(859) Changes in Palliative Care Consults and Technology Dependence in Spinal Muscular Atrophy Patients

Introduction: Spinal muscular atrophy (SMA) is a progressive neuromuscular disorder characterized by progressive muscular atrophy, weakness, and paralysis. Treatment is largely supportive and requires a coordinated team approach, including integrated palliative care. In 2016, Spinraza (nusinersen) was approved to treat SMA symptoms. While a promising therapy, it is vital to balance hope for a cure with realities of progressive illnesses. This study's goal is to evaluate changes in palliative care team involvement and technology dependence with novel treatments.

Methods: The Pediatric Health Information System (PHIS) database was queried for pediatric patients (age ≤18) admitted to a hospital in the United States with an SMA diagnosis, identified by ICD 9 and 10 codes. Palliative care consultation, tracheostomy dependence, and surgical feeding tubes were identified. Patients admitted prior to Spinraza approval (2004-2015) were compared to those admitted after Spinraza approval (2017-2023).

Results: 16,429 patients with a diagnosis of SMA were identified; 10,190 were admitted between 2004-2015, and 6,239 between 2017-2023. Between the two groups, there was an increase in palliative care consultations (2.5% to 6.5%). There were increases in technology use, including tracheostomy (20.3% to 23.4%), surgical feeding tubes (48.4% to 62.8%), and mechanical ventilator use during the hospital stay (42.6% to 49.4%). Patients tended to be older (6.44 vs 7.08), and have more complex chronic conditions (2.22 vs 2.35). The mortality rate during hospitalization decreased (6.0 to 4.5%). Of 139 patients identified as receiving Spinraza therapy, they tended to be younger (average age 2.99), with a higher mortality rate (6.5%), but an increased use of a palliative care team (10.8%). Rates of technology dependence were similar to the 2017-2023 group (22.3% had tracheostomies and 66.2% had surgical feeding tubes).

Conclusions: This study points to increased dependence on medical technology among SMA patients, despite the promise of innovative treatments. The increase in palliative care consultations reported reflects the evolving nature of the field and highlights the importance of quantifying outcomes when discussing novel therapies with families. A holistic approach is imperative for each child with a progressive illness.