European principles of care for women and girls with inherited bleeding disorders.

Publisher: Blackwell Science Country of Publication: England NLM ID: 9442916 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1365-2516 (Electronic) Linking ISSN: 13518216 NLM ISO Abbreviation: Haemophilia Subsets: MEDLINE

Original Publication: Osney Mead, Oxford, UK : Blackwell Science, [1994-

Hemophilia A*/diagnosis ; Hemophilia A*/therapy ; Quality of Life*; Comprehensive Health Care ; Delivery of Health Care ; Europe ; Female ; Humans

Introduction: Despite increasing awareness of issues faced by women and girls with inherited BDs (WGBD), standards of care are lacking, with disparities in diagnosis and treatment for WGBD across Europe. We aimed to develop practical principles of care (PoC) to promote standardization of care for WGBD within European Haemophilia Treatment and Comprehensive Care Centres (HTC/CCCs).
Methods: The co-creation process, supported by the European Association for Haemophilia and Allied Disorders, consisted of four multidisciplinary meetings with health care providers (HCPs) experienced in WGBD care, and European Haemophilia Consortium representatives, combined with broad patient and HCP consultations in the European haemophilia community. Relevant medical societies outside Europe were contacted for confirmation.
Results: We developed ten PoC for WGBD, stressing the importance and benefits of a centralized, multidisciplinary, comprehensive, family-centred approach to support and manage WGBD during all life stages. These PoC emphasise the right to equitable access and quality of care for all people with BDs, irrespective of gender. Multiple medical societies outside Europe also confirmed their support for endorsement.
Conclusions: Ten PoC for WGBD evolved from an iterative process among stakeholders, supported by relevant medical societies worldwide. These PoC can serve as a benchmark for diagnosis and comprehensive multidisciplinary management of WGBD, and improve awareness of their unique challenges. They offer a framework to guide HTC/CCCs in providing equitable care for all WGBD, both in their own services and in other healthcare settings. Implementation of these principles aims to positively impact the health, wellbeing and quality of life for WGBD.
(© 2021 The Authors. Haemophilia published by John Wiley & Sons Ltd.)

Kadir RA, Economides DL, Sabin CA, Owens D, Lee CA. Frequency of inherited bleeding disorders in women with menorrhagia. Lancet. 1998;351:485-489.
Knol HM, Mulder AB, Bogchelman DH, Kluin-Nelemans HC, van der Zee AG, Meijer K. The prevalence of underlying bleeding disorders in patients with heavy menstrual bleeding with and without gynecologic abnormalities. Am J Obstet Gynecol. 2013;209:e1-7.
Srivaths LV, Zhang QC, Byams VR, et al. Differences in bleeding phenotype and provider interventions in postmenarchal adolescents when compared to adult women with bleeding disorders and heavy menstrual bleeding. Haemophilia. 2018;24:63-69.
Weyand AC, James PD. Sexism in the management of bleeding disorders. Res Pract Thromb Haemost. 2021;5:51-54. https://doi.org/10.1002/rth2.12468.
Jacobson AE, Vesely SK, Brien SHO, Campbell J. Patterns of von Willebrand disease screening in girls and adolescents with heavy menstrual bleeding. Obstet Gynecol. 2018;131:1121-1129.
Atiq F, Saes JL, Punt MC, et al. Major differences in clinical presentation, diagnosis and management of men and women with autosomal inherited bleeding disorders. EClinicalMedicine. 2021;32:100726.
Balak DMW, Gouw SC, Plug I, Van der Bom JG. Prenatal diagnosis for haemophilia: a nationwide survey among female carriers in the Netherlands. Haemophilia. 2012;18:584-592. http://doi.org/10.1111/j.1365-2516.2011.02742.x.
Maclean PE, Fijnvandraat K, Beijlevelt M, Peters M. The impact of unaware carriership on the clinical presentation of haemophilia. Haemophilia. 2004;10:560-564.
Nau A, Gillet B, Guillet B, et al. Bleeding complications during pregnancy and delivery in haemophilia carriers and their neonates in Western France: an observational study. Haemophilia. 2020;26:1046-1055. http://doi.org/10.1111/hae.14117.
Punt MC, Waning ML, Mauser-Bunschoten EP, et al. Maternal and neonatal bleeding complications in relation to peripartum management in women with Von Willebrand disease: a systematic review. Blood Rev. 2020;39:100633.
Punt M, Ruigrok ND, Bloemenkamp KWM, Schutgens REG, Kremer Hovinga ICL, van Galen KPM. Obstetrical bleeding in women with MYH9-related disease - A systematic review. Haemophilia. 2020;27:e278-e283. http://doi.org/10.1111/hae.14147.
Punt MC, Schuitema PCE, Bloemenkamp KWM, Hovinga Kremer, I CL, van Galen KPM. Menstrual and obstetrical bleeding in women with inherited platelet receptor defects - A systematic review. Haemophilia. 2020;26:216-227.
Kulkarni R, Presley RJ, Lusher JM, et al. Complications of haemophilia in babies (first two years of life): a report from the centers for disease control and prevention universal data collection system. Haemophilia. 2017;23:207-214.
Plug I, Mauser-Bunschoten EP, Bröcker-Vriends AH, et al. Bleeding in carriers of hemophilia. Blood. 2006;108:52-56.
Paroskie A, Gailani D, DeBaun MR, Sidonio RF. A cross-sectional study of bleeding phenotype in haemophilia A carriers. Br J Haematol. 2015;170:223-228.
Noone D, Skouw-Rasmussen N, Lavin M, van Galen KPM, Kadir RA. Barriers and challenges faced by women with congenital bleeding disorders in Europe: results of a patient survey conducted by the European Haemophilia Consortium. Haemophilia. 2019;25:468-474.
Arya S, Wilton P, Page D, et al. “ Everything was blood when it comes to me ”: understanding the lived experiences of women with inherited bleeding disorders. J Thromb Haemost. 2020;18:3211-3221.
Arya S, Wilton P, Page D, et al. “They don't really take my bleeds seriously”: barriers to care for women with inherited bleeding disorders. J Thromb Haemost. 2021;19:1506-1514.
Mauser-Bunschoten EP, Kadir RA, Laan ETM, et al. Managing women-specific bleeding in inherited bleeding disorders: a multidisciplinary approach. Haemophilia. 2021;27:463-469. http://doi.org/10.1111/hae.14221.
Eising HP, Sanders YV, De Meris J, Leebeek F, Meijer K. Women prefer proactive support from providers for treatment of heavy menstrual bleeding: a qualitative study in adult women with moderate or severe Von Willebrand disease. Haemophilia. 2018;24:950-956.
Punt MC, Aalders TH, Bloemenkamp KWM, et al. The experiences and attitudes of hemophilia carriers around pregnancy: a qualitative systematic review. J Thromb Haemost. 2020;18:1626-1636.
van Galen KPM, Lavin M, Skouw-Rasmussen N, et al. Clinical management of woman with bleeding disorders: a survey among European haemophilia treatment centres. Haemophilia. 2020;26:657-662.
Sidonio RF, Haley KM, Fallaize D. Impact of diagnosis of von Willebrand disease on patient outcomes: analysis of medical insurance claims data. Haemophilia. 2017;23:743-749.
Zia A, Jain S, Kouides P, et al. Bleeding disorders in adolescents with heavy menstrual bleeding in a multicenter prospective US cohort. Haematologica. 2020;105:1969-1976.
van Galen KPM, Sanders YV, Vojinovic U, et al. Joint bleeds in von Willebrand disease patients have significant impact on quality of life and joint integrity: a cross-sectional study. Haemophilia. 2015;21:e185-e192.
Gilbert L, Paroskie A, Gailani D, Debaun M, Sidonio R. Haemophilia A carriers experience reduced health-related quality of life. Haemophilia. 2015;21:761-765.
Olsson A, Hellgren M, Berntorp E, Baghaei F. Association between bleeding tendency and health-related quality of life in carriers of moderate and severe haemophilia. Haemophilia. 2015;21:742-746.
Young JE, Grabell J, Tuttle A, et al. Evaluation of the self-administered bleeding assessment tool (Self-BAT) in haemophilia carriers and correlations with quality of life. Haemophilia. 2017;23:e536-e538. https://doi.org/10.1111/hae.13354.
van Galen K, Key NS, Ljung LR, Manco-Johnson MJ, van den Berg HM, Srivastava A. New Hemophilia Carrier Nomenclature to define hemophilia in women and girls: Communication from the SSC on Factor VIII, Factor IX and rare coagulation disorders and Women's Health issues in Thrombosis and Hemostasis. submitted.
Winikoff R, Lee C. Hemophilia carrier status and counseling the symptomatic and asymptomatic adolescent. J Pediatr Adolesc Gynecol. 2010;23:S43-S47.
Deforest M, Grabell J, Albert S, et al. Generation and optimization of the self-administered bleeding assessment tool and its validation as a screening test for von Willebrand disease. Haemophilia. 2015;21:e384-e388.
Higham J, O'Brien P, Shaw R. Assessment of menstrual blood loss using a pictorial chart. Br J Obs Gynaecol. 1990;97:734-739.
Von Willebrand, E. Hereditar Pseudohemophili. Haemophilia. 1999;5:223-231. http://doi.org/10.1046/j.1365-2516.1.
Hoffman R, Brenner B. Corpus luteum hemorrhage in women with bleeding disorders. Women's Heal. 2009;5:91-95.
Côté I, Jacobs P, Cumming D. Work loss associated with increased menstrual loss in the United States. Obstet Gynecol. 2002;100:683-687.
Frick KD, Clark MA, Steinwachs DM, et al. Financial and quality-of-life burden of dysfunctional uterine bleeding among women agreeing to obtain surgical treatment. Women's Heal. 2009;19:70-78.
Hurskainen R, Aalto AM, Teperi J, et al. Psychosocial and other characteristics of women complaining of menorrhagia, with and without actual increased menstrual blood loss. Br J Obstet Gynaecol. 2001;108:281-285.
von Mackensen S, Myrin Westesson L, Kavakli K, et al. The impact of psychosocial determinants on caregivers’ burden of children with haemophilia (results of the BBC study). Haemophilia. 2019;25:424-432.
Recht M, Neufeld EJ, Sharma VR, et al. Impact of acute bleeding on daily activities of patients with congenital hemophilia with inhibitors and their caregivers and families: observations from the dosing observational study in hemophilia (DOSE). Value Heal. 2014;17:744-748.
Barlow JH, Stapley J, Ellard DR. Living with haemophilia and von Willebrand's: a descriptive qualitative study. Patient Educ Couns. 2007;68:235-242.
Duncan A, Kellum A, Peltier S, Cooper DL, Saad H. Disease burden in patients with glanzmann's thrombasthenia: perspectives from the glanzmann's thrombasthenia patient/caregiver questionnaire. J Blood Med. 2020;11:289-295.
VanderMeulen H, Petrucci J, Floros G, Meffe F, Dainty KN, Sholzberg M. The experience of postpartum bleeding in women with inherited bleeding disorders. Res Pract Thromb Haemost. 2019;3:733-740.
Schoep ME, Nieboer TE, van der Zanden M, Braat DDM, Nap AW. The impact of menstrual symptoms on everyday life: a survey among 42,879 women. Am J Obstet Gynecol. 2019;220:569.e1-569.e7.
Breakey VR, Blanchette VS, Bolton-Maggs PHB. Towards comprehensive care in transition for young people with haemophilia. Haemophilia. 2010;16:848-857.
d'Oiron R, O'Brien S, James AH. Women and girls with haemophilia: Lessons learned. Haemophilia. 2021;27(S3):75-81. https://doi.org/10.1111/hae.14094.
Gillham A, Greyling B, Wessels TM, et al. Uptake of genetic counseling, knowledge of bleeding risks and psychosocial impact in a south african cohort of female relatives of people with hemophilia. J Genet Couns. 2015;24:978-986.
Bernard W, Lambert C, Henrard S, Hermans C. Screening of haemophilia carriers in moderate and severe haemophilia A and B: prevalence and determinants. Haemophilia. 2018;24:e142-e144.
Miller R. Counselling about diagnosis and inheritance of genetic bleeding disorders: haemophilia A and B. Haemophilia. 1999;5(2):77-83. https://doi.org/10.1046/j.1365-2516.1999.00288.x.
Shah LL, Daack-Hirsch S, Ersig AL, Paik A, Ahmad F, Williams J. Family relationships associated with communication and testing for inherited cardiac conditions. West J Nurs Res. 2019;41:1576-1601.
Thomas S, Herbert D, Street A, Barnes C, Boal J, Komesaroff P. Attitudes towards and beliefs about genetic testing in the haemophilia community: a qualitative study. Haemophilia. 2007;13(5):633-641. https://doi.org/10.1111/j.1365-2516.2007.01454.x.
Boardman, FK, Hale, R, Gohel, R, Young, PJ. Preventing lives affected by hemophilia: a mixed methods study of the views of adults with hemophilia and their families toward genetic screening. Mol Genet Genomic Med. 2019;7:1-19.
Leuzinger-Bohleber M, Teising M. ‘Without being in psychoanalysis I would never have dared to become pregnant’: psychoanalytical observations in a multidisciplinary study concerning a woman undergoing prenatal diagnostics. Int J Psychoanal. 2012;93:293-315.
Colvin BT, Astermark J, Fischer K, et al. European principles of haemophilia care. Haemophilia. 2008;14:361-374.
Lee CA, Chi C, Shiltagh N, et al. Review of a multidisciplinary clinic for women with inherited bleeding disorders. Haemophilia. 2009;15:359-360.
Kadir RA, Davies J, Winikoff R, et al. Pregnancy complications and obstetric care in women with inherited bleeding disorders. Haemophilia. 2013;19(4):1-10.
Kouides PA, Phatak PD, Burkart P, et al. Gynaecological and obstetrical morbidity in women with type I von Willebrand disease: results of a patient survey. Haemophilia. 2000;6:643-648.
Kadir RA, Edlund M, Von Mackensen S. The impact of menstrual disorders on quality of life in women with inherited bleeding disorders. Heamophilia. 2010;16:832-839.
Marret H, Fauconnier A, Chabbert-Buffet N, et al. Clinical practice guidelines on menorrhagia: management of abnormal uterine bleeding before menopause. Eur J Obstet Gynecol Reprod Biol. 2010;152:133-137.
Magnay JL, O'Brien S, Gerlinger C, Seitz C. A systematic review of methods to measure menstrual blood loss. BMC Womens Health. 2018;18:1-13.
Breymann C, Auerbach M. Iron deficiency in gynecology and obstetrics: clinical implications and management. Hematol Am Soc Hematol Educ Progr. 2017:152-159.
Mirza FG, Abdul-Kadir R, Breymann C, Fraser IS, Taher A. Impact and management of iron deficiency and iron deficiency anemia in women's health. Expert Rev Hematol. 2018;11:727-736.
Davies J, Kadir RA. Heavy menstrual bleeding: an update on management. Thromb Res. 2017;151:S70-S77.
Parks MA, Zwayne N, Temkit M. Bleeding patterns among adolescents using the levonorgestrel intrauterine device: a single institution review. J Pediatr Adolesc Gynecol. 2020;33:555-558.
Michaelides K, Tuddenham E, Turner C, Lavender B, Lavery S. Live birth following the first mutation specific pre-implantation genetic diagnosis for haemophilia A. Thromb Haemost. 2006;95:373-279.
Peyvandi F, Garagiola I, Mortarino M. Prenatal diagnosis and preimplantation genetic diagnosis: novel technologies and state of the art of PGD in different regions of the world. Haemophilia. 2011;17:14-17.
Salomon LJ, Sotiriadis A, Wulff CB, Odibo A, Akolekar R. Risk of miscarriage following amniocentesis or chorionic villus sampling: systematic review of literature and updated meta-analysis. Ultrasound Obstet Gynecol. 2019;54:442-451.
Alfirevic Z, Navaratnam K, Mujezinovic F. Amniocentesis and chorionic villus sampling for prenatal diagnosis. Cochrane Database Syst Rev. 2017. https://doi.org/10.1002/14651858.cd003252.pub2.
Tsui NBY, Kadir RA, Chan KC, et al. Noninvasive prenatal diagnosis of hemophilia by microfluidics digital PCR analysis of maternal plasma DNA. Blood. 2011;117:3684-3691.
Vermeulen C, Geeven G, de Wit E, et al. Sensitive monogenic noninvasive prenatal diagnosis by targeted haplotyping. Am J Hum Genet. 2017;101:326-339.
Cutler J, Chappell LC, Kyle P, Madan B. Third trimester amniocentesis for diagnosis of inherited bleeding disorders prior to delivery. Haemophilia. 2013;19:904-907.
Punt MC, Waning ML, Mauser-Bunschoten EP, et al. Maternal and neonatal bleeding complications in relation to peripartum management in hemophilia carriers: a systematic review. Blood Rev. 2021;100826.
Hawke L, Grabell J, Sim W, et al. Obstetric bleeding among women with inherited bleeding disorders: a retrospective study. Haemophilia. 2016;22:906-911.
Sentilhes L, Gromez A, Clavier E, Resch B, Descamps P, Marpeau L. Long-term psychological impact of severe postpartum hemorrhage. Acta Obstet Gynecol Scand. 2011;90:615-620.
Thompson JF, Ford JB, Raynes-Greenow CH, Roberts CL, Ellwood DA. Women's experiences of care and their concerns and needs following a significant primary postpartum hemorrhage. Birth. 2011;38:327-335.
Gottvall K, Waldenström U. Does a traumatic birth experience have an impact on future reproduction?. BJOG An Int J Obstet Gynaecol. 2002;109:254-260.
Huq FY, Kadir RA. Management of pregnancy, labour and delivery in women with inherited bleeding disorders. Haemophilia. 2011;17(1):20-30.
Leebeek FWG, Duvekot J, Kruip MJH. A. How I manage pregnancy in carriers of hemophilia and patients with von Willebrand disease. Blood. 2020;136:2143-2150.
Kouides P. Present day management of inherited bleeding disorders in pregnancy. Expert Rev Hematol. 2016;9:987-995.
Shakur H, et al. Effect of early tranexamic acid administration on mortality, hysterectomy, and other morbidities in women with post-partum haemorrhage (WOMAN): an international, randomised, double-blind, placebo-controlled trial. Lancet. 2017;389:2105-2116.
Saccone G, Della Corte L, D’Alessandro P, et al. Prophylactic use of tranexamic acid after vaginal delivery reduces the risk of primary postpartum hemorrhage. J Matern-Fetal Neonatal Med. 2020;33(19):3368-3376. https://doi.org/10.1080/14767058.2019.1571576.
Gilad O, Merlob P, Stahl B, Klinger G. Outcome following tranexamic acid exposure during breastfeeding. Breastfeed Med. 2014;9:407-410.
Byams VR, Beckman MG, Grant AM, Parker CS. Developing a public health research agenda for women with blood disorders. J women's Heal. 2010;19:1231-1234.
Soldin OP, Mattison DR. Sex differences in pharmacokinetics and pharmacodynamics. Clin Pharmacokinet. 2009;48:143-157.

Keywords: bleeding disorder; heavy menstrual bleeding; postpartum haemorrhage and reproduction; pregnancy; principles of care; women

Date Created: 20210803 Date Completed: 20210924 Latest Revision: 20210924

20221213

10.1111/hae.14379

34343384