Factors of vulnerability in therapeutic itineraries of rare diseases: an integrative review

Abstract

Objective: The article is concerned with broadening the understanding of ethical conflicts experienced by rare disease patients, identifying the main vulnerability factors in the therapeutic itineraries of people living in this condition.

Method: For that, an integrative review of the literature developed in the international databases, published between the years 2000 to 2016, comprising Portuguese, English and Spanish languages was carried out.

Results and discussion: The results, based on the qualitative analysis of the selected studies, made it possible to identify the following vulnerability factors: lack of information; Professional unpreparedness; Difficulty in reaching a diagnosis; Long paths covered / various health services; Lack of privacy and autonomy in participating in rare disease research; Difficulties of treatments and / or treatments of auto cost; Financial difficulties / impoverishment; Lack of organization and structure of health services; Discrimination, deprivation and / or stigmatization, and emotional distress.

Conclusion: In discussing these different dimensions of vulnerability from the field of bioethics, the article concludes by the need to address of therapeutic itineraries from new interdisciplinary perspectives, seeking to ensure respect for the right to life, health and protection of the dignity of People living with rare diseases.