G373(P) When parents and professionals agree: an example of shared decision making in a case of spinal muscular atrophy type 1. (25th October 2020)

• Record Type: Journal Article
• Authors: Shahid, S; Punjabi, N; Sahdev, N; Khalid, H; Khalid, U; Punjabi, K; Gour, A
• Is Part Of: Archives of disease in childhood. Volume 105(2020)Supplement 1
• Page Start: A134
• Page End: A134
• Publication Date: 2020-10-25
• Subjects: Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/archdischild-2020-rcpch.320
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : Introduction: The media has covered many cases on when parents disagree with the opinions of healthcare professionals, most notably in the case of Charlie Gard. This particular case of a female infant with spinal muscular atrophy (SMA) type 1 is an example of when the decisions of parents align with the advice of healthcare professionals and attempts to uncover what factors may have contributed to this cohesion. SMA is the term given for a group of genetic disorders leading to muscle atrophy and weakness. Type 1 is the most severe phenotype, presenting under 6 months of age and with a 68% mortality rate within 2 years of life. Case Synopsis: Female infant M was born at term, with no concerns, to consanguineous parents. At 7 weeks old, her mother took her to the GP with concerns of a lack of limb movement. Examination found multiple neurological abnormalities, including absent rooting reflex, global hypotonia and upgoing Babinski reflexes. M was thus diagnosed with SMA Type 1. At 16 weeks old, M was admitted to the Paediatric Intensive Care Unit with respiratory deterioration requiring intubation. M's parents decided that a tracheostomy would make her quality of life worse, even if it did prove life-prolonging. They opted to palliate on non-invasive ventilation. Discussion: The decision to palliate a patient is burdensome to all involved and is influenced by many factors. Evidence shows that parents of those with SMA assess success of care not on outcome, but on factors including positive communication with the healthcare team. Furthermore, those who highly rate their experience of this communication have lower levels of long-term grief. Ensuring parental understanding at each stage is crucial for shared decision-making. The parents of this patient were involved in every step. Their refusal of a tracheostomy despite the potential for added life is not a common decision and as both parental and professional opinions here aligned, reflects the honest and realistic communication between both parties. Conclusion: This case shows how effective communication between the medical team and parents can improve the parental experience of paediatric palliation and help attain shared decision-making.