A «aceitação» em portadores assintomáticos e sintomáticos da doença de Machado-Joseph

Abstract

Machado-Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), is an autosomal dominant neurodegenerative disorder of late onset. The presence of the disease is frequently accompanied by important psychological alterations, especially in more advanced stages of the disease. The present work aims to examine the levels of acceptance, psychological well-being, family satisfaction, and self-compassion in a sample of 40 carriers of the MJD mutation, of whom 11 were asymptomatic and 29 symptomatic, as well as how these constructs were interconnected. A protocol was used that followed a retrospective design, and included a semi-structured interview and various psychological assessment scales. In asymptomatic carriers, acceptance was associated with family satisfaction, psychological well-being, and self-compassion. In the case of symptomatic carriers, acceptance proved to be significantly associated with self-compassion and psychological well-being. The results indicate that the levels of acceptance, psychological well-being, family satisfaction, and self-compassion decrease with the onset of symptoms. Data supports the importance of developing psychotherapeutic plans aimed at increasing acceptance, not only upon the appearance of symptoms, but also as a preventive approach, through psychological interventions that integrate the practice of mindfulness and compassion, not only directed at the individuals but also their family and social network.