Wilson disease: The look of medical staff, patients and family members


Por: Rodriguez, L, Llorens, O, Sanjuan, M and Carbonell, C

Publicada: 1 abr 2019 Ahead of Print: 1 mar 2019
Resumen:
Background: Wilson disease (WD) is a rare congenital disease that causes hepatic, neurological or lenticular degeneration due to the accumulation of copper. Sometimes it is incapacitating with implications in the quality of life of those of affected and their families. The objective of this work was to identify the needs of medical staff and the social and emotional needs of patients with WD and their families. Methods: A qualitative research was developed in the Valencian Region during 2015-2016, five interviews with medical staff and two focus groups were made, one with family members and another with patients using a script divided into: diagnosis, treatment, health care and quality of life. The information was collected in audio/video and transcribed. An analysis of discourse (professional vs family/affected) determining needs was made. Results: Medical staff felt away from the emotional needs of families and patients and need more information in order to facilitate an early diagnosis. Families expressed difficulties regarding the treatment's adherence, especially when those patients are teenagers who have doubts about the cooper-free diet. Patients had problems with the treatment's instructions. Also, those with neurological problems felt stigmatised by the physical consequences. Conclusions: Medical staff need more knowledge about this pathology. Better educational training for them would facilitate the diagnosis. Families and patients need more information about the guidelines for the treatment's administration and foods that should be excluded from the diet. The correct administration of the treatment will allow those affected to improve their quality of life with a total or partial recovery of their symptoms.

Filiaciones:
:
 FISABIO UVEG, Unidad Mixta Invest Enfermedades Raras, Ave Cataluna 21, Valencia 46020, Spain

:
 FISABIO UVEG, Unidad Mixta Invest Enfermedades Raras, Ave Cataluna 21, Valencia 46020, Spain

 Generalitat Valenciana, Direcc Gen Salud Publ, Valencia, Spain

 CIBER Epidemiol & Salud Publ, Madrid, Spain

Sanjuan, M:
 Univ Valencia, Dept Sociol & Antropol Social, Valencia, Spain

:
 FISABIO UVEG, Unidad Mixta Invest Enfermedades Raras, Ave Cataluna 21, Valencia 46020, Spain
ISSN: 11355727





REVISTA ESPANOLA DE SALUD PUBLICA
Editorial
MINISTERIO DE SANIDAD Y CONSUMO, PASEO DEL PRADO 18-20, MADRID, 28071, SPAIN, España
Tipo de documento: Article
Volumen: 93 Número:
Páginas:
WOS Id: 000490195500001
ID de PubMed: 30924466
imagen Open Access

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