Psychopathological dimensions in subjects with hereditary ATTR V30M amyloidosis and their relation with life events due to the disease

Abstract

Background: Chronic physical illness has been associated with emotional distress. Chronic diseases may change usual family patterns with economic, social and family losses. Hereditary ATTR V30M amyloidosis is a rare, fatal inherited systemic amyloidosis, with chronic evolution and beginning in adulthood.

Aims and methods: To evaluate psychopathological dimensions and how they correlated with disease-related life events, 209 symptomatic and asymptomatic carriers, participated in the study. Sociodemographic and Family and Personal History Disease questionnaires and brief symptom inventory (BSI) were applied.

Results: BSI indices, global severity index (GSI), positive symptom index (PSI) and positive symptom total (PST) scored higher than general population. Independent predictors for GSI >0.83 were female sex (OR = 3.46, p = .005) and being symptomatic carriers (OR = 3.03, p = .039). Independent predictors of a PST >26.99 were female sex (OR = 3.74, p = .012) symptomatic carrier (OR = 5.32, p = .025), age between 15 and 24 years at affected parent’s death (OR = 5.26, p = .04). Independent predictors of a PSI >1.56 were being asymptomatic carrier (OR = 6.3, p = .036); to have children (OR = 3.19, p = .043) and have ≤14 years at parent’s disease onset (OR = 6.39, p = .05).

Conclusions: Results point to an important vulnerability of this population for psychological distress and psychiatric disease. Early life events related to disease, being sick and sex are associated with psychopathological distress.

Keywords:

• Anxiety
• BSI
• depression
• hereditary ATTR V30M amyloidosis
• life-events
• psychopathological symptoms

Disclosure statement

Lopes A. received honoraria from Pfizer for presentations at courses of TTR-related FAP. Coelho T. has received support from Pfizer, Ionis Pharmaceuticals and Alnylan Pharmaceuticals to attend scientific meetings, integrates the speaker’s bureau of Pfizer, and received honoraria. Sequeiros, J. received honoraria from Pfizer for presentations on genetic counselling of TTR-related FAP and courses, and for the preparation of leaflets and webinars on genetic counselling. Remaining authors report no conflict of interest.