Case Description
An 11-year-old, 40kg, boy with sickle cell anemia (HbSS), asthma, and obstructive sleep apnea was transferred to our End-stage Lung Failure Program for consideration for ECMO bridge to lung transplant (LTx). Upon initial presentation at the local facility, he was diagnosed with ACS due to SARS-CoV-2 and received supplemental oxygen, corticosteroids, convalescent plasma, and remdesivir, but his hypoxemia worsened despite the escalation of maximum conventional mechanical ventilation and proning. His chest X-ray showed diffuse bilateral airspace opacities and a large pneumomediastinum from the significantly high ventilator pressures (Figure 1A). To sustain life, he underwent cannulation of the right internal jugular with a 27 Fr dual-lumen, single bicaval catheter on hospital day 15 for VV-ECMO. Over the next three weeks, there was no evidence of lung recovery with multiple failed VV-ECMO weaning trials. Therefore, he was transferred to our institution on ECMO day 43. To maximize rehabilitation, a tracheostomy was placed for liberation from sedation. While at our institution, a total of six exchange transfusions were performed to strictly target a hemoglobin S (HbS) level ≤15% to minimize the effect of sickled red blood cells (RBCs) (Figure 2). Routine transfusions were performed per our institutional ECMO protocol with packed RBCs and Fresh frozen plasma (FFP) with extended blood group matching for the E, Kell, and Duffy antigens to minimize the risk of alloimmunization. His VV-ECMO course was uncomplicated without major bleeding or thrombotic events. Surveillance echocardiograms showed stable right ventricular pressure and function. The intensive physical rehabilitation and maximal enteral nutrition via gastrostomy tube significantly improved his deconditioning and allowed for weaning of VV-ECMO support. He was successfully decannulated after 71 days of VV-ECMO. On post-decannulation chest X-ray, he had bilateral patchy airspace opacities but with marked improvement from his pre-ECMO appearance (Figure 1B). Due to his complicated course, hydroxyurea therapy was re-initiated while on ECMO and maximized with an increase of fetal hemoglobin (HbF) levels from 0.3% to 11.5% during three months of therapy. He was also transitioned to enoxaparin therapy for an additional 2 weeks upon ECMO decannulation and then to apixaban during hospitalization given an increased risk of thrombosis due to SCD and SARS-CoV-2. After completing inpatient rehabilitation, he was ultimately discharged home with chronic mechanical ventilation five months after his initial presentation.