Bleeding and quality of life in people with Glanzmann thrombasthenia—insights from the Glanzmann’s 360 study

Abstract

Background

Glanzmann thrombasthenia (GT) is a rare platelet function disorder that results in severe bleeding. We assessed clinical symptoms and psychological parameters to identify the unmet needs associated with GT.

Objectives

Glanzmann’s 360 is a mixed-methods study designed to give a contemporary snapshot of the impact of living with GT.

Methods

The study comprised a self-completion online survey complemented by interviews conducted with affected individuals and carers recruited via social media and hemophilia treatment centers.

Results

The survey was completed by 88 people with GT and 29 carers of children/young people with GT aged <16 years. The population ranged in age from <2 years to >70 years; 56% were female. Although 47% had been diagnosed with GT under the age of 2 years, 12% were diagnosed after 20 years of age. For 82%, a bleeding phenotype was apparent by the age of 5 years. Most respondents (88%) had experienced at least one bleed in the past week. Bleeding disproportionally affected women. Bleeds resulted in frequent hospital contact and considerable psychological distress: 26% of the population had scores suggestive of low self-esteem, while 30% met criteria suggestive of symptomatic depression. Exploratory analyses suggest that bleed experiences are associated with impaired health-related quality of life.

Conclusion

The Glanzmann’s 360 study reveals the significant physical, psychosocial, and quality-of-life impairments that are likely to be linked to the frequent bleeds experienced by those with GT. Clinicians treating people with GT should promote access to multidisciplinary comprehensive care, including psychosocial support.