felt that hormonal contraception was unsafe in the context of their diseases, even women without clear disease-related health risks; (3) Health care providers influenced women's consideration and selection of contraceptive methods; (4) Women rarely expected that their rheumatologists would prescribe their contraception, yet their contraception prescribers lacked knowledge about their disease-specific health risks. CONCLUSIONS: Women perceived that hormonal contraception is unsafe in the context of their autoimmune diseases and cited medication fatigue and safety concerns as reasons why they did not use contraception. Some women did not appear to receive patient-centered contraception counseling from providers. Our interviews reveal areas for improving contraception care, including augmenting shared decision-making and educating patients and providers about contraception safety. IMPLICATIONS: Our findings highlight potential tensions between patients' preferences for contraception, safety considerations, and ethical and medically responsible contraception care by health care providers in the rheumatology context. Future work is needed to develop person-centered, medically-appropriate models for contraception care that meet the needs of medically vulnerable populations.

Antirheumatic medications in pregnancy and breastfeeding.

PURPOSE OF REVIEW: As active rheumatic and musculoskeletal disease during pregnancy increases the risk for pregnancy loss, preterm birth, and maternal illness, ongoing management with pregnancy-compatible medications can improve these outcomes. Selecting and taking these medications can be challenging for rheumatologists and patients due to limited knowledge about potential risks and benefits. RECENT FINDINGS: Fortunately, the American College of Rheumatology, American College of Obstetrics and Gynecology, British Rheumatology Society, and the European League Against Rheumatism have each published recommendations to guide the use of antirheumatic medications in pregnancy and lactation. Each of these groups endorsed the use of hydroxychloroquine, azathioprine, sulfasalazine, corticosteroids, NSAIDs, and tumor necrosis factor inhibitors in pregnancy. They also agreed that methotrexate, mycophenolate, cyclophosphamide, and leflunomide should be avoided in pregnancy. New medications, including small-molecules and biologics, have limited data to support safety in pregnancy and are not currently recommended during this period. Most antirheumatic medications are compatible with lactation. SUMMARY: Because many patients are hesitant to use antirheumatic medications during pregnancy, honest and accurate discussions about pregnancy planning and management are important to help women make decisions that are in their and their offspring's best interest.

2020 American College of Rheumatology Guideline for the Management of Reproductive Health in Rheumatic and Musculoskeletal Diseases.

OBJECTIVE: To develop an evidence-based guideline on contraception, assisted reproductive technologies (ART), fertility preservation with gonadotoxic therapy, use of menopausal hormone replacement therapy (HRT), pregnancy assessment and management, and medication use in patients with rheumatic and musculoskeletal disease (RMD). METHODS: We conducted a systematic review of evidence relating to contraception, ART, fertility preservation, HRT, pregnancy and lactation, and medication use in RMD populations, using Grading of Recommendations Assessment, Development and Evaluation methodology to rate the quality of evidence and a group consensus process to determine final recommendations and grade their strength (conditional or strong). Good practice statements were agreed upon when indirect evidence was sufficiently compelling that a formal vote was unnecessary. RESULTS: This American College of Rheumatology guideline provides 12 ungraded good practice statements and 131 graded recommendations for reproductive health care in RMD patients. These recommendations are intended to guide care for all patients with RMD, except where indicated as being specific for patients with systemic lupus erythematosus, those positive for antiphospholipid antibody, and/or those positive for anti-Ro/SSA and/or anti-La/SSB antibodies. Recommendations and good practice statements support several guiding principles: use of safe and effective contraception to prevent unplanned pregnancy, pre-pregnancy counseling to encourage conception during periods of disease quiescence and while receiving pregnancy-compatible medications, and ongoing physician-patient discussion with obstetrics/gynecology collaboration for all reproductive health issues, given the overall low level of available evidence that relates specifically to RMD. CONCLUSION: This guideline provides evidence-based recommendations developed and reviewed by panels of experts and RMD patients. Many recommendations are conditional, reflecting a lack of data or low-level data. We intend that this guideline be used to inform a shared decision-making process between patients and their physicians on issues related to reproductive health that incorporates patients' values, preferences, and comorbidities.

2020 American College of Rheumatology Guideline for the Management of Reproductive Health in Rheumatic and Musculoskeletal Diseases.

OBJECTIVE: To develop an evidence-based guideline on contraception, assisted reproductive technologies (ART), fertility preservation with gonadotoxic therapy, use of menopausal hormone replacement therapy (HRT), pregnancy assessment and management, and medication use in patients with rheumatic and musculoskeletal disease (RMD). METHODS: We conducted a systematic review of evidence relating to contraception, ART, fertility preservation, HRT, pregnancy and lactation, and medication use in RMD populations, using Grading of Recommendations Assessment, Development and Evaluation methodology to rate the quality of evidence and a group consensus process to determine final recommendations and grade their strength (conditional or strong). Good practice statements were agreed upon when indirect evidence was sufficiently compelling that a formal vote was unnecessary. RESULTS: This American College of Rheumatology guideline provides 12 ungraded good practice statements and 131 graded recommendations for reproductive health care in RMD patients. These recommendations are intended to guide care for all patients with RMD, except where indicated as being specific for patients with systemic lupus erythematosus, those positive for antiphospholipid antibody, and/or those positive for anti-Ro/SSA and/or anti-La/SSB antibodies. Recommendations and good practice statements support several guiding principles: use of safe and effective contraception to prevent unplanned pregnancy, pre-pregnancy counseling to encourage conception during periods of disease quiescence and while receiving pregnancy-compatible medications, and ongoing physician-patient discussion with obstetrics/gynecology collaboration for all reproductive health issues, given the overall low level of available evidence that relates specifically to RMD. CONCLUSION: This guideline provides evidence-based recommendations developed and reviewed by panels of experts and RMD patients. Many recommendations are conditional, reflecting a lack of data or low-level data. We intend that this guideline be used to inform a shared decision-making process between patients and their physicians on issues related to reproductive health that incorporates patients' values, preferences, and comorbidities.

Sexual and reproductive healthcare for adolescent and young adult women with epilepsy: A qualitative study of pediatric neurologists and epileptologists.

OBJECTIVE: The objective of this study was to explore the attitudes and practices of pediatric neurologists and epileptologists regarding sexual and reproductive healthcare for adolescent and young adult women with epilepsy (WWE). BACKGROUND: Adolescent and young adult WWE have unique sexual and reproductive healthcare needs, including counseling on teratogenesis, folic acid, and interactions between contraception and antiseizure medications. There are no prior studies regarding sexual and reproductive healthcare practices of pediatric neurologists or epileptologists. DESIGN/METHODS: Individual semi-structured interviews were conducted with pediatric neurologists and epileptologists regarding their attitudes, practices, and experiences with sexual and reproductive healthcare for adolescent and young adult WWE. Interviews were audio-recorded and transcribed verbatim. Qualitative analysis was conducted using a thematic analysis approach. RESULTS: Six child neurologists and 10 epileptologists (44% male) participated. Major themes included the following: (1) Sexual and reproductive healthcare is important for adolescent WWE, and neurologists have a key role in providing this care. (2) Sexual and reproductive healthcare should be comanaged with a primary care provider or women's health provider although neurologists have significant concerns regarding comanagement. (3) There is wide variability in sexual and reproductive healthcare practice among pediatric neurologists and epileptologists. Important subthemes included parent education and differences in sexual and reproductive healthcare practices for women with intellectual disabilities. (4) Many systemic and interpersonal barriers exist to delivering sexual and reproductive healthcare to adolescent and young adult WWE. Important barriers included limited time; provider, patient, or family discomfort; and lack of necessary knowledge or expertise. (5) Providers desire standardization of sexual and reproductive healthcare for adolescent WWE along with patient and provider education. CONCLUSION: This is the first study to assess attitudes and practices of pediatric neurologists and epileptologists regarding sexual and reproductive healthcare for adolescent and young adult WWE. Our findings suggest that there is a need for development of improved systems for sexual and reproductive healthcare delivery and comanagement for adolescent and young adult WWE. Providers identified many barriers and facilitators that might serve as the basis for interventions to improve care.

Perspectives of Adult Rheumatologists Regarding Family Planning Counseling and Care: A Qualitative Study.

OBJECTIVE: Little is known about whether and how rheumatologists provide family planning counseling and reproductive health care (FPCC) to reproductive-age women with rheumatic diseases. This qualitative study sought to assess rheumatologists' perspectives, attitudes, and practices regarding FPCC. METHODS: Semistructured interviews were conducted with a geographically diverse US sample of rheumatologists (n = 12). Interviews were transcribed verbatim, and a code book was inductively developed based on transcript content. Two coders applied the code book to all transcripts, and coding differences were adjudicated to full agreement. The finalized coding was used to conduct a thematic analysis. RESULTS: Six themes were identified across interviews. Rheumatologists said that they 1) feel responsible for providing some FPCC to patients, 2) experience tension between respecting patients' autonomy and their own anxieties about managing high-risk pregnancies, 3) view patient-initiated conversations as FPCC facilitators, and they regard lack of guidelines and the presence of competing clinical priorities as barriers to FPCC, 4) are reluctant to prescribe contraception, 5) desire greater access to resources to help guide FPCC, and 6) recognize the benefits of multidisciplinary collaboration with gynecologists. CONCLUSION: Rheumatologists feel a sense of responsibility to provide some aspects of FPCC to reproductive-age female patients. However, their own apprehensions about managing complicated pregnancies may negatively influence how they advise patients about pregnancy planning or avoidance. Rheumatologists do not prescribe contraception but rarely refer patients to gynecologists for contraceptive care. Future work should focus on eliminating barriers and identifying solutions that support rheumatologists' efforts to provide high-quality FPCC to patients.

Pregnancy, Periods, and "The Pill": Exploring the Reproductive Experiences of Women with Inflammatory Arthritis.

OBJECTIVE: Women with inflammatory arthritis appear to have fewer children as compared with healthy women, but few studies have assessed how patients' attitudes and decision making influence their family sizes. Little is also known about how patients experience other aspects of their reproductive lives, such as menstruation and contraception. METHODS: We partnered with ArthritisPower, a patient-powered research network, and its associated online patient community, CreakyJoints, to create and disseminate a survey among female members aged 18-50 years with inflammatory arthritis. RESULTS: Women in the final sample (n = 267) were 40 years old on average; most had rheumatoid arthritis (79%) and were predominantly white and college educated. Many women chose to limit childbearing because of their arthritis (58%); they feared that their arthritis was heritable, their diseases and medications could directly harm a fetus, they would be incapable of physically caring for a child, and arthritis could cause premature death, preventing them from raising their children. Infertility affected 40% of the sample. Half of women experienced subjective arthritis flares around the time of menstruation. Oral contraceptive pills (OCPs) did not worsen disease activity for most women and even prevented menstrual-associated arthritis flares for a subset of women. CONCLUSION: Our findings suggest that infertility, but also potentially outsized fear and anxiety related to their diagnoses, may affect the family sizes of women with inflammatory arthritis. The observation that menstruation worsens disease activity for some women requires additional study, and OCP use should be explored as a possible treatment for menstrual-associated arthritis. Clinicians may wish to consider how they communicate patients' individual pregnancy-associated risks, reassure patients when appropriate, and help to guide and support patients to make well-informed reproductive decisions.

Development of ReproKnow, a reproductive knowledge assessment for women with rheumatic diseases.

BACKGROUND: The objective of this study was to develop an assessment tool, ReproKnow, to evaluate the reproductive health knowledge of women with a wide range of rheumatic diseases. METHODS: The 10-item multiple-choice questionnaire was developed with feedback from a panel of content experts and female patients with rheumatic diseases. Construct validity using known-groups analysis was evaluated through comparison of median total ReproKnow scores between rheumatology fellows and nurses. Female patients aged 18-50 years were recruited to take ReproKnow and demographic questionnaires in two outpatient clinics. Associations between patients' mean total knowledge scores and demographic characteristics were assessed using independent-sample t-tests. Questions were also categorized by topical area, and the percentages were calculated. RESULTS: The completion rate of questions in ReproKnow was 100% across all users. Median ReproKnow scores were significantly higher among rheumatology fellows than among nurses (p = 0.045). The 153 patients recruited to the study had at least one of 15 rheumatic diseases. Patients' mean knowledge score was 5.05 (SD 2.24) out of a possible high score of 10. Patients who were younger, White, and more educated had significantly higher scores than did other patients (p's < 0.05). Patients who bore children after their disease diagnosis had higher knowledge scores than did women whose children were born prior to their diagnosis; in contrast, women with histories of surgical sterilization or hysterectomy had lower knowledge scores than other women. Knowledge scores of women who used potentially fetotoxic medications did not vary from the remainder of the sample. Patients demonstrated gaps in knowledge about birth outcomes, contraceptive efficacy, and breastfeeding safety. CONCLUSIONS: Initial testing of ReproKnow suggests that it may be a promising tool to assess the reproductive health knowledge of women with diverse rheumatic diseases. Specific knowledge deficits elicited from ReproKnow may be important targets for future educational interventions.

Contraception Use Among Reproductive-Age Women With Rheumatic Diseases.

OBJECTIVE: To determine contraception use among a cohort of reproductive-age women (ages 18-50 years) with rheumatic diseases. METHODS: We conducted a study of administrative data from a single, large medical center between the years 2013 and 2014. Women who had 1 of 21 possible rheumatic disease diagnoses and had at least 2 outpatient rheumatology visits were included in this analysis. We used logistic regression analyses to evaluate adjusted associations between the use of prescription contraception, use of potentially fetotoxic medications, and visits with rheumatologists, primary care providers, and gynecologists. RESULTS: Of 2,455 women in this sample, 32.1% received any prescription contraception, and 7.9% of women used highly effective prescription methods (intrauterine devices, implants, and surgical sterilization). More than 70% of women took ≥1 type of fetotoxic medication during the 2-year study timeframe. Fetotoxic medication use was not associated with overall use of prescription contraception, but was associated with the use of highly effective contraceptive methods (adjusted odds ratio [OR] 2.26 [95% confidence interval (95% CI) 1.44-3.54]). Women who saw gynecologists or primary care providers were more likely to use prescription contraception overall (adjusted OR 3.35 [95% CI 2.77-4.05] and 1.43 [95% CI 1.18-1.73], respectively). Women who saw gynecologists were more likely to use highly versus moderately effective contraceptive methods (adjusted OR 2.35 [95% CI 1.41-3.94]). Rheumatology visits were not associated with use of prescription contraception in any models. CONCLUSION: This is the largest study to date to describe contraceptive use among reproductive-age women with rheumatic diseases, and the findings reveal low use of prescription contraception. Urgent efforts are needed to improve contraceptive care and access for some women with rheumatic diseases.