BEYOND LIMITS: SIX YEARS OF COMMUNITY-BASED PALLIATIVE CARE EFFORTS FOR A CHILD WITH SPINAL MUSCULAR ATROPHY (SMA) TYPE-1

Abstract

Purpose: Spinal Muscular Atrophy (SMA) is a rare neuromuscular disease that results in muscle weakness and atrophy, where symptoms appear at the first six months of life and most die by two years. Pediatric palliative care (PPC) prevents and relieves suffering in children with life-threatening illnesses. The study aims to enumerate the challenges encountered in rendering PPC for a child with SMA Type I and describe the best available community-based PPC practices in a resource-limited country. Case Description: CM was referred to community-based PPC when she was 15 months old. At that time, she needed respiratory aid via ventilator through tracheostomy, feeding aid via percutaneous endoscopic gastrostomy tube, and she was confined to bed. She was taken cared for by the PPC team for six years and nine months delivered through home visits and continued through telemedicine during the pandemic. The care team comprises doctors, nurses, physical therapists, social workers, counselor, and administrative support. Outcomes: Upon referral to PPC, examinations were conducted on biological, social, emotional, and spiritual dimensions. Interprofessional goal setting yielded the following consensual goals: Provide adequate control for impairments and optimum nutritional support, assist with family's needs, maintain a sense of peace among family members and care providers, and achieve the best possible quality of life. Interventions were based on the biopsychosocial-spiritual model (BSM) delivered across professions. The care team initiated creative ways to convert limited resources to optimum PPC. Throughout the provision of best available care to CM, the set goals were generally achieved, with positive feedback from the family and the benefit of surpassing the patient’s life expectancy. Conclusion: Children with conditions like SMA-Type 1 and their families may benefit from PPC employing a BSM approach.