Determinants and Consequences of the Transition to Adulthood for Adolescents With Severe Haemophilia: TRANSHEMO 2, an Ancillary Study to the TRANSHEMO Project

Haemophilia is a rare genetic disorder which, in its severe form and in the absence of treatment, can be life-threatening. Since the 1960s and the introduction of coagulation factor concentrates, the life expectancy of people with haemophilia has increased rapidly. Today, for most affected individuals, the disease is experienced as a chronic condition. The transition process enabling adolescents and young adults (AYA) with a chronic disease to move into adult life can be complex, as they must face all the changes experienced by AYA in general, combined with issues related to their chronic condition and its management. A successful transition involves a transfer of responsibility from parents to AYA regarding the management of their health condition, as well as the acquisition by AYA of knowledge, skills and autonomy. A difficult transition may lead to decreased adherence to follow-up or treatment, deterioration of overall health status and/or quality of life, or difficulties in entering adult life. In this context, the national cross-sectional study TRANSHEMO was initiated in 2017. Its objective was to compare adherence to healthcare management between two groups of AYA with severe haemophilia (adolescents \[for whom the transition is ongoing\] versus young adults \[for whom the transition may have been completed\]), and to identify the determinants of this adherence. The results showed that young adults had a lower adherence rate than adolescents (82.2% vs. 61.2%, p\

Inclusion Criteria: * Young adults who participated in the TRANSHEMO project during adolescence and who are currently aged 20-29 years; * Young adults with severe haemophilia (haemophilia A or B); * Young adults registered in the FranceCoag registry; * Young adults who have received the participant information sheet for the TRANSHEMO 2 project; * Young adults who did not object to participation in the present study. Exclusion Criteria: * patients with comprehension difficulties; * patients who are unable to read and/or write; * patients who express opposition to participation in this study.