P1.015: Management of Pediatric Sickle Cell Disease and Multi-Organ Dysfunction With ECMO: A Single- Center Experience

Introduction: Extracorporeal Membrane Oxygenation (ECMO) is a rescue therapy for severe cardiopulmonary impairment. Its use and outcomes for shock and Multi-Organ Dysfunction (MOD) are unexplored in pediatric Sickle Cell Disease (SCD). SCD is associated with hemolytic anemia, hypercoagulopathy, vaso-occlusion, and vasculopathy that complicate ECMO management in SCD. This case series describes our center’s experience with VA ECMO in children with SCD.

Case Description: 1. 5 y/o with HbSS presented with DIC and MOD. ECMO was initiated for cardiac arrest and patient was found to have S. Pneumoniae bacteremia. Due to ongoing shock, MOD, and severe neurological injury, ECMO was discontinued and patient died.
2. 4 m/o with Trisomy 21, HbSS, and complete AV Canal post-repair developed biventricular failure and refractory Junctional Ectopic Tachycardia (JET). ECMO was started for cardiogenic shock and discontinued due to clinical improvement.
3. 14 y/o with HbSS and lupus presented with pancreatitis, transaminitis, AKI, and DIC. ECMO was initiated for respiratory failure, fulminant liver failure, renal failure, and shock, complicated by cerebral intraparenchymal hemorrhages. Due to ongoing shock and severe neurological injury, ECMO was discontinued and patient died.
4. 19 y/o with HbSS and Autoimmune Hemolytic Anemia (AIHA) presented with pain crises, Acute chest syndrome (ACS), cardiogenic shock, and subsegmental pulmonary embolism prompting ECMO initiation. Patient had refractory anemia from AIHA and multiple antibodies limiting transfusions. After decannulation, she developed renal failure, ARDS, encephalopathy and died.
5. 17 y/o with HbSC presented with pain crisis, transaminitis, DIC, and AKI. ECMO was initiated after cardiac arrest, CRRT initiated for hyperkalemia, plasmapheresis for DIC and anemia, and Anakinra initiated for HLH. She was decannulated after 5 days but succumbed to refractory HLH and disseminated fungal infection.

Discussion: Our institution provides care for a large SCD population but only five patients underwent ECMO in the last 15 years. Unlike the conventional use of ECMO for ACS, our patients required support for refractory shock with MOD with VA ECMO. There was significant acute organ failure and mortality risk upon admission, noted by high PRISM III and VIS scores. Challenges included anticoagulation in prothrombotic state with ongoing hemolysis due to SCD, complicated by MOD-induced coagulopathy. In our patients, ECMO was complicated by renal failure requiring dialysis, intracerebral hemorrhage, and severe infection. This case series highlights the distinct characteristics of MODS in SCD and special considerations for utilizing ECMO in this high-risk population.

Conclusion: Patients with SCD requiring ECMO support are rare with high mortality, as evident in our cohort with high disease severity scores and ECMO complications. Due to history of multiple blood transfusions, and risk of DIC and coagulopathy with MOD, ECMO use in SCD presents unique challenges in anticoagulation. Further studies describing ECMO use in SCD are necessary to improve outcomes.