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.