P2.C052: Complications of Acute Chest syndrome and Septic Shock in a Pediatric Sickle Cell Patient requiring ECMO

Introduction: Sickle Cell disease (SCD) is a hemoglobinopathy notable for vaso-occlusive crises, acute chest syndrome (ACS) and other organ dysfunction. ACS occurs most commonly from respiratory infections (viral vs.bacterial). Viral, mycoplasma, and streptococcus pneumoniae are the most common pathogens.
Nearly all sickle cell patients with Hgb SS develop functional asplenia and are at increased risk of bacterial infection especially with encapsulated bacteria. It is crucial preventative measures are taken to minimize risk.
This case denotes severe complications of septic shock secondary to RSV, pneumococcal pneumonia and ACS in a pediatric patient with SCD requiring VA ECMO.

Case Description: 9yo M with Hgb SS admitted from outside ER to the PICU for acute respiratory failure secondary to RSV and superimposed pneumococcal pneumonia with ACS.
The patient completed the vaccine series up to age 4, with the exception of the flu vaccine or pneumococcal vaccines PCV20 or PPSV23. No hydroxyurea use prior to admit.
On arrival the patient was unresponsive, sedated, actively being hand ventilated with bag/mask, and had poor perfusion. He was immediately given fluid resuscitation, dilute epinephrine boluses, and intubated. He then had a PEA arrest with ROSC after 12 minutes of CPR per PALS. He required continuous vasoactive support for poor perfusion. Despite initial improvement, he then progressed to worsening lactic acidosis prompting cannulation to VA ECMO (right internal jugular/right carotid). Initial ECHO demonstrated severe left and right ventricular systolic dysfunction with LV dilation. Peak lactic acid was 14.1 mmol/L. Post-cannulation, he was noted to have dusky appearing phalanges. Balloon atrial septostomy was performed the next day for left heart decompression. He was converted to VV ECMO 12 days later and decannulated successfully on day 19. No neurologic deficits were present at discharge.

Discussion: This patient’s SCD likely contributed to rare complications with sepsis and ECMO. Vaso-occlusion and high vasoconstriction needs prior to and during ECMO probably led to the development of dry gangrene of upper and lower extremities requiring multiple digit and metatarsal amputations (Figure 1).
Goal S% was < 20-30%. Our hematology team assessed the continued need for vaso-occlusive crisis prevention with scheduled partial exchange transfusions and hydroxyurea.

Conclusion: Preventative medicine is vital. High risk populations >2 years of age such as those with SCD are recommended by the CDC to receive 20- or 23-valent Pneumococcal vaccine if not previously given. Penicillin prophylaxis is recommended coverage for patients < 2yo. Hydroxyurea use in the pediatric population accounts for increased survival, primarily through decrease in mortality associated with ACS and infection.
In ACS, severity and rate of worsening pulmonary function and increased need of respiratory support determines the need for simple vs. exchange transfusion. Exchange transfusion is recommended for severe declines in respiratory function.