Maximizing Survival: VA-ECMO As a Lifeline in a Critically Ill Pediatric Patient with Sickle Cell Disease, Acute Chest Syndrome, and Streptococcal Toxic Shock Syndrome

Streptococcal toxic shock syndrome (STSS) is a rapid-onset medical crisis marked by refractory shock and multi-organ failure, with a high mortality rate. This condition often leads to death shortly after symptom onset. In patients with sickle cell disease (SCD), the simultaneous occurrence of acute chest syndrome (ACS) with STSS amplifies the clinical challenge, exacerbating the disease's complexity. Encouraging outcomes have been observed in various pediatric cases with the application of veno-arterial extracorporeal membrane oxygenation (VA-ECMO), leading to its incorporation into septic shock management guidelines. However, reports on VA-ECMO's efficacy in pediatric sickle cell patients with sepsis are scarce, highlighting an area ripe for further clinical exploration and understanding.

The objective was to elucidate the therapeutic challenges and outcomes of VA-ECMO as a critical intervention in the acute management of ACS and STSS secondary to Group A Streptococcus infection in SCD.

In this inquiry, we utilized historical patient data through a comprehensive chart review. This allowed us to distill valuable insights and patterns from the clinical course that could enhance patient care in future scenarios.

Drawing from our review, we present the case of a fully vaccinated five-year-old female with SCD, hemoglobin SS phenotype, who exhibited symptoms including cough, fever, headache, abdominal pain, and labored breathing. Non-adherence to prescribed hydroxyurea (HU) was noted. Clinical examination revealed diminished breath sounds, intercostal retractions, and jaundice. The patient received a blood transfusion and pain management in the emergency department. A head CT scan ruled out stroke, while a chest X-ray confirmed ACS. Initial antibiotic therapy with azithromycin and ceftriaxone was escalated to cefepime due to rapid respiratory decline, leading to intubation.

A CT chest scan confirmed necrotizing pneumonia and pleural effusion, which tested positive for Group A Streptococcus. Echocardiography showed reduced left ventricular function, and the patient was placed on VA-ECMO after vasopressors failed to stabilize her condition. The patient remained on VA-ECMO for 10 days. Her recovery was complicated by acute kidney injury, hyperbilirubinemia, and thrombosis in the internal and middle jugular veins. The renal condition improved with intravenous fluids, and diuretics were administered for fluid overload. Imaging revealed a distended gallbladder and elevated direct bilirubin levels were managed with ursodiol, leading to improvement.

After stabilizing cardiac and respiratory functions, the patient was extubated and had the chest tube removed. She improved in the general ward and was eventually discharged to inpatient rehabilitation. At a six-month follow-up, the patient had returned to her baseline health, with no further sickle cell crises or emergency department visits. The patient also resumed long-term HU treatment with improved adherence.

Acute chest syndrome and STSS, though rare, can profoundly impact the complex pathophysiology of sickle cell disease (SCD) and lead to severe medical crises. The use of VA-ECMO in pediatric SCD cases is scarcely documented. This situation underscores the need for healthcare providers to evaluate the potential benefits of VA-ECMO for managing severe sickle cell complications, carefully weighing its advantages against the associated risks.

Raj:Jazz: Speakers Bureau; Pfizer: Honoraria, Speakers Bureau; US world meds: Consultancy; Sanofi: Consultancy.