Homozygous SPTA1-associated hereditary pyropoikilocytosis presenting as hydrops fetalis.

INTRODUCTION: Hereditary pyropoikilocytosis (HPP) is a heterogeneous inherited disorder of red blood cell (RBC) membrane and cytoskeletal proteins that leads to hemolytic anemia. HPP is characterized by marked poikilocytosis, microspherocytes, RBC fragmentation, and elliptocytes on peripheral blood smear. Mutations in SPTA1 can cause HPP due to a quantitative defect in α-spectrin and can lead to profound fetal anemia and nonimmune hydrops fetalis, which can be managed with intrauterine transfusion. CASE PRESENTATION: We present a case of a 26-year-old G4P2102 woman of Amish-Mennonite ancestry with a pregnancy complicated by fetal homozygosity for an SPTA1 gene variant (SPTA1c.6154delG) as well as severe fetal anemia and hydrops fetalis, which was managed with four intrauterine transfusions between 26 and 30 weeks gestation. Pre-transfusion peripheral smears from fetal blood samples showed RBC morphology consistent with HPP. The neonate had severe hyperbilirubinemia at birth, which has resolved, but remains transfusion-dependent at 6 months of life. DISCUSSION/CONCLUSION: To our knowledge, this is the first report that correlates homozygosity of the SPTA1c.6154delG gene variant with RBC dysmorphology and establishes the diagnosis of HPP.

Perinatal outcomes according to umbilical artery Doppler assessment among fetuses with congenital heart disease.

PURPOSE: To evaluate a cohort of fetuses with congenital heart disease (CHD) who underwent serial umbilical artery (UA) Doppler surveillance and assess perinatal outcome according to UA Doppler assessment. METHODS: A retrospective cohort study of singleton fetuses with CHD at a single academic center was performed between 2018 and 2020. Fetuses with a chromosomal abnormality or growth restriction were excluded. We compared fetuses with normal versus abnormal UA Doppler assessment at any time in pregnancy. Abnormal UA Doppler assessment was defined as decreased end diastolic flow, determined by an elevated systolic/diastolic ratio >95th percentile for gestational age, or absent/reversed end diastolic flow. Logistic regression assessed the odds of fetuses with CHD and abnormal UA Doppler assessment having a composite adverse perinatal (defined as fetal, neonatal, or infant death), adjusting for relevant covariates. RESULTS: We identified a cohort of 171 fetuses with CHD that met inclusion criteria. Of these, 154 (90%) had normal UA Doppler assessment and 17 (10%) had abnormal UA Doppler assessment throughout pregnancy. Maternal characteristics did not differ between groups except for maternal race and history of preeclampsia. There was no statistically significant difference in primary outcome between groups [14% (21/154) of fetuses with normal UA Doppler assessment had an adverse perinatal outcome compared to 24% (4/17) of those with abnormal UA Doppler assessment, p = 0.28]. CONCLUSION: UA Doppler assessment is unlikely to predict adverse perinatal outcome in normally grown, euploid singleton fetuses with CHD.

Delivery outcomes in the subsequent pregnancy following the conservative management of placenta accreta spectrum disorder: a systematic review and meta-analysis.

OBJECTIVE: Cesarean hysterectomy is generally presumed to decrease maternal morbidity and mortality secondary to placenta accreta spectrum disorder. Recently, uterine-sparing techniques have been introduced in conservative management of placenta accreta spectrum disorder to preserve fertility and potentially reduce surgical complications. However, despite patients often expressing the intention for future conception, few data are available regarding the subsequent pregnancy outcomes after conservative management of placenta accreta spectrum disorder. Thus, we aimed to perform a systematic review and meta-analysis to assess these outcomes. DATA SOURCES: PubMed, Scopus, and Web of Science databases were searched from inception to September 2022. STUDY ELIGIBILITY CRITERIA: We included all studies, with the exception of case studies, that reported the first subsequent pregnancy outcomes in individuals with a history of placenta accreta spectrum disorder who underwent any type of conservative management. METHODS: The R programming language with the "meta" package was used. The random-effects model and inverse variance method were used to pool the proportion of pregnancy outcomes. RESULTS: We identified 5 studies involving 1458 participants that were eligible for quantitative synthesis. The type of conservative management included placenta left in situ (n=1) and resection surgery (n=1), and was not reported in 3 studies. The rate of placenta accreta spectrum disorder recurrence in the subsequent pregnancy was 11.8% (95% confidence interval, 1.1-60.3; I2=86.4%), and 1.9% (95% confidence interval, 0.0-34.1; I2=82.4%) of participants underwent cesarean hysterectomy. Postpartum hemorrhage occurred in 10.3% (95% confidence interval, 0.3-81.4; I2=96.7%). A composite adverse maternal outcome was reported in 22.7% of participants (95% confidence interval, 0.0-99.4; I2=56.3%). CONCLUSION: Favorable pregnancy outcome is possible following successful conservation of the uterus in a placenta accreta spectrum disorder pregnancy. Approximately 1 out of 4 subsequent pregnancies following conservative management of placenta accreta spectrum disorder had considerable adverse maternal outcomes. Given such high incidence of adverse outcomes and morbidity, patient and provider preparation is vital when managing this population.

Placenta Accreta Spectrum Disorders: Update and Pictorial Review of the SAR-ESUR Joint Consensus Statement for MRI.

Placenta accreta spectrum (PAS) disorders are a major cause of maternal morbidity and mortality and are increasing in incidence owing to a rising rate of cesarean delivery. US is the primary imaging tool for evaluation of PAS disorders, which are most often diagnosed during routine early second-trimester US to assess fetal anatomy. MRI serves as a complementary modality, providing value when the diagnosis is equivocal at US and evaluating the extent and topography of myoinvasion for surgical planning in severe cases. While the definitive diagnosis is established by a combined clinical and histopathologic classification at delivery, accurate antenatal diagnosis and multidisciplinary management are critical to guide treatment and ensure optimal outcomes for these patients. Many MRI features of PAS disorders have been described in the literature. To standardize assessment at MRI, the Society of Abdominal Radiology (SAR) and European Society of Urogenital Radiology (ESUR) released a joint consensus statement to provide guidance for image acquisition, image interpretation, and reporting of PAS disorders. The authors review the role of imaging in diagnosis of PAS disorders, describe the SAR-ESUR consensus statement with a pictorial review of the seven major MRI features recommended for use in diagnosis of PAS disorders, and discuss management of these patients. Familiarity with the spectrum of MRI findings of PAS disorders will provide the radiologist with the tools needed to more accurately diagnose this disease and make a greater impact on the care of these patients. ©RSNA, 2023 Supplemental material is available for this article. Quiz questions for this article are available through the Online Learning Center. See the invited commentary by Jha and Lyell in this issue.

Outcomes following a clinical algorithm allowing for delayed hysterectomy in the management of severe placenta accreta spectrum.

BACKGROUND: The incidence of placenta accreta spectrum is rising. Management is most commonly with cesarean hysterectomy. These deliveries often are complicated by massive hemorrhage, urinary tract injury, and admission to the intensive care unit. Up to 60% of patients require transfusion of ≥4 units of packed red blood cells. There is also a significant risk of death of up to 7%. OBJECTIVE: The purpose of this study was to assess the outcomes of patients with antenatal diagnosis of placenta percreta that was managed with delayed hysterectomy as compared with those patients who underwent immediate cesarean hysterectomy. STUDY DESIGN: We performed a retrospective study of all patients with an antepartum diagnosis of placenta percreta at our large academic institution from January 1, 2012, to May 30, 2018. Patients were treated according to standard clinical practice that included scheduled cesarean delivery at 34-35 weeks gestation and intraoperative multidisciplinary decision-making regarding immediate vs delayed hysterectomy. In cases of delayed hysterectomy, the hysterotomy for cesarean birth used a fetal surgery technique to minimize blood loss, with a plan for hysterectomy 4-6 weeks after delivery. We collected data regarding demographics, maternal comorbidities, time to interval hysterectomy, blood loss, need for transfusion, occurrence of urinary tract injury and other maternal complications, and maternal and fetal mortality rates. Descriptive statistics were performed, and Wilcoxon rank-sum and chi-square tests were used as appropriate. RESULTS: We identified 49 patients with an antepartum diagnosis of placenta percreta who were treated at Vanderbilt University Medical Center during the specified period. Of these patients, 34 were confirmed to have severe placenta accreta spectrum, defined as increta or percreta at the time of delivery. Delayed hysterectomy was performed in 14 patients: 9 as scheduled and 5 before the scheduled date. Immediate cesarean hysterectomy was completed in 20 patients: 16 because of intraoperative assessment of resectability and 4 because of preoperative or intraoperative bleeding. The median (interquartile range) estimated blood loss at delayed hysterectomy of 750 mL (650-1450 mL) and the sum total for delivery and delayed hysterectomy of 1300 mL (70 -2150 mL) were significantly lower than the estimated blood loss at immediate hysterectomy of 3000 mL (2375-4250 mL; P<.01 and P=.037, respectively). The median (interquartile range) units of packed red blood cells that were transfused at delayed hysterectomy was 0 (0-2 units), which was significantly lower than units transfused at immediate cesarean hysterectomy (4 units [2-8.25 units]; P<.01). Nine of 20 patients (45%) required transfusion of ≥4 units of red blood cells at immediate cesarean hysterectomy, whereas only 2 of 14 patients (14.2%) required transfusion of ≥4 units of red blood cells at the time of delayed hysterectomy (P=.016). There was 1 maternal death in each group, which were incidences of 7% and 5% in the delayed and immediate hysterectomy patients, respectively. CONCLUSION: Delayed hysterectomy may represent a strategy for minimizing the degree of hemorrhage and need for massive blood transfusion in patients with an antenatal diagnosis of placenta percreta by allowing time for uterine blood flow to decrease and for the placenta to regress from surrounding structures.

Postpartum Tubal Sterilization: Making the Case for Urgency.

The parturient who requests postpartum sterilization has given consideration to and has made decisions regarding this aspect of her medical care long before her delivery. She arrives at parturition expecting the postpartum procedure to be performed as intended. The American Congress of Obstetricians and Gynecologists has reaffirmed its opinion that postpartum sterilization is an urgent procedure, owing to the safety and superior effectiveness of tubal sterilization via minilaparotomy in the immediate postpartum period, and the adverse consequences for mothers, babies, and society when the procedure is not actualized as desired and intended. In contrast, recent practice guidelines for obstetric anesthesia address anesthetic procedural aspects and short-term safety but overlook the long-term complications and considerations associated with failure to perform postpartum sterilization as planned. In practice, procedure completion rates are strikingly low, reportedly ranging from 31% to 52%. Reasons for failure to complete abound and include inadequate resources or inavailability of necessary personnel; obstetrician reluctance due to concerns for patient regret in younger women or medical comorbidities; barriers related to provision of obstetric care in a religiously affiliated hospital, or incomplete, improperly completed, or unavailable original federal consent forms among Medicaid-insured women. The federal requirement to wait 30 days after signing informed consent, and to retain the original signed document to be physically verified at time of the procedure, serves as a significant source of health care disparity for Medicaid-dependent mothers. This article reviews these larger issues of maternal health and comprehensive maternal care to broaden the anesthesiologist's appreciation of major benefits and potential risks of postpartum sterilization, including long-term effects, to promote an evidence-based, informed, and proactive role in delivering equitable, safe, and optimal care for these patients.

Development and Validation of an Automated, Real-Time Predictive Model for Postpartum Hemorrhage.

OBJECTIVE: To develop and validate a predictive model for postpartum hemorrhage that can be deployed in clinical care using automated, real-time electronic health record (EHR) data and to compare performance of the model with a nationally published risk prediction tool. METHODS: A multivariable logistic regression model was developed from retrospective EHR data from 21,108 patients delivering at a quaternary medical center between January 1, 2018, and April 30, 2022. Deliveries were divided into derivation and validation sets based on an 80/20 split by date of delivery. Postpartum hemorrhage was defined as blood loss of 1,000 mL or more in addition to postpartum transfusion of 1 or more units of packed red blood cells. Model performance was evaluated by the area under the receiver operating characteristic curve (AUC) and was compared with a postpartum hemorrhage risk assessment tool published by the CMQCC (California Maternal Quality Care Collaborative). The model was then programmed into the EHR and again validated with prospectively collected data from 928 patients between November 7, 2023, and January 31, 2024. RESULTS: Postpartum hemorrhage occurred in 235 of 16,862 patients (1.4%) in the derivation cohort. The predictive model included 21 risk factors and demonstrated an AUC of 0.81 (95% CI, 0.79-0.84) and calibration slope of 1.0 (Brier score 0.013). During external temporal validation, the model maintained discrimination (AUC 0.80, 95% CI, 0.72-0.84) and calibration (calibration slope 0.95, Brier score 0.014). This was superior to the CMQCC tool (AUC 0.69 [95% CI, 0.67-0.70], P <.001). The model maintained performance in prospective, automated data collected with the predictive model in real time (AUC 0.82 [95% CI, 0.73-0.91]). CONCLUSION: We created and temporally validated a postpartum hemorrhage prediction model, demonstrated its superior performance over a commonly used risk prediction tool, successfully coded the model into the EHR, and prospectively validated the model using risk factor data collected in real time. Future work should evaluate external generalizability and effects on patient outcomes; to facilitate this work, we have included the model coefficients and examples of EHR integration in the article.

Management of Ebstein Anomaly in the Current Era: The Story of One Fetus and the Collaboration of Many-A Case Report.

Collaborative multicenter research has significantly increased our understanding of fetal Ebstein anomaly, delineating risk factors for adverse outcomes as well as predictors of postnatal management. These data are incorporated into prenatal care and therapeutic strategies and inform family counseling and delivery planning to optimize care. This report details the translation of findings from multicenter studies into multidisciplinary prenatal care for a fetus with Ebstein anomaly, supraventricular tachycardia, and a circular shunt, including transplacental therapy to control arrhythmias and achieve ductal constriction, informed and coordinated delivery room management, and planned univentricular surgical palliation.

Placenta accreta spectrum care infrastructure: an evidence-based review of needed resources supporting placenta accreta spectrum care.

The incidence of placenta accreta spectrum, the deeply adherent placenta with associated increased risk of maternal morbidity and mortality, has seen a significant rise in recent years. Therefore, there has been a rise in clinical and research focus on this complex diagnosis. There is international consensus that a multidisciplinary coordinated approach optimizes outcomes. The composition of the team will vary from center to center; however, central themes of complex surgical experts, specialists in prenatal diagnosis, critical care specialists, neonatology specialists, obstetrics anesthesiology specialists, blood bank specialists, and dedicated mental health experts are universal throughout. Regionalization of care is a growing trend for complex medical needs, but the location of care alone is just a starting point. The goal of this article is to provide an evidence-based framework for the crucial infrastructure needed to address the unique antepartum, delivery, and postpartum needs of the patient with placenta accreta spectrum. Rather than a clinical checklist, we describe the personnel, clinical unit characteristics, and breadth of contributing clinical roles that make up a team. Screening protocols, diagnostic imaging, surgical and potential need for critical care, and trauma-informed interaction are the basis for comprehensive care. The vision from the author group is that this publication provides a semblance of infrastructure standardization as a means to ensure proper preparation and readiness.

Placenta Accreta Spectrum.

Placenta accreta spectrum (PAS) is one of the most dangerous conditions in pregnancy and is increasing in frequency. The risk of life-threatening bleeding is present throughout pregnancy but is particularly high at the time of delivery. Although the exact cause is unknown, the result is clear: Severe PAS distorts the uterus and surrounding anatomy and transforms the pelvis into an extremely high-flow vascular state. Screening for risk factors and assessing placental location by antenatal ultrasonography are essential for timely diagnosis. Further evaluation and confirmation of PAS are best performed in referral centers with expertise in antenatal imaging and surgical management of PAS. In the United States, cesarean hysterectomy with the placenta left in situ after delivery of the fetus is the most common treatment for PAS, but even in experienced referral centers, this treatment is often morbid, resulting in prolonged surgery, intraoperative injury to the urinary tract, blood transfusion, and admission to the intensive care unit. Postsurgical complications include high rates of posttraumatic stress disorder, pelvic pain, decreased quality of life, and depression. Team-based, patient-centered, evidence-based care from diagnosis to full recovery is needed to optimally manage this potentially deadly disorder. In a field that has relied mainly on expert opinion, more research is needed to explore alternative treatments and adjunctive surgical approaches to reduce blood loss and postoperative complications.

Early Femoral Access by Acute Care Surgeons: A Multidisciplinary Approach to Prevent Maternal Exsanguination in Placenta Accreta Spectrum.

Resuscitative endovascular balloon occlusion of the aorta (REBOA) use has expanded to the obstetric condition of placenta accreta spectrum (PAS). Early reports of REBOA for PAS describe prophylactic catheter deployment. We developed a multidisciplinary approach to PAS, with early femoral artery access and selective REBOA deployment. We compared morbidity, mortality, and blood loss before and after implementation of our multidisciplinary protocol for PAS. Prior to, femoral access was obtained only emergently, and maternal death occurred in 2/3 cases (66%). Following protocol implementation, there was one maternal death (6%). There were no access-related complications. We have not yet needed to deploy the REBOA during PAS cases. In contrast to urgent hemorrhage control or prophylactic REBOA deployment, routine early femoral arterial access and selective REBOA deployment as part of a multidisciplinary team approach is a novel strategy for managing PAS. Our experience suggests most PAS cases do not require prophylactic REBOA deployment.

Effect of panniculus elevation device on postoperative pain after cesarean delivery: a randomized controlled trial.

BACKGROUND: Suboptimal treatment of postpartum pain is associated with persistent pain and postpartum depression. Multimodal analgesia can achieve superior pain relief and decreased opioid consumption after surgery. There is limited and conflicting data on abdominal support devices to reduce postoperative pain and opioid use after cesarean delivery. OBJECTIVE: This study aimed to examine whether the use of a panniculus elevation device decreases opioid use and improves postoperative pain after cesarean delivery. STUDY DESIGN: This was an unblinded prospective trial in which eligible, consenting patients aged ≥18 years were randomized to the panniculus elevation device group or no device group within 36 hours after cesarean delivery. The device studied adheres to the abdomen and lifts the panniculus. Moreover, it can be repositioned during use. Patients with a vertical skin incision or chronic opioid use disorder were excluded. Participants were surveyed 10 and 14 days after delivery about opioid use and pain satisfaction. The primary outcome was total morphine milligram equivalents used after delivery. The secondary outcomes were inpatient and outpatient opioid use, subjective pain scores, and Patient-Reported Outcomes Measurement Information System pain interference scores. An a priori subgroup analysis was performed on participants with obesity who may uniquely benefit from panniculus elevation. RESULTS: Of 538 patients screened for inclusion from April 2021 to July 2022, 484 were eligible, and 278 were consented and randomized. Moreover, 56 participants (20%) were lost to follow-up, leaving 222 (118 in the device group vs 104 in the control group) for analysis. Follow-up frequency was similar between groups (P=.09). Demographic and clinical characteristics were similar between groups. There was no statistically significant difference in total opioid use, other opioid use measures, or pain satisfaction outcomes. Median device use was 5 days (interquartile range, 3-9), and 64% of participants randomized to device use stated that they would use it again. Here, similar trends were observed among participants with obesity (n=152). CONCLUSION: The use of a panniculus elevation device did not significantly reduce total opioid use after cesarean delivery in patients.

A Large Thyroid Goiter in a Newborn With Congenital Hypothyroidism: Timeline for Decrease in Size of Thyroid.

Congenital hypothyroidism rarely causes a clinically significant neck mass in newborns. We present the case of a newborn with congenital hypothyroidism and significantly enlarged goiter and discuss imaging considerations and medical and surgical management. This infant was prenatally discovered to have a midline neck mass on 28 week ultrasound measuring 6.0 cm × 3.4 cm × 5.8 cm. Diagnostic cordocentesis demonstrated elevated thyroid-stimulating hormone (TSH, 361 µIU/mL). Maternal evaluation for thyroid disease and antithyroid antibodies was negative. A Cesarean section at 38 weeks gestation was recommended due to hyperextension of the fetal neck. The infant was intubated for respiratory distress. Postnatal magnetic resonance imaging revealed a 5.5 cm × 4.4 cm × 7.6 cm goiter and laboratory studies confirmed the diagnosis of primary hypothyroidism (TSH 16.7 µIU/mL). Treatment was initiated with intravenous levothyroxine and transitioned to oral supplementation. Serial ultrasounds showed decreased goiter volume over several weeks, with recent volume per lobe being 22% and 44% of original volume. This case demonstrates the importance of prompt diagnosis and initiation of thyroid hormone replacement, allowing for significant goiter regression without surgical intervention and ensuring normal growth and neurodevelopmental outcome. Surgical management should be considered for those with persistent compressive symptoms despite optimal medical management.

Impact of fetal trisomy 21 on umbilical artery Doppler indices.

OBJECTIVES: Umbilical artery (UA) Doppler indices are surrogate measures of placental function, most commonly used to assess fetal wellbeing in pregnancies with fetal growth restriction. Fetuses with trisomy 21 (t21) are reported to have elevated UA Doppler indices, but reference percentiles are currently lacking for this population. We hypothesized that gestational age-specific values of UA Doppler indices in pregnancies complicated by t21 will be elevated compared to established percentiles based on euploid pregnancies. We aimed to assess UA Doppler indices longitudinally in fetuses with t21 in order to demonstrate Doppler patterns across gestation in this population, compare them with euploid fetuses, and investigate their association with pregnancy outcomes. METHODS: We conducted a retrospective cohort study of singleton pregnancies with confirmed fetal t21 who underwent UA Doppler surveillance antenatally from January 2012 to August 2019. UA Doppler indices, including systolic/diastolic (S/D) ratio, pulsatility index (PI), and resistance index (RI) were extracted from ultrasound reports or directly from ultrasound images. UA S/D, PI, and RI percentiles by gestational week were created from available observations from our cohort via a data-driven approach using a generalized additive model. A secondary analysis was run to statistically compare t21 values to established percentiles based on observations from a historical population of euploid fetuses. RESULTS: UA Doppler measurements from 86 t21 fetuses and 130 euploid fetuses were included in our analysis. Median (IQR) maternal age in t21 pregnancies and euploid pregnancies were 35 years (29-38) and 30 years (27-33), respectively. As in euploid fetuses, we found a negative association between Doppler indices and gestational age in the t21 fetuses. Maternal tobacco use, obesity, or chronic hypertension had no significant effect on UA Doppler indices. As hypothesized, values for UA S/D ratio, PI, and RI at the 2.5th, 5th, 10th, 25th, 50th, 75th, 90th, 95th, and 97.5th percentiles by gestational week were significantly higher in t21 fetuses compared to euploid fetuses (p<.001). Overall, 55.8% (48/86) of the t21 fetuses demonstrated at least one Doppler value above the 95th percentile for gestational age based on euploid reference standard. At birth, eight (9.3%) of the t21 fetuses were small for gestational age. When these pregnancies were removed from analysis, UA Doppler indices remained significantly higher than established percentiles at each week of gestation (p < .001). Only three pregnancies ended in fetal demise in the t21 population, two of which had persistently elevated Dopplers above the 95th percentile per established reference percentiles. CONCLUSIONS: At each week of gestation, UA Doppler indices in t21 fetuses were significantly higher than established percentiles from a euploid population. Reference intervals based on euploid fetuses may therefore not be appropriate for antenatal surveillance of fetuses with t21. Prospective studies are needed to investigate the role and impact of serial UA Doppler velocimetry in the surveillance of pregnancies complicated by fetal t21.

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.

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.