Determinants and Consequences of the Transition to Adulthood Among Adolescents With Haemophilia
Condition: Haemophilia
MeSH terms: Hemophilia A
Study type: Observational

Summary: 
      Severe haemophilia is a rare disease characterized by spontaneous bleedings from early
      childhood, which may lead to various complications especially in joints. Due to advances in
      medical care and more specifically in the development of prophylactic strategies by the
      application of clotting factor concentrates, life expectancy of persons with severe
      haemophilia has significantly increased over the last decades. This progress requires a
      long-term follow-up, including into adulthood. The adherence to a regular clinical follow-up
      and to a prophylactic treatment then depends on how successful patients' transition from
      childhood to adulthood has been as this process involves a transfer of responsibility from
      parents to patients concerning the management of their health. Beyond the issue of patients'
      adherence, a suboptimal transition may also impair quality of life and the entry into
      adulthood, especially at the social, emotional and professional levels. Only a few studies
      have been conducted to identify the specific needs and difficulties young persons with severe
      haemophilia experience during their transition from childhood to adulthood, and none of these
      studies has been carried out in France where the features of the health care system are very
      specific.

      Therefore, this study aims to address the issue of transition into adulthood among young
      persons with severe haemophilia in France. This study will focus not only on the facilitators
      and barriers of the access to health care but also, from a more global perspective, on all
      the specific concerns and difficulties they may experience as they grow into adulthood which
      may impair their long-term health related quality of life as well as their personal
      empowerment. This study will also allow to identify some of the socio- cognitive, emotional,
      and familial determinants of a good transition into adulthood.
    

Detailed description: 
      The main objective of this study is to assess the impact of transition from adolescence into
      adulthood especially on adherence to health care, among young people with severe haemophilia
      in France.

      The operational objectives of this study are: i) to compare the level of adherence in
      adolescents and in young adults (YA) ii) to identify determinants (medical, organisational,
      socio-demographic and social, and psychosocial and behavioural factors) of the level of
      adherence in YPWH (young people with haemophilia), iii) to assess specific factors involved
      in suboptimal level of adherence in the sub-groups of adolescents on one hand and of YA on
      the other hand, iv) to identify groups of patients (clusters) regarding both their level of
      adherence and their psychosocial characteristics, v) to examine trough a qualitative approach
      YPWH needs and expectations towards the health care system during the transition process, and
      to identify some ways to improve their global care.

      This study is an exploratory, observational, multicentric, transversal study aimed at
      describing the perceptions of adolescents (14-17 years old) with severe haemophilia to those
      of young adults (20-29 years old) regarding their expectations and their feelings about
      growing into adulthood. Every patient enrolled in the FranceCoag national cohort (cohort of
      French patients suffering from inherited deficiencies of coagulation proteins), suffering
      from severe haemophilia, aged from 14 to 17 years old or from 20 to 29 years old, will be
      offered to get enrolled in the TRANSHEMO study (expected number eligible of participants: 154
      adolescents and 389 young adults). , among whom 70% are expected to take part in the study).
      The patients' participation in this study will not modify their medical or paramedical care.
      A multifocal approach which combines both quantitative and qualitative data collection will
      be proposed.
    

Intervention name(s): Patient questionnaire
Intervention type(s): Other
Intervention description(s): quantitative and qualitative data collection will be proposed.
Observational model: Other
Time perspective: Cross-Sectional
Sampling method: Probability Sample
Study population: 
        cohort of persons with severe haemophilia
      
Keywords: adolescents; young adults