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Health issues in women and girls affected by haemophilia with a focus on nomenclature, heavy menstrual bleeding, and musculoskeletal issues
dc.contributor.authorWeyand, Angela C.
dc.contributor.authorSidonio, Robert F.
dc.contributor.authorSholzberg, Michelle
dc.date.accessioned2022-06-01T20:30:34Z
dc.date.available2023-06-01 16:30:33en
dc.date.available2022-06-01T20:30:34Z
dc.date.issued2022-05
dc.identifier.citationWeyand, Angela C.; Sidonio, Robert F.; Sholzberg, Michelle (2022). "Health issues in women and girls affected by haemophilia with a focus on nomenclature, heavy menstrual bleeding, and musculoskeletal issues." Haemophilia 28: 18-25.
dc.identifier.issn1351-8216
dc.identifier.issn1365-2516
dc.identifier.urihttps://hdl.handle.net/2027.42/172841
dc.description.abstractIntroductionWomen and girls affected by haemophilia, including haemophilia carriers (WGH) are at risk of bleeding symptoms that may go unrecognized, including heavy menstrual bleeding (HMB) and musculoskeletal bleeding. Terminology continues to evolve.AimTo describe the current recommendations for nomenclature surrounding WGH, and the current understanding of HMB, iron deficiency, and musculoskeletal complaints in these patients.MethodsLiterature was reviewed and summarized.ResultsWith regards to nomenclature, women with factor levels less than 50% should be classified as having haemophilia, while carriers with normal levels should be characterized accordingly to symptomatology. HMB and resultant iron deficiency are common among WGH, have a multitude of downstream effects, and maybe overlooked due to stigma around menstruation. Musculoskeletal bleeding and resultant joint changes are increasingly recognized in this population but do not necessarily correlate with factor levels.ConclusionAlthough progress has been made in the care of WGH, much work remains to further improve their care.
dc.publisherWiley Periodicals, Inc.
dc.publisherAll About Hemophilia
dc.subject.otherhemarthrosis
dc.subject.otherbleeding
dc.subject.otherhemophilia
dc.subject.othermenstruation
dc.subject.othernomenclature
dc.subject.othervon willebrand disease
dc.subject.otherwomen’s health
dc.titleHealth issues in women and girls affected by haemophilia with a focus on nomenclature, heavy menstrual bleeding, and musculoskeletal issues
dc.typeArticle
dc.rights.robotsIndexNoFollow
dc.subject.hlbsecondlevelOncology and Hematology
dc.subject.hlbtoplevelHealth Sciences
dc.description.peerreviewedPeer Reviewed
dc.description.bitstreamurlhttp://deepblue.lib.umich.edu/bitstream/2027.42/172841/1/hae14535.pdf
dc.description.bitstreamurlhttp://deepblue.lib.umich.edu/bitstream/2027.42/172841/2/hae14535_am.pdf
dc.identifier.doi10.1111/hae.14535
dc.identifier.sourceHaemophilia
dc.identifier.citedreferenceKouides PA, Byams VR, Philipp CS, et al. Multisite management study of menorrhagia with abnormal laboratory haemostasis: a prospective crossover study of intranasal desmopressin and oral tranexamic acid. Br J Haematol. 2009; 145 ( 2 ): 212 - 220.
dc.identifier.citedreferenceChaudhury A, Sidonio R Jr, Jain N, et al. Women and girls with haemophilia and bleeding tendencies: outcomes related to menstruation, pregnancy, surgery and other bleeding episodes from a retrospective chart review. Haemophilia. 2021; 27 ( 2 ): 293 - 304.
dc.identifier.citedreferenceAmesse LS, Pfaff-Amesse T, Leonardi R, Uddin D, French JA 2nd. Oral contraceptives and DDAVP nasal spray: patterns of use in managing vWD-associated menorrhagia: a single-institution study. J Pediatr Hematol Oncol. 2005; 27 ( 7 ): 357 - 363.
dc.identifier.citedreferenceGill JC, Castaman G, Windyga J, et al. Hemostatic efficacy, safety, and pharmacokinetics of a recombinant von Willebrand factor in severe von Willebrand disease. Blood. 2015 Oct 22; 126 ( 17 ): 2038 - 2046.
dc.identifier.citedreferenceKatayama H, Nagao A, Hosokai R, Suzuki T. [ Successful long-term management of ovarian bleeding and menorrhagia with prothrombin complex concentrate in a patient with congenital factor X deficiency ]. Rinsho Ketsueki. 2018; 59 ( 11 ): 2428 - 2431. Japanese.
dc.identifier.citedreferenceNapolitano M, Di Minno MN, Batorova A, et al. Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies. Haemophilia. 2016; 22 ( 5 ): 752 - 759.
dc.identifier.citedreferencePark YH, Lim JH, Yi HG, Lee MH, Kim CS. Factor V Deficiency in Korean Patients: clinical and laboratory features, treatment, and outcome. J Korean Med Sci. 2016; 31 ( 2 ): 208 - 213
dc.identifier.citedreferenceRugeri L, Martinaud C, Beurrier P, et al. Gynecological and obstetric outcome in the French cohort of women with factor XIII deficiency. Thromb Res. 2020; 191: 22 - 25
dc.identifier.citedreferenceRaso S, Lambert C, Boban A, Napolitano M, Siragusa S, Hermans C. Can we compare haemophilia carriers with clotting factor deficiency to male patients with mild haemophilia? Haemophilia. 2020; 26 ( 1 ): 117 - 121.
dc.identifier.citedreferenceCanadian Hemophilia Society. Symptomatic Carriers of Hemophilia. All About Hemophilia. 2010;
dc.identifier.citedreferenceGilbert L, Paroskie A, Gailani D, Debaun MR, Sidonio RF. Haemophilia A carriers experience reduced health-related quality of life. Haemophilia. 2015; 21 ( 6 ): 761 - 765.
dc.identifier.citedreferenceBryant P, Boukouvala A, McDaniel J, Nance D. Hemophilia A in females: considerations for clinical management. AHA. 2020; 143 ( 3 ): 289 – 94.
dc.identifier.citedreferenceKhair K, Holland M, Pollard D. The experience of girls and young women with inherited bleeding disorders. Haemophilia. 2013; 19 ( 5 ): e276 - 281.
dc.identifier.citedreferenceMauser-Bunschoten EP. Symptomatic carriers of hemophilia. Treatment of Hemophilia [Internet]. 2008. Available from: https://www1.wfh.org/publication/files/pdf-1202.pdf
dc.identifier.citedreferenceDi Michele DM, Gibb C, Lefkowitz JM, Ni Q, Gerber LM, Ganguly A. Severe and moderate haemophilia A and B in US females. Haemophilia. 2014; 20 ( 2 ): e136 - 143.
dc.identifier.citedreferenceWorld Federation of Hemophilia. Carriers and women with hemophilia [Internet]. 2012. Available from: https://www.wfh.org/en/abd/carriers/carriers-and-females-with-hemophilia-en.
dc.identifier.citedreferenceSrivastava A, Brewer AK, Mauser-Bunschoten EP, et al. Guidelines for the management of hemophilia. Haemophilia. 2013; 19 ( 1 ): e1 - 47.
dc.identifier.citedreferenceCarcao MD, van den Berg HM, Ljung R, et al. Correlation between phenotype and genotype in a large unselected cohort of children with severe hemophilia A. Blood. 2013; 121 ( 19 ): 3946 - 3952.
dc.identifier.citedreferenceMiesbach W, Alesci S, Geisen C, Oldenburg J. Association between phenotype and genotype in carriers of haemophilia A. Haemophilia. 2011; 17 ( 2 ): 246 - 251.
dc.identifier.citedreferenceParoskie A, Oso O, Almassi B, DeBaun MR, Sidonio RF. Both hemophilia health care providers and hemophilia a carriers report that carriers have excessive bleeding. J Pediatr Hematol Oncol. 2014; 36 ( 4 ): e224 - e230.
dc.identifier.citedreferenceOlsson 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 ( 6 ): 742 – 746.
dc.identifier.citedreferenceWells AJ, Mclaughlin P, Simmonds JV, et al. A case-control study assessing bone mineral density in severe haemophilia A in the UK. Haemophilia. 2015; 21 ( 1 ): 109 - 115.
dc.identifier.citedreferenceIorio A, Fabbriciani G, Marcucci M, Brozzetti M, Filipponi P. Bone mineral density in haemophilia patients: a meta-analysis. Thromb Haemost. 2010; 103 ( 3 ): 596 - 603.
dc.identifier.citedreferencePaschou SA, Anagnostis P, Karras S, et al. Bone mineral density in men and children with haemophilia A and B: a systematic review and meta-analysis. Osteoporos Int. 2014; 25 ( 10 ): 2399 - 2407.
dc.identifier.citedreferenceAnagnostis P, Vakalopoulou S, Slavakis A, et al. Reduced bone mineral density in patients with haemophilia A and B in Northern Greece. Thromb Haemost. 2012; 107 ( 3 ): 545 - 551.
dc.identifier.citedreferenceRecht M, Liel MS, Turner RT, Klein RF, Taylor JA. The bone disease associated with factor VIII deficiency in mice is secondary to increased bone resorption. Haemophilia. 2013; 19 ( 6 ): 908 - 912.
dc.identifier.citedreferenceAmos-Landgraf, JM, Cottle A, Plenge RM, et al., X chromosome-inactivation patterns of 1,005 phenotypically unaffected females. Am J Hum Genet, 2006. 79 ( 3 ): 493 - 499.
dc.identifier.citedreferenceMerskey, C, The occurrence of haemophilia in the human female. Q J Med. 1951; 20 ( 79 ): 299 - 312.
dc.identifier.citedreferenceMacLean, PE, Fijnvandraat K, Beijlevelt M, Peters M, The impact of unaware carriership on the clinical presentation of haemophilia. Haemophilia, 2004; 10 ( 5 ): 560 - 564.
dc.identifier.citedreferenceArya, 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 ( 12 ): 3211 - 3221.
dc.identifier.citedreferenceArya, 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 ( 6 ): 1506 - 1514.
dc.identifier.citedreferencePlug, I, Mauser-Bunschoten EP, Bröcker-Vriends AHJT, et al., Bleeding in carriers of hemophilia. Blood, 2006. 108 ( 1 ): 525 - 526.
dc.identifier.citedreferenceMiller, CH, Bean CJ, Genetic causes of haemophilia in women and girls. Haemophilia, 2021. 27 ( 2 ): e164 - e179.
dc.identifier.citedreferencevan Galen, KPM, d’Oiron R, James P, et al., A new hemophilia carrier nomenclature to define hemophilia in women and girls: communication from the SSC of the ISTH. J Thromb Haemost, 2021; 19 ( 8 ): 1883 - 1887
dc.identifier.citedreferenceMauser Bunschoten, EP, van Houwelingen JC, Visser EJS, van Dijken PJ, Kok AJ, Sixma JJ. Bleeding symptoms in carriers of hemophilia A and B. Thromb Haemost, 1988; 59 ( 3 ): 349 - 352.
dc.identifier.citedreferenceElbatarny, M, Mollah S, Grabell J, et al., Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project. Haemophilia, 2014; 20 ( 6 ): 831 - 835.
dc.identifier.citedreferenceSrivastava, A, Santagostino E, Dougall A, et al., WFH guidelines for the management of hemophilia, 3rd edition. Haemophilia, 2020; 26 Suppl 6: 1 - 158.
dc.identifier.citedreferenceCarrel, L, Willard HF, X-inactivation profile reveals extensive variability in X-linked gene expression in females. Nature, 2005. 434 ( 7031 ): 400 - 404.
dc.identifier.citedreferenceGaragiola, I, Mortarino M, Siboni SM, et al., X Chromosome inactivation: a modifier of factor VIII and IX plasma levels and bleeding phenotype in Haemophilia carriers. Eur J Hum Genet, 2021; 29 ( 2 ): 241 - 249.
dc.identifier.citedreferenceCygan, PH, Kouides PA, Regulation and importance of factor VIII levels in hemophilia A carriers. Curr Opin Hematol, 2021; 28 ( 5 ): 315 - 322.
dc.identifier.citedreferencevan Galen, K, Lavin M, Skouw-Rasmussen N, et al., European principles of care for women and girls with inherited bleeding disorders. Haemophilia, 2021; 27 ( 5 ): 837 - 847.
dc.identifier.citedreferenceOlsson, A, Hellgren, M., Berntorp, E, Ljung, R, Baghaei, F. Clotting factor level is not a good predictor of bleeding in carriers of haemophilia A and B. Blood Coagul Fibrinolysis, 2014; 25 ( 5 ): 471 - 475.
dc.identifier.citedreferenceParoskie, A, Gailani D, DeBaun MR, Sidonio RF Jr, A cross-sectional study of bleeding phenotype in haemophilia A carriers. Br J Haematol, 2015; 170 ( 2 ): 223 - 228.
dc.identifier.citedreferenceJames, PD, Mahlangu J, Bidlingmaier C, et al., Evaluation of the utility of the ISTH-BAT in haemophilia carriers: a multinational study. Haemophilia, 2016; 22 ( 6 ): 912 - 918.
dc.identifier.citedreferencePuetz, J, Cheng D, Descriptive analysis of bleeding symptoms in haemophilia carriers enrolled in the ATHNdataset. Haemophilia, 2021; 27 ( 6 ): 1045 - 1050.
dc.identifier.citedreferenceSidonio, RF, D Mili F, Li T, et al., Females with FVIII and FIX deficiency have reduced joint range of motion. Am J Hematol, 2014; 89 ( 8 ): 831 - 836.
dc.identifier.citedreferenceGilbert, L, Rollins L, Hilmes M, et al., Haemophilia A carriers demonstrate pathological and radiological evidence of structural joint change s. Haemophilia, 2014; 20 ( 6 ): e426 - e429.
dc.identifier.citedreferenceHaamid F, Sass AE, Dietrich JE. NASPAG clinical recommendation. Heavy menstrual bleeding in adolescents. J Pediatr Adolesc Gynecol. 2017; 30: 335 - 340.
dc.identifier.citedreferenceCôté I, Jacobs P, Cumming D. Work loss associated with increased menstrual loss in the United States. Obstet Gynecol. 2002; 100 ( 4 ): 683 - 637.
dc.identifier.citedreferenceWang W, Bourgeois T, Kilma J, Berlan ED, Fischer AN, O’Brien SH. Iron deficiency and fatigue in adolescent females with heavy menstrual bleeding. Haemophilia. 2013; 19 ( 2 ): 225 - 230.
dc.identifier.citedreferenceKennedy KER, Onyeonwu C, Nowakowski S, et al. Menstrual regularity and bleeding is associated with sleep duration, sleep quality and fatigue in a community sample. J Sleep Res. 2021: e13434.
dc.identifier.citedreferenceMcGrath M, Quint EH, Weyand AC. Depression in adolescents and young adults with heavy menstrual bleeding in a referral clinic setting. Am J Hematol. 2021; 96 ( 4 ): E105 - E108
dc.identifier.citedreferenceWeyand AC, Fitzgerald KD, McGrath M, et al. Depression in female adolescents with heavy menstrual bleeding. J Pediatr. 2021:S0022-3476(21)00880-5.
dc.identifier.citedreferenceZia A, Jain S, Kouides P, et al. Bleeding disorder in adolescents with heavy menstrual bleeding in a multicenter prospective US cohort. Haematologica. 2020; 105: 1969 - 1976.
dc.identifier.citedreferenceKadir RA, Economides DL, Sabin CA, Owens D, Lee CA. Frequency of inherited bleeding disoders in women with menorrhagia. Lancet. 1998; 351: 485 - 489.
dc.identifier.citedreferenceDowlut-McElroy T, Williams KB, Carpenter SL, Strickland JL. Menstrual patterns and treatment of heavy menstrual bleeding in adolescents with bleeding disorders. J Pediatr Adolesc Gynecol. 2015; 28: 499 - 501.
dc.identifier.citedreferenceMcLintock C. Women with bleeding disorders: clinical and psychological issues. Haemophilia. 2018; 24 ( S6 ): 20 - 24.
dc.identifier.citedreferenceKadir RA, Economides DL, Sabin CA, Pollard D, Lee CA. Assessment of menstrual blood loss and gynaecological problems in patients with inherited bleeding disorders. Haemophilia. 1999; 5 ( 1 ): 40 - 48.
dc.identifier.citedreferenceWeyand AC, James PD. Sexism in the management of bleeding disorders. Res Pract Thromb Haemost. 2020; 5 ( 1 ): 51 - 54.
dc.identifier.citedreferenceNational Hemophilia Foundation. Project Red Flag: Real Talk About Women’s Bleeding Disorders. http://www.projectredflag.org/.
dc.identifier.citedreferenceOleka C, Dietrich JE. HMB in the adolescent: a review of the modern approach to diagnosis and management. Clin Obstet Gynecol. 2020; 63 ( 3 ): 553 - 560.
dc.identifier.citedreferenceSpence M, de Repentigny K, Bowman M, Hopman W, Thibeault L, James P. Validation of the pictorial blood loss assessment chart using modern sanitary products. Haemophilia. 2021; 27 ( 5 ): e632 - e635.
dc.identifier.citedreferenceZia A, Kouides P, Khodyakov D, et al, Standardizing care to manage bleeding disorders in adolescents with heavy menses—a joint project from the ISTH pediatric/neonatal and women’s health SSCs. J Thromb Haemost. 2020; 18 ( 10 ): 2759 - 2774
dc.identifier.citedreferenceMokhtar GM, Ibrahim WE, Kassim NA, Ragab IA, Saad AA, Raheem HGA Alterations of platelet functions in children and adolescents with iron-deficiency anemia and response to therapy, Platelets. 2015; 26: 5, 448 - 452
dc.identifier.citedreferenceBrown MC, White MH, Friedberg R, et al. Elevated von Willebrand factor levels during heavy menstrual bleeding episodes limit the diagnostic utility for von Willebrand disease. Res Pract Thromb Haemost. 2021; 5 ( 4 ): e12513.
dc.identifier.citedreferenceGBD 2015 Disease and Injury Incidence and Prevalence Collaborators. Global, regional, and national incidence, prevalence, and years lived with disability for 310 disease and injuries, 1990–2015: a systematic analysis for the Global Burden of Disease Study 2015. Lancet. 2016; 388: 1545 - 602.
dc.identifier.citedreferenceDugan C, MacLean B, Cabolis K, Abeysiri S, Khong A, Sajic M, Richards T. Women’s Health research Collaborative. The misogyny of iron deficiency. Anaesthesia. 2021; 76 Suppl 4: 56 - 62.
dc.identifier.citedreferenceCamaschella C. Iron-deficiency anemia. N Engl J Med. 2015; 372 ( 19 ): 1832 - 43.
dc.identifier.citedreferenceMei Z, Addo OY, Jefferds ME, et al. Physiologically based serum ferritin thresholds for iron deficiency in children and non-pregnant women: a US National Health and Nutrition Examination Surveys (NHANES) serial cross-sectional study. Lancet Haematol. 2021; 8 ( 8 ): e572 - e582.
dc.identifier.citedreferenceKrayenbuehl PA, Battegay E, Breymann C, Furrer J, Schulthess G. Intravenous iron for the treatment of fatigue in nonanemic, premenopausal women with low serum ferritin concentration. Blood. 2011; 118 ( 12 ): 3222 - 7.
dc.identifier.citedreferencePittori C, Buser A, Gasser UE, et al. A pilot iron substitution programme in female blood donors with iron deficiency without anaemia. Vox Sang. 2011; 100 ( 3 ): 303 - 11.
dc.identifier.citedreferenceStoffel NU, Cercamondi CI, Brittenham G, et al. Iron absorption from oral iron supplements given on consecutive versus alternate days and as single morning doses versus twice-daily split dosing in iron-depleted women: two open-label, randomised controlled trials. Lancet Haematol. 2017; 4 ( 11 ): e524 - e533. https://doi.org/10.1016/S2352-3026(17)30182-5. Epub 2017 Oct 9. PMID: 29032957
dc.identifier.citedreferenceStoffel NU, Zeder C, Brittenham GM, Moretti D, Zimmermann MB. Iron absorption from supplements is greater with alternate day than with consecutive day dosing in iron-deficient anemic women. Haematologica. 2020; 105 ( 5 ): 1232 - 1239.
dc.identifier.citedreferenceTolkien Z, Stecher L, Mander AP, Pereira DI, Powell JJ. Ferrous sulfate supplementation causes significant gastrointestinal side-effects in adults: a systematic review and meta-analysis. PLoS One. 2015; 10 ( 2 ): e0117383.
dc.identifier.citedreferenceMatteson KA, Rahn DD, Wheeler TL 2nd, et al. Nonsurgical management of heavy menstrual bleeding: a systematic review. Obstet Gynecol. 2013; 121 ( 3 ): 632 - 643.
dc.identifier.citedreferenceNakajima ST, Archer DF, Ellman H. Efficacy and safety of a new 24-day oral contraceptive regimen of norethindrone acetate 1 mg/ethinyl estradiol 20 micro g (Loestrin 24 Fe). Contraception. 2007; 75 ( 1 ): 16 - 22
dc.identifier.citedreferenceHuguelet PS, Laurin JL, Thornhill D, Moyer G. Use of the levonorgestrel intrauterine system to treat heavy menstrual bleeding in adolescents and young adults with inherited bleeding disorders and Ehlers-Danlos syndrome. J Pediatr Adolesc Gynecol. 2021:S1083-3188(21)00341-7.
dc.identifier.citedreferenceCampos RR, Baêta T, Silva-Filho A, Rezende SM, Rocha ALL. Use of a levonorgestrel 52-mg intrauterine system in the control of abnormal uterine bleeding in women with inherited bleeding disorders. Contraception. 2020; 102 ( 4 ): 254 - 258.
dc.identifier.citedreferenceConnell NT, Flood VH, Brignardello-Petersen R, et al. ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease. Blood Adv. 2021; 5 ( 1 ): 301 - 325.
dc.working.doiNOen
dc.owningcollnameInterdisciplinary and Peer-Reviewed


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