Heavy menstrual bleeding affects 9 in 10 women with EDS: Study
Delays in diagnosis of condition lead to emergency stays, surgery, transfusions
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Heavy menstrual bleeding affects nearly 9 in 10 women with Ehlers-Danlos syndrome (EDS), with half experiencing severe bleeding, a new study reports.
Despite this high burden, many women experience extended delays in EDS diagnosis — often after emergency visits, transfusions, and major surgeries to treat heavy menstrual bleeding.
“Increased awareness amongst primary care providers (PCP) and early gynaecology involvement may improve timely diagnosis, prevent unnecessary hysterectomies, and monitor for other gynaecologic complications of EDS,” scientists wrote in a letter to the editor.
The letter to the editor, “Timely Diagnosis and Management in Adult Patients With Ehlers-Danlos Syndrome Experiencing Gynaecological Bleeding,” was published in the journal Haemophilia.
Many EDS patients tend to bleed or bruise easily
EDS is a group of inherited conditions that affect the body’s connective tissues, which provide support for skin, joints, blood vessels, and organs. People with EDS often have very flexible (hypermobile) joints, stretchy or fragile skin, slow wound healing, and chronic pain.
Due to weakened blood vessels, many EDS patients also tend to bleed or bruise easily. In particular, women with EDS may experience heavy menstrual bleeding or significant bleeding after childbirth. Still, very little has been published about how best to manage heavy menstrual bleeding in women with EDS.
To address this gap, scientists at the University of Alberta in Canada collected clinical data on 42 women with EDS (median age 38) followed by the Northern Alberta Bleeding Disorders Program. The team specifically investigated data related to the time to an EDS diagnosis, the rates of gynecological bleeding, and the management of heavy menstrual bleeding.
Of those examined, most were diagnosed with hypermobile EDS (69%), followed by unspecified (19%), and vascular EDS (12%). Researchers also sourced data from the same number of women with von Willebrand disease (VWD), a common inherited bleeding disorder also marked by easy bruising and bleeding, including heavy menstrual bleeding.
Diagnosis delayed despite known family history of bleeding disorders
According to the analysis, women with EDS were diagnosed at an older age than those with VWD (median 33 years vs. 26 years). Before being diagnosed, women in both groups experienced frequent bleeding episodes (median 4 vs. 3).
And yet, diagnosis was delayed even though a large proportion of women with EDS and VWD (48% vs. 33%) had a known family history of bleeding disorders before seeing a blood specialist.
The consequences of delayed diagnosis were significant. In the EDS group alone, there were 71 emergency department visits and four hospitalizations for bleeding. All hospitalizations and blood transfusions, and most (61%) of emergency visits, happened before the EDS diagnosis.
Pregnancy and gynecological bleeding were common among women with EDS. The group reported 101 pregnancies and 61 live births. Heavy menstrual bleeding affected nearly all (88%) of women, with half (50%) experiencing severe episodes requiring emergency department visit, hospitalization, blood transfusion, or a gynecologic procedure to control the bleeding.
Severe bleeding after childbirth occurred in 31%, hemorrhagic ovarian cysts (blood-filled sac on the ovary) in 14%, and bleeding during pregnancy in 10%.
Despite these high rates, half as many women with EDS had a documented gynecology consultation as those with VWD (31% vs. 67%). Overall, however, the rates of gynecological bleeding were similar between the two disease groups.
Our study demonstrates a high prevalence of diagnostic delays in women with EDS, with an older age at diagnosis compared to matched VWD patients. Despite a high burden of [heavy menstrual bleeding] in nearly 90% of our cohort, gynaecology consultation was underutilised in women with EDS.
Many women with EDS required medical treatment for heavy menstrual bleeding. Medications included tranexamic acid (78%), desmopressin injections (57%), oral contraceptive pills (49%), and an intrauterine device (43%).
Surgical procedures were also common, with 30% undergoing a hysterectomy (removal of the uterus), 11% having endometrial ablation (uterine lining destruction), and 8% undergoing dilation and curettage (uterine tissue removal). Most hysterectomies (78%) and nearly half of ablations (43%) were performed before women were diagnosed with either EDS or VWD.
Iron deficiency was another major issue. Half (50%) of women with EDS were iron-deficient before diagnosis, and one-third (33%) remained iron-deficient at their most recent follow-up.
“Our study demonstrates a high prevalence of diagnostic delays in women with EDS, with an older age at diagnosis compared to matched VWD patients,” the researchers wrote. “Despite a high burden of [heavy menstrual bleeding] in nearly 90% of our cohort, gynaecology consultation was underutilised in women with EDS. We call for further action in multidisciplinary collaboration between PCP, haematologists, patients/families and patient care organisations to improve education, awareness and advocacy.”
