title: Familial amyloid polyneuropathy: elaboration of a therapeutic patient education programme, "EdAmyl".
abstract: BACKGROUND
  Transthyretin-related amyloidosis (ATTR) is an autosomal dominant disease affecting the peripheral and autonomic nervous system, heart, eyes and kidneys. It is the most disabling hereditary polyneuropathy in adults. The French National Reference centre for this disease was accredited in 2005 with 10 lines of action. One of them is to inform and educate patients about their disease to improve their care and reduce morbidities. We thus decided to elaborate a therapeutic patient education (TPE) programme, starting with patients' needs assessment.
  
  
  METHODS
  A qualitative research study was conducted with one-to-one semi-structured interviews of selected individuals. Recorded interviews were analysed to identify the skills that patients need to acquire. A TPE programme was elaborated on the basis of these findings.
  
  
  RESULTS
  Seven patients, one asymptomatic carrier and two healthy spouses were interviewed. Analysis of the interviews showed that interviewees had a good knowledge of the disease and its symptoms but they had difficulties explaining the disease mechanism and did not have an adequate knowledge of the available treatment options, although they knew that liver transplant might halt progression of the disease. ATTR amyloidosis appeared to have a major negative impact on the patient's physical and mental well-being. Patients feared loss of autonomy and having to require assistance from their relatives and spouses. All interviewees were keen to participate in a TPE programme. Based on this needs assessment, we identified seven skills that patients need to acquire and several pedagogical goals to be achieved during the education programme. An interdisciplinary team then elaborated a complete TPE programme.
  
  
  CONCLUSIONS
  Elaboration of a TPE programme for ATTR amyloidosis required to obtain useful information from the patients themselves, and their relatives, concerning their perception of their disease. This needs' assessment constituted the basis for designing the first TPE programme, to our knowledge, for ATTR amyloidosis. After translation, this programme could be applied in other EU countries and worldwide for this rare disease.
authors: French Reference Centre for Familial Amyloid Polyneuropathy, NNerf, Neurology Department, Hôpital Bicêtre, Assistance Publique Hôpitaux de Paris (APHP), HUPS , Le Kremlin-Bicêtre, France .
authors: Marie
authors: M
authors: Théaudin
authors: Cécile
authors: C
authors: Cauquil
authors: Teresa
authors: T
authors: Antonini
authors: Vincent
authors: V
authors: Algalarrondo
authors: Céline
authors: C
authors: Labeyrie
authors: Sophie
authors: S
authors: Aycaguer
authors: Mireille
authors: M
authors: Clément
authors: Marie
authors: M
authors: Kubezyk
authors: Géraldine
authors: G
authors: Nonnez
authors: Agnès
authors: A
authors: Morier
authors: Catherine
authors: C
authors: Bourges
authors: Amandine
authors: A
authors: Darras
authors: Laurence
authors: L
authors: Mouzat
authors: David
authors: D
authors: Adams
mesh_terms: D000328:Adult; D000368:Aged; D028227:Amyloid Neuropathies, Familial / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D006126:Group Processes; D006801:Humans; D008297:Male; D008875:Middle Aged; D020380:Needs Assessment; D010353:Patient Education as Topic / Q000458:organization & administration*; D016730:Program Development; D020377:Self Efficacy
keywords: ATTR amyloidosis; familial amyloid polyneuropathy; needs assessment; pedagogy; qualitative research; therapeutic patient education; transthyretin