title: Surveillance of female patients with inherited bleeding disorders in United States Haemophilia Treatment Centres.
abstract: Inherited bleeding disorders are especially problematic for affected girls and women due to the monthly occurrence of menstrual periods and the effects on reproductive health. Although heavy menstrual bleeding (HMB) is the most common manifestation, females with inherited bleeding disorders (FBD) experience other bleeding symptoms throughout the lifespan that can lead to increased morbidity and impairment of daily activities. The purpose of this article is to describe the utility of a female-focused surveillance effort [female Universal Data Collection (UDC) project] in the United States Haemophilia Treatment Centres (HTCs) and to describe the baseline frequency and spectrum of diagnoses and outcomes. All FBD aged 2 years and older receiving care at selected HTCs were eligible for enrollment. Demographic data, diagnoses and historical data regarding bleeding symptoms, treatments, gynaecological abnormalities and obstetrical outcomes were analysed. Analyses represent data collected from 2009 to 2010. The most frequent diagnoses were type 1 von Willebrand's disease (VWD) (195/319; 61.1%), VWD type unknown (49/319; 15.4%) and factor VIII deficiency (40/319; 12.5%). HMB was the most common bleeding symptom (198/253; 78.3%); however, 157 (49.2%) participants reported greater than four symptoms. Oral contraceptives were used most frequently to treat HMB (90/165; 54.5%), followed by desmopressin [1-8 deamino-D-arginine vasopressin (DDAVP)] (56/165; 33.9%). Various pregnancy and childbirth complications were reported, including bleeding during miscarriage (33/43; 76.7%) and postpartum haemorrhage (PPH) (41/109; 37.6%). FBD experience multiple bleeding symptoms and obstetrical-gynaecological morbidity. The female UDC is the first prospective, longitudinal surveillance in the US focusing on FBD and has the potential to further identify complications and reduce adverse outcomes in this population.
authors.affiliation: Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA 30333, USA. vbyams@cdc.gov
authors.forename: V R
authors.initials: VR
authors.lastname: Byams
authors.forename: P A
authors.initials: PA
authors.lastname: Kouides
authors.forename: R
authors.initials: R
authors.lastname: Kulkarni
authors.forename: J R
authors.initials: JR
authors.lastname: Baker
authors.forename: D L
authors.initials: DL
authors.lastname: Brown
authors.forename: J C
authors.initials: JC
authors.lastname: Gill
authors.forename: A M
authors.initials: AM
authors.lastname: Grant
authors.forename: A H
authors.initials: AH
authors.lastname: James
authors.forename: B A
authors.initials: BA
authors.lastname: Konkle
authors.forename: J
authors.initials: J
authors.lastname: Maahs
authors.forename: M M
authors.initials: MM
authors.lastname: Dumas
authors.forename: S
authors.initials: S
authors.lastname: McAlister
authors.forename: D
authors.initials: D
authors.lastname: Nance
authors.forename: D
authors.initials: D
authors.lastname: Nugent
authors.forename: C S
authors.initials: CS
authors.lastname: Philipp
authors.forename: J M
authors.initials: JM
authors.lastname: Soucie
authors.forename: E
authors.initials: E
authors.lastname: Stang
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D025861:Blood Coagulation Disorders, Inherited / Q000453:epidemiology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003271:Contraceptive Agents, Female / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008595:Menorrhagia / Q000188:drug therapy; D008875:Middle Aged; D011159:Population Surveillance; D006473:Postpartum Hemorrhage / Q000453:epidemiology; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000453:epidemiology; D011446:Prospective Studies; D014481:United States / Q000453:epidemiology; D055815:Young Adult
chemical_list: D003271:Contraceptive Agents, Female