Viver com a doença de Andrade

: desafios para a enfermagem

Abstract

Introduction: Andrade's disease is a rare, hereditary, neurodegenerative disease whose symptoms usually arise in the second half of the second decade of life. Liver transplantation and, more recently, Tafamidis have allowed the natural course of the disease to change, increasing the hope and quality of life of the person affected by the mutation. Objectives: to describe how people experience the uncertainty of the health/illness transition caused by hereditary neurodegenerative disease; to describe the relation between the social representations of the disease, the cultural and health beliefs, the previous experience with the hereditary disease, and the change in the therapeutic approach; to determine its influence in the experiences of the FAP in the perspective of the person carrying the mutation. Methods: study with a qualitative, interpretative and ethnographic approach. Made in the community of Póvoa de Varzim and Vila do Conde. The participants where 31 people with PAF 8 privileged witnesses, 106 residents in the two municipalities, and the newspapers of the Portuguese Association of Paramyloidosis were analyzed. Documentary analysis, analysis of social representations with the support of IRAMUTEQ software and the analysis of qualitative data collected through interviews and the analysis procedures of Milles, Huberman and Saldaña (2014) were carried out. Results: despite the therapeutic advances, FAP is still understood, by the participants, as a serious illness causing great suffering. Its hereditary character affects the carrier and the family, being considered a distinctive factor in the community and perceived as a stigma by people with FAP. The uncertainty of the transition to the condition of the mutation carrier is mediated by personal conditions, community conditions, and societal conditions. From the analysis of the collected data were identified 5 focuses of the nursing attention that should be valued in clinical practice. Conclusion: the people living with FAP in the community of Vila do Conde and Póvoa de Varzim present nursing care needs that have not yet responded in this community. This study makes evident the need to design and validate a model for monitoring the people and families living with Andrade's disease.

Date of Award	15 Mar 2019
Original language	Portuguese
Awarding Institution	Universidade Católica Portuguesa
Supervisor	Felismina Rosa Parreira Mendes (Supervisor)