The ISTH-BAT score and outcomes after endometrial ablation in women with heavy menstrual bleeding.

BACKGROUND: The International Society on Thrombosis and Haemostasis bleeding assessment tool (ISTH-BAT), is used during the diagnostic workup of bleeding disorders. Data on ISTH-BAT scores in women with heavy menstrual bleeding (HMB) undergoing endometrial ablation (EA) could be essential in optimizing HMB counselling. OBJECTIVE: To investigate the postsurgical incidence of amenorrhea, dysmenorrhea, quality of life, re-intervention after EA, and ISTH-BAT score. METHODS: This study included women who have undergone EA because of HMB. During a follow-up of 2 to 5 years, ISTH-BAT, pictorial blood assessment chart (PBAC), and Short Form-36 survey (SF-36) were administered. At 10 years of follow-up surgical re-interventions were evaluated. RESULTS: Seventy-one women were included of whom 77% (n = 55) had an ISTH-BAT score < 6, versus 23% (n = 16) ISTH-BAT score ≥6 (mean age 46.3 versus 42.3, p = 0.004). In the ISTH-BAT ≥6 group versus < 6 group, amenorrhea occurred in 63% (10/16) versus 82% (45/55) (p = 0.111), dysmenorrhea in 38% (6/16) versus 18% (10/55) (p = 0.111), and surgical re-intervention in 19% (3/16) versus 25% (14/55) (p = 0.582). SF-36 item (Bodily) pain was lower in the ISTH-BAT ≥6 group versus < 6 (median score 58.7 vs. 80.0, p = 0.104). CONCLUSIONS: An ISTH-BAT score ≥6 may be related to a lower amenorrhea incidence and higher dysmenorrhea rate after EA.

Menstrual problems in chronic immune thrombocytopenia: A monthly challenge - a cohort study and review.

Immune thrombocytopenia (ITP) may cause menstrual problems. This cross-sectional study assessed menstrual problems in premenopausal chronic ITP women by several questionnaires, including the pictorial bleeding assessment calendar (PBAC; score ≥100 indicates heavy menstrual bleeding [HMB]), and the menorrhagia multiattribute scale (MMAS). Spearman was used for assessing correlations. A literature review was performed in Pubmed. The cohort comprised 37 women (mean age 31 ± 9). A total of 29/37 (78%) had experienced clinical menstrual problems in the present or past. Of the 33 patients who returned the PBAC, 13 (39%) had a score of ≥100. The median MMAS score was 79 (IQR 60-95). The PBAC scores correlated with the MMAS. Both questionnaires were unrelated to the platelet count. Patients with a levonorgestrel intrauterine device (LNG-IUD) had lower PBAC scores than patients with other or no hormonal therapy. MMAS scores were correlated with fatigue. The review identified 14 papers. HMB occurred in 6%-55% at ITP diagnosis and 17%-79% during disease. Menstrual symptoms influenced the quality of life, particularly in patients with a low platelet count. This explorative study suggested that HMB is frequent in women with chronic ITP despite management and platelet counts >50 *109 /l. An LNG-IUD seemed to reduce blood loss significantly.

Prevalence, burden and treatment effects of vaginal bleeding in women with (suspected) congenital platelet disorders throughout life: a cross-sectional study.

Congenital platelet disorders (CPDs) are rare bleeding disorders that are associated with mucocutaneous bleeds. However, data on vaginal bleeding in women with CPDs are scarce. A set of generic and bleeding-specific questionnaires were used to evaluate the prevalence of vaginal bleeding, its impact on quality of life (QoL) and sexual functioning and the consequences for pregnancy, miscarriage and delivery in a cohort of women who were referred for diagnostic evaluation for CPDs. A total of 78 women included in the study were either diagnosed with a CPD (n = 35) or were clinically suspected of a CPD (n = 43). Heavy menstrual bleeding (HMB) was reported by a large proportion of women, which mainly started at menarche. In all, 76% of women received any kind of HMB treatment, often leading to surgical prodecures. HMB was shown to have a high impact on QoL, which improved upon treatment. Even though women reported that vaginal bleeding affects sexuality, this topic is not frequently discussed with physicians. Heavy blood loss frequently occurred after miscarriage/delivery, often requiring treatment. Women with (suspected) CPDs frequently encounter HMB, negatively impacting daily life and sexual functioning. Together with peripartum bleeding, these data highlight the burden of vaginal bleeding in CPDs and importance of adequate treatment.

A qualitative study on the experiences of haemophilia carriers before, during and after pregnancy.

INTRODUCTION: Haemophilia carriers (HCs) face considerable haemostatic and psychological challenges during reproduction. AIM: To explore the perspectives of HCs on healthcare in the current standard of haemophilia treatment during all reproductive phases: preconception, pregnancy, childbirth and the postpartum period. In addition, we examined the psychological impact of haemophilia during these phases. MATERIAL AND METHODS: Focus group discussions (FGDs) and semi-structured interviews were conducted with HCs in January/February 2020 until data saturation was reached. All sessions were recorded, transcribed verbatim and analysed by two independent researchers through thematic content analysis using MAXQDA® software. The results were then discussed within the research team until consensus was reached. The constructed themes were shared with and reviewed by the HCs. RESULTS: Fifteen HCs were included in three FGDs and four interviews. Five central themes were constructed: (1) communication by healthcare professionals, (2) lack of knowledge, (3) feeling insecure, (4) autonomy and (5) family experiences with haemophilia. Desired improvements in care mainly concerned counselling during preconception and pregnancy. This included timely access to comprehensive information during each consecutive phase, acceptance of HCs' choices by healthcare providers and healthcare tailored to the HC's family experience with haemophilia. CONCLUSIONS: In recent years, haemophilia treatment has seen major advances, which could impact general and reproductive care for HCs. HCs indicated that reproductive care would benefit from a more personal and informative approach. Healthcare professionals could use these insights to adapt their consultations to meet the needs of these women when they are preparing for having children.

Menstrual and obstetrical bleeding in women with inherited platelet receptor defects-A systematic review.

INTRODUCTION: Women with inherited platelet receptor defects (IPRD) may have an increased risk of heavy menstrual bleeding (HMB) and postpartum haemorrhage (PPH). AIM: To present a systematic overview of the literature on the prevalence and management of menstrual and obstetrical bleeding in women with IPRD. METHODS: Electronic databases were searched for original patient data on the prevalence and management of HMB and PPH in women with known IPRD or who were being investigated for IPRD. RESULTS: Sixty-nine papers (61 case reports/series and 8 cohort studies) were included. Overall, studies were rated as 'poor quality'. The included cohort studies reported HMB in 25% (13/52) of women with Bernard-Soulier syndrome and in 22.1% (34/154) of women with Glanzmann thrombasthenia. In total, 164 deliveries in women with IPRD were described. Excessive bleeding occurred in 16.9% (11/65) of deliveries described in the largest cohort. PPH occurred in 63.2% (55/87) of deliveries described in case reports/series. PPH occurred in 73.7% (14/19) of deliveries that were not covered by prophylaxis compared with 54.2% (32/59) of deliveries that were (OR = 2.36, 95% CI 0.75-7.40). Neonatal bleeding complications were reported in 10.0% (8/80) of deliveries. In all (6/6) deliveries with neonatal bleeding complications wherein the presence of alloantibodies was investigated, either antiplatelet or anti-HLA antibodies were detected. DISCUSSION/CONCLUSION: Menstrual and particularly obstetrical bleeding problems frequently occur in women with IPRD, based on small case reports and series of poor quality. International collaboration, preferably on prospective studies, is needed to improve clinical management of women-specific bleeding in IPRD.

Clinical management of woman with bleeding disorders: A survey among European haemophilia treatment centres.

INTRODUCTION: The impact of bleeding for women with bleeding disorders (WBD) is of increasing focus and importance. Despite this, optimal management strategies are unclear and knowledge gaps persist. AIM: To examine practices and define research priorities on diagnosis and management of WBD in Europe. METHODS: An electronic survey on clinical management of WBD was sent to 136 European haemophilia treatment centres (HTCs), including open questions on knowledge gaps and research priorities. RESULTS: Fifty-nine HTCs from 12 Western (WE) and 13 Central/Eastern European (CEE) countries completed the survey. Less than half runs a joint clinic (24 HTCs, 42%). Most centres without a joint clinic have a named obstetrician (81%) and/or gynaecologist (75%) available for collaboration. Overall 18/54 (33%) European HTCs do not offer preimplantation genetic diagnosis. Third trimester amniocentesis to guide obstetric management is available 28/54 HTCs (52%), less frequent in CEE compared to WE countries (5/17 vs 23/37, P = .03). 53% of HTCs (28/53) reported that only 0%-25% of WBD seek medical advice for heavy menstrual bleeding (HMB). An algorithm managing acute HMB in WBD is lacking in 22/53 (42%) HTCs. The main reported knowledge and research gaps are lack of awareness & education on WBD among patients and caregivers, optimal diagnostic strategies and effective multidisciplinary management of pregnancy & HMB. CONCLUSION: Joint clinics, prenatal diagnostics and algorithms for managing acute HMB are lacking in many European HTCs. HMB may be an underestimated issue. This survey highlights the need to prioritize improvement of knowledge and patient care for WBD across Europe.

Quality of intrapartum care: direct observations in a low-resource tertiary hospital.

BACKGROUND: The majority of the world's perinatal deaths occur in low- and middle-income countries. A substantial proportion occurs intrapartum and is avoidable with better care. At a low-resource tertiary hospital, this study assessed the quality of intrapartum care and adherence to locally-tailored clinical guidelines. METHODS: A non-participatory, structured, direct observation study was held at Mnazi Mmoja Hospital, Zanzibar, Tanzania, between October and November 2016. Women in active labour were followed and structure, processes of labour care and outcomes of care systematically recorded. Descriptive analyses were performed on the labour observations and compared to local guidelines and supplemented by qualitative findings. A Poisson regression analysis assessed factors affecting foetal heart rate monitoring (FHRM) guidelines adherence. RESULTS: 161 labouring women were observed. The nurse/midwife-to-labouring-women ratio of 1:4, resulted in doctors providing a significant part of intrapartum monitoring. Care during labour and two-thirds of deliveries was provided in a one-room labour ward with shared beds. Screening for privacy and communication of examination findings were done in 50 and 34%, respectively. For the majority, there was delayed recognition of labour progress and insufficient support in second stage of labour. While FHRM was generally performed suboptimally with a median interval of 105 (interquartile range 57-160) minutes, occurrence of an intrapartum risk event (non-reassuring FHR, oxytocin use or poor progress) increased assessment frequency significantly (rate ratio 1.32 (CI 1.09-1.58)). CONCLUSIONS: Neither international nor locally-adapted standards of intrapartum routine care were optimally achieved. This was most likely due to a grossly inadequate capacity of birth attendants; without whom innovative interventions at birth are unlikely to succeed. This calls for international and local stakeholders to address the root causes of unsafe intrafacility care in low-resource settings, including the number of skilled birth attendants required for safe and respectful births.

Primary postpartum hemorrhage in women with von Willebrand disease and carriers of hemophilia: a retrospective analysis.

Background: Between 2002 and 2011, the incidence of severe primary postpartum hemorrhage (PPH) in Dutch women with von Willebrand disease (VWD) and hemophilia carriers (HCs) was 8% vs 4.5% in the general population. Objectives: To determine the contemporary incidence of severe primary PPH in women with VWD and HCs. Methods: All women with VWD or HCs who delivered between 2012 and 2017 were selected from all 6 Dutch hemophilia treatment centers. Data on patient and disease characteristics, peripartum hematologic and obstetric management, and outcomes were retrospectively collected. Incidence of severe primary (≥1000 mL of blood loss ≤24 hours after childbirth) and primary (≥500 mL within ≤24 hours after childbirth) PPH was compared with the (1) previous cohort and (2) general Dutch population and between (3) women with VWD and HCs with third-trimester coagulation activity levels <50 international units (IU)/dL vs ≥50 IU/dL and (4) women treated with vs without peripartum hemostatic prophylaxis. Results: Three-hundred forty-eight deliveries (151 VWD, 167 hemophilia A, and 30 hemophilia B carriers) were included. The severe primary PPH incidence was 10% (36/348) and remained stable over time, whereas this incidence has increased in the general population (to 8%), leading to a similar risk (P = .17). Severe primary PPH risk was comparable between women with coagulation activity levels <50 and ≥50 IU/dL (11% [7/66] vs 10% [29/279]; odds ratio, 1.02; 95% CI, 0.43-2.44) and comparable between those with and those without prophylaxis (12% [11/91] vs 10% [25/254]; odds ratio, 1.26; 95% CI, 0.59-2.68). Conclusion: Severe primary PPH in women with VWD and HCs remained stable and is comparable with the increasing prevalence in the general population. More research is needed to find the optimal pregnancy management strategy for safe delivery in VWD and HC.

Platelet count and indices as postpartum hemorrhage risk factors: a retrospective cohort study.

BACKGROUND: Severe postpartum hemorrhage (SPPH) is the leading cause of maternal mortality and morbidity worldwide. Platelet anomalies frequently occur during pregnancy. However, their role in the etiology of SPPH is largely unknown. OBJECTIVE: To study the relation between platelet parameters and SPPH. METHODS: This retrospective single-center cohort included deliveries between 2009 and 2017. SPPH was defined as ≥1000 ml blood loss within 24 h after delivery. Platelet parameters were measured within 72 h before delivery. Multiple imputation was performed for missing data. Odds ratios were adjusted (aORs) for maternal age, multiple gestation, macrosomia, induction of labor, preeclampsia, and hemolysis, elevated liver enzymes, and low platelets syndrome. RESULTS: A total of 23 205 deliveries were included. Of the 2402 (10.4%) women with thrombocytopenia (<150 × 109 /L), 10.3% developed SPPH, compared with 7.6% of women with a normal platelet count (aOR: 1.34, 95% CI: 1.14-1.59). Women with a platelet count of <50 × 109 /L were most at risk (aOR of 2.24 [1.01-4.94]) compared with the reference group with normal platelet counts; the aOR was 1.22 (0.77-1.93) for the 50-99 × 109 /L platelet count group and 1.31 (1.10-1.56) for the 100-149 × 109 /L platelet count group. Plateletcrit was associated with SPPH (aOR 1.15 [1.08-1.21] per 0.05% decrease), and, although rarely present, a platelet distribution width (PDW) ≥23% (n = 22) also increased the odds of SPPH (aOR 6.05 [2.29-16.20]). CONCLUSION: Different degrees of thrombocytopenia were independently associated with the occurrence of SPPH. Despite their relation to SPPH, plateletcrit and a PDW of ≥23% have limited additional value in addition to platelet count.

The experiences and attitudes of hemophilia carriers around pregnancy: A qualitative systematic review.

BACKGROUND: Hemophilia carriers (HCs) face specific psychosocial challenges related to pregnancy, caused by their inherited bleeding disorder. Optimal support from healthcare providers can only be realized by exploring medical and psychological healthcare requirements. OBJECTIVE: To review all published evidence on the experiences and attitudes of HCs regarding reproductive decision-making, prenatal diagnosis, pregnancy, childbirth, and puerperium to provide an accessible overview of this information for health care providers. STUDY SELECTION: Cochrane library, PubMed/MEDLINE, EMBASE, CINAHL, and PsycINFO were searched for original qualitative data. Two authors performed study selection, risk-of-bias assessment, data extraction, and data analysis through meta-summary. The extracted themes were discussed within the research team. FINDINGS: Fifteen studies with an overall moderate quality were included. The following findings were identified: (a) Quality of life of family members with hemophilia influences reproductive decision-making; (b) Genetic counselling is generally considered useful; (c) The development of a specialized carrier clinic is considered valuable; (d) HCs describe prenatal diagnosis as beneficial yet psychosocially challenging; and (e) noninvasive prenatal diagnosis and preimplantation genetic diagnosis are predominantly considered beneficial. These findings are limited by the overall moderate quality of included studies and the possibly partly outdated results in the current era of hemophilia treatment. CONCLUSIONS: Available qualitative literature on HCs around pregnancy focuses on genetic counselling and prenatal diagnosis. Future studies are needed on the experiences and needs of HCs through pregnancy and puerperium as well as in light of emerging hemophilia diagnosis and treatment options.

Reliability and Feasibility of the Self-Administered ISTH-Bleeding Assessment Tool.

Introduction Standardized bleeding assessment tools (BATs), such as the International Society for Thrombosis and Hemostasis (ISTH)-BAT, are screening instruments used during the diagnostic workup of suspected bleeding disorders. A self-administered ISTH-BAT (self-BAT) would enhance screening and save time during an outpatient clinic visit. Aim This study was aimed to investigate the reliability and feasibility of the self-BAT. Methods The electronic self-BAT was created from the ISTH-BAT and paper-version of self-BAT and optimized by patients and physicians. Patients with a (suspected) congenital platelet defect (CPD), who had previously undergone physician-administered ISTH-BAT assessment, were invited to complete the self-BAT. Optimal self-BAT cut-off values to detect a bleeding tendency, as defined by the ISTH-BAT, were evaluated by receiver operator characteristic (ROC) curve analysis to reach a sensitivity ≥95%. Reliability was tested by assessing sensitivity, specificity, and intraclass correlation (ICC). Feasibility was evaluated on comprehension and length of self-BAT. Results Both versions of the BAT were completed by 156 patients. Optimal cut-off values for self-BAT to define a bleeding tendency were found to be identical to those of the ISTH-BAT. Normal/abnormal scores of the ISTH-BAT and self-BAT were agreed in 88.5% (138/156, 95% confidence interval [CI]: 0.83-0.93) of patients. The sensitivity and specificity of the self-BAT to detect a bleeding tendency were 96.9 and 48.1%, respectively. The ICC was 0.73. Self-BAT questions were graded by 96.8% (151/156) as "very easy," "easy," and "satisfactory" and questionnaire length as "exactly right" by 91% (142/156) of patients. Conclusion In patients with a (suspected) CPD, the self-BAT is sufficiently reliable and feasible to detect a bleeding tendency, which supports its use as a screening tool.

Delphi consensus statement on intrapartum fetal monitoring in low-resource settings.

OBJECTIVE: To determine acceptable and achievable strategies of intrapartum fetal monitoring in busy low-resource settings. METHODS: Three rounds of online Delphi surveys were conducted between January 1 and October 31, 2017. International experts with experience in low-resource settings scored the importance of intrapartum fetal monitoring methods. RESULTS: 71 experts completed all three rounds (28 midwives, 43 obstetricians). Consensus was reached on (1) need for an admission test, (2) handheld Doppler for intrapartum fetal monitoring, (3) intermittent auscultation (IA) every 30 minutes for low-risk pregnancies during the first stage of labor and after every contraction for high-risk pregnancies in the second stage, (4) contraction monitoring hourly for low-risk pregnancies in the first stage, and (5) adjunctive tests. Consensus was not reached on frequency of IA or contraction monitoring for high-risk women in the first stage or low-risk women in the second stage of labor. CONCLUSION: There is a gap between international recommendations and what is physically possible in many labor wards in low-resource settings. Research on how to effectively implement the consensus on fetal assessment at admission and use of handheld Doppler during labor and delivery is crucial to support staff in achieving the best possible care in low-resource settings.

Strategies for intrapartum foetal surveillance in low- and middle-income countries: A systematic review.

BACKGROUND: The majority of the five million perinatal deaths worldwide take place in low-resource settings. In contrast to high-resource settings, almost 50% of stillbirths occur intrapartum. The aim of this study was to synthesise available evidence of strategies for foetal surveillance in low-resource settings and associated neonatal and maternal outcomes, including barriers to their implementation. METHODS AND FINDINGS: The review was registered with Prospero (CRD42016038679). Five databases were searched up to May 1st, 2016 for studies related to intrapartum foetal monitoring strategies and neonatal outcomes in low-resource settings. Two authors extracted data and assessed the risk of bias for each study. The outcomes were narratively synthesised. Strengths, weaknesses, opportunities and threats analysis (SWOT) was conducted for each monitoring technique to analyse their implementation. There were 37 studies included: five intervention and 32 observational studies. Use of the partograph improved perinatal outcomes. Intermittent auscultation with Pinard was associated with lowest rates of caesarean sections (10-15%) but with comparable perinatal outcomes to hand-held Doppler and Cardiotocography (CTG). CTG was associated with the highest rates of caesarean sections (28-34%) without proven benefits for perinatal outcome. Several tests on admission (admission tests) and adjunctive tests including foetal stimulation tests improved the accuracy of foetal heart rate monitoring in predicting adverse perinatal outcomes. CONCLUSIONS: From the available evidence, the partograph is associated with improved perinatal outcomes and is recommended for use with intermittent auscultation for intrapartum monitoring in low resource settings. CTG is associated with higher caesarean section rates without proven benefits for perinatal outcomes, and should not be recommended in low-resource settings. High-quality evidence considering implementation barriers and enablers is needed to determine the optimal foetal monitoring strategy in low-resource settings.