SPINAL MUSCULAR ATROPHY IN CHILDHOOD AND THE ROLE OF THE MULTIDISCIPLINARY TEAM IN PALLIATIVE CARE: CHALLENGES AND STRATEGIES IN PROMOTING QUALITY OF LIFE

Spinal Muscular Atrophy (SMA) is a genetic, autosomal recessive, and progressive neuromuscular disease caused by a mutation or deletion of the SMN1 gene, responsible for the production of survival motor neuron (SMN) protein. Deficiency of this protein causes the degeneration of motor neurons in the spinal cord, resulting in symmetrical muscle weakness, muscle atrophy, and progressive functional impairment. Clinical manifestations of SMA include hypotonia, areflexia, delayed or lost motor development milestones, dysphagia, respiratory difficulties, and osteoarticular changes such as scoliosis and contractures. Early diagnosis is essential and is based on clinical evaluation combined with genetic testing. This study aims to: Analyze the role of the multidisciplinary team in the palliative care of children with Spinal Muscular Atrophy (SMA), highlighting the challenges faced and the strategies used to promote the quality of life of patients and their families; To describe the main clinical characteristics of Spinal Muscular Atrophy in childhood and its physical, emotional, and social repercussions for the child and their family; To identify the roles of different professionals in the multidisciplinary team in the palliative care of children with SMA. This is an integrative literature review, with a qualitative approach and descriptive character. For the search for articles, scientific publication databases were used using the health descriptors: Spinal Muscular Atrophy; Multidisciplinary Assistance; Palliative Care; Quality of Life, applying the Boolean operators AND and OR. The results of the study highlighted the physical, emotional, and psychosocial needs of children with spinal muscular atrophy in palliative care; Humanized Assistance in Palliative Care for Pediatric Patients with SMA and the Strategies and Challenges in Palliative Care with the use of Modifying Therapies and the Paradigms Between New Technologies and Palliative Philosophy. Interdisciplinary Strategies for Symptom Management and Comfort Promotion in the Care of Pediatric Patients with SMA, focusing on the Family Support Network and the Grief Process from the perspective of the Multiprofessional Team for Caregivers. It is concluded that the integrated work of the multiprofessional team is fundamental for palliative care in children with SMA, as well as the importance of professional qualification and empathetic interpersonal relationships, contributing to the improvement of care provided in a holistic and humanized way, promoting the well-being and quality of life of children and their families.