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Palliative Care Approaches in Machado-Joseph Disease
Mara Arruda1, Carolina Vidal2, Maria João Serpa2
1Unidade de Saúde da Ilha Terceira - Centro de Saúde da Praia da Vitória, Portugal.
Journal of Palliative Medicine
|February 27, 2025
Summary
This study describes the first Machado-Joseph disease (MJD) patient cohort in palliative care, identifying common symptoms like swallowing difficulties and insomnia. Multidisciplinary approaches significantly improved quality of life for advanced MJD patients.
Area of Science:
- Neurology
- Palliative Care
Background:
- Spinocerebellar ataxias (SCAs) are rare neurodegenerative disorders.
- Machado-Joseph disease (MJD) is the most common autosomal dominant ataxia.
- No disease-modifying treatments exist for MJD; palliative care is recommended.
Purpose of the Study:
- To describe the first cohort of MJD patients managed by a palliative care team.
- To identify prevalent symptoms in advanced MJD.
- To detail multidisciplinary interventions for improving quality of life.
Main Methods:
- Retrospective analysis of MJD patients in a palliative care unit (PCU).
- Data collected included demographics, functional status, symptom scores, treatments, and advanced care planning.
- Nonparametric statistical tests were used for analysis.
Main Results:
- Median follow-up was 5 years; median disease duration was 18.5 years.
- Most frequent symptoms were swallowing difficulties, constipation, depression, and insomnia (more prevalent in MJD).
- Mortality rate was 30%, with 55% of deaths linked to disease progression.
Conclusions:
- This is the first cohort description of MJD patients in a PCU.
- Swallowing difficulties, constipation, depression, and insomnia are key symptoms in advanced MJD.
- Interdisciplinary approaches, alongside pharmacological treatment, are crucial for managing advanced MJD.
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