A challenge for AFH Bruxelles Thomas Sanni on behalf of the THE3P - PowerPoint PPT Presentation

Patients with mild haemophilia : Half of the haemophiliac population and yet a quasi absence of educational guidance A challenge for AFH Bruxelles Thomas Sannié on behalf of the THE3P team and with the help of Lars Lehrmann from the Danish Hamophilia Society EHC Round Table, November 30 2015

Haemophilia A rare genetic disorder characterised by bleeding due to a deficiency in clotting • factor VIII or IX Affects mainly boys but there are also a few rare cases of girls affected • The level of factor VIII or IX determines, in principle, the degree of the bleeding • disorder and patient care < 1 % Severe • Spontaneous or 2167 patients frequent bleeds that is 32,85% Haemophilia • self infusion • Educational program Bleeds occurring 1 à 5 % 1123 patients that • offers(patients and family) during traumas or is 17,02 % moderate developed by HTC’s and AFH surgery haemophilia chapters Spontaneous rare • bleeding 6 to 40 % Bleeding in case • Indication fact coag rare 3305 patients that • of traumas or • Desmopressin and first aid is 50,11 % surgery Mild haemostatics No spontaneous (mild haemophiia haemophilia • • Educational programs bleeds women ++) almost don’t exist Source FranceCoag Nov.2015

Mild haemophilia: absent from educational programmes Why? • – A forgotten group? – Little use of clotting factors? – Few educational needs expressed by patients? – Educational offers unsuited for these patients? – Low knowledge of who to contact with problems regarding haemophilia? However, AFH together with health professionals note that there are • needs to which they must respond: To respond to these questions : • – The interdisciplinary group THE3P* has tried to identify the educational needs of people living with mild haemophilia (patients and family) * Therapeutic Education Patients, Parents and Professionals composed of the community of healthcares from the various branches (learned societies Cometh, Fidel’hem, Grikh, Permedes), and the patients and families community

1. Little qualitative research published • Individual interviews with 18 young mild haemophiliacs (aged 17-31) (2012)* : – Lack of awareness of the % of their deficient factor – Reluctance to acknowledge their haemophilia – Wait and see attitude when hemorrhagic accidents occur – More confidence in their personal experimentation than in the advice/information provided by the carers – Healthcare professionals seen as “overdoing it” *Nilson J et al. A qualitative study identifying the knowledge, attitudes and behaviours of young men with mild haemophilia. Haemophilia 2012 ; 18 : e120-e145.

1. A Danish quantitative research published rapport • Methodology: – Three Quality of Life (QoL) studies have been conducted with questionaire in 1988 (n=135), 2001 (n=164) and 2012 (n=172). – The target group was haemophiliacs in all ages with moderate or mild haemophilia A, B or von Willebrands disease type 3. *Rapport published by The Danish Haemophilia Society 2013 (only in Danish at the moment): http://www.bloderforeningen.dk/default.asp?MenuID=1129

1. A Danish quantitative research published rapport (2) • The study show that around 33% of the haemophiliacs encounter problems getting the right treatment outside opening hours, at the two Danish haemophilia centres. • Some of these problems are due to not having the right knowledge of who to contact, and second not being able to self medicate, which is mostly problematic for moderate bleeders because they are not used to self medicate and do not have regular contact with the haemophilia centres.

3. Survey carried out under the aegis of the THE3P interdisciplinary group • Methodology : – 3 focus groups (FG) with patients and families living with mild haemophilia (n = 22) 1 of whom is also a healthcare professional (n = 11) FG composition location 11 healthcare providers + 1 parent + 2 patients (THE3P group) FG I Paris 5 adults patients, 1 parent, 1 sister adult and carrier FG II Paris 3 adults, 4 parents, 3 teenagers, 1 child FG III Bordeaux – Recruiting of patients and families via regional AFH branches and HTCs No criteria for exclusion of patients and families – No particular grouping in the various groups – 1 moderator (AFH Educationnal project leader) and 1 observer (resource patient)

Guide for interviewing the focus groups Theme Questions (and follow up questions) How did you react when you learned that you had mild haemophilia? Patients’ views on mild Do you feel as a person with a disease ? haemophilia What do you know about mild haemophilia? In your opinion, what is it due to? Knowledge Can there be complications? What do you know about treatments? How do you keep informed? Do you feel sufficiently informed? - concerning transmission in your family? concerning complications? concerning treatments? As a result of mild haemophilia, has anything changed in your life? If so, what? Experience What is your attitude towards telling people you have mild haemophilia or keeping it to of the yourself? How is information passed on in your family concerning the hereditary risk? disorder Expectations Now after your experience, what would you like to know a little bit more about that would be useful in your daily life ? What advice would you give to a patient who has just learned he has mild haemophilia? In retrospect, what are your expectations concerning health professional in general? In your opinion, what would help you to follow the advice of health professionals? We plan to set up some training on mild haemophilia : what it is, what the treatment is, what should be done in case of a knock or a bang, transmission in the family, information to pass on … we would like to know what you think about this. What would be the advantage of an educational programme over the various sources of information at your disposal? Educational How do you imagine the sessions? individual, in a group ? where? how long ? program

3 .Results • Patients and families describe a paradox : • neglect and playing down the disorder on the one hand and the difficulties of living with it on the other hand • Communication with healthcare professionals is usually not very or not at all appropriate to their situation • Healthcare professionals express difficulties in finding the appropriate terms, probably linked to their wish to both reassure patients and to warn them about the risks

3 .Results (2) • Patients and families describe a paradox : • Even if patients and families acknowledge they are sufficiently informed about the disorder, they feel misunderstood and would like healthcare professionals to take into account their own experience as a patient of mild haemophilia, more empathy, and the possibility of sharing with the professionals an a priori assessment of risk situations and an analysis afterwards of incidents already experienced • In an emergency, they point out their limits and dread underestimation by emergency professionals

4. Results in terms of needs analysis, educational and skills needs • From a needs analysis by the 3 Focus Groups the GT THE3P defined the educational needs and suggested the associated skills. The result was 4 main themes : • Disorders and patient care (follow up) • Experience of the disorder : feelings relating to the disorder, peer support, PPR support • Relationship with healthcare professionals : Haemophilia Treatment Centres, emergency professionals, general practitioners • Emotional, social and professional life : information from third parties, professionals, physical and sporting activities, practical everyday life and rights

4. Results: 2 examples in terms of educational needs and suggested skills Educational needs for • EXPERIENCE OF THE DISORDER • Associated skills ( subjective experience of the • Expressing views on disorder) the disorder to be understood in the duality of being haemophiliac (I • am normal/I am not normal - I am symptomatic/I am • Expressing experience asymptomatic) to receive individualised adapted responses (positive, negative or • to construct personal views (adolescent, young adult) – • neutral) felt at the time be accepted in various stages of adaptation to mild haemophilia of the diagnosis and to be guided (at the right time) through the various • phases of the disorder (silent and active) certain bleeding to be guided in spite of the weak expression of mild • episodes haemophilia to get individualised answers to a specific expression of • • Identifying the haemophilia to have the guilt lifted in relation to the word « disease » • possibilities of when it is seen as derogative to get explanations on the specifics of the « mild » aspect guidance (including • of haemophilia exchanges with peers) to have the choice between “knowing” or “not knowing” • respected to widen knowledge through multiple sources • (healthcare professionals, family, peers, internet etc.)