Distressing Bleeding Symptoms in Interviews of Patients With Hypermobile Ehlers-Danlos Syndrome and Hypermobility...

Hypermobile Ehlers-Danlos Syndrome (hEDS) and hypermobility spectrum disorder (HSD) are associated with symptoms in many organ, but those related to haemostasis are not fully characterized.

To determine the frequency of clinically significant excessive bleeding in this population as measured by the International Society on Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH-BAT) and its associated health-related quality of life (HRQoL) concerns.

Thirty-five participants were enrolled from the hEDS GENE study. We use a mixed methodology approach to explore bleeding and other symptoms and their effect on participants life and health. This included self-administered quantitative questionnaires and semi-structured interviews, including the ISTH-BAT. We assessed severity of distress related to bleeding using a novel quantitative assessment score based on our participant interview responses.

Nearly one third of participants reported clinically significant bleeding (ISTH-BAT mean = 4.97) yet less than 10% of those received evaluation by haematology for bleeding. Heavy menstrual bleeding (HMB) was the most common bleeding symptom. Poor HRQoL related to multiple bleeding domains was reported. More than one third of participants reported significant distress from bleeding symptoms with a mean ISTH-BAT score of 8.25.

Bleeding is a significant clinical concern and cause of distress in people with hypermobility correlating with the severity of bleeding, reflected by a high bleeding score. This unique assessment tool needs validation in a larger patient cohort. Increased awareness and further evaluation of this condition is needed to adequately serve this patient population.