A recently reported case series details 3 cases involving young high school athletes with sickle cell trait (SCT) who died from exertional collapse associated with sickle cell trait (ECAST). The case series was reported in the journal Pediatric Emergency Care by Katherine Shoush Cools, MD, of the University of North Carolina, Chapel Hill, North Carolina, and colleagues.
“Although exertional related death (ERD) is an uncommon cause of death in the pediatric population, identifying athletes at risk for ERD may be helpful in reducing the occurrence as counseling and urgent medical evaluation may be prioritized in these athletes,” Dr Cools and colleagues explained in their report.
The patients in this case series had SCT, were all of ages 14 to 16 years, and had collapsed while in a sports practice session. Each had presented at a hospital with metabolic acidosis that was not responsive to fluid replenishment, in addition to other features. They each developed kidney failure and had a cause of death of, or diagnosis supporting, ECAST with fatal exertional rhabdomyolysis. In all cases, autopsy findings revealed sickle cell vaso-occlusion in various organs and in some cases muscles or vasculature.
The first case involved a 14-year-old White male who developed muscle weakness during football practice and collapsed, before being taken to an emergency department. He presented with tachycardia, tachypnea, fever, drowsiness, and metabolic acidosis. He was treated with ice packs and intravenous fluids but had to be admitted due to exertional heat stroke and dehydration. His condition worsened, and he went into cardiac arrest. He also developed disseminated intravascular coagulation and acute myoglobinuric kidney failure secondary to rhabdomyolysis. His condition deteriorated despite treatments, and he developed multiorgan system failure and died within 24 hours of his initial collapse.
The second case Dr Cools and colleagues presented involved a 16-year-old Black male who developed cramping during football practice. Emergency medical services evaluated him at the site but detected no injuries or illnesses. He went home but became unresponsive and was taken to a hospital. He received treatment for dehydration, metabolic acidosis, hyperkalemia, hyperglycemia, and rhabdomyolysis, but his conditioned worsened. He developed acute myoglobinuric renal failure from rhabdomyolysis, and he died within 24 hours of arrival at the hospital.
The third case involved a 14-year-old Hispanic male who had stomach pain and collapsed during baseball practice. His SCT status had been unknown at the time but was identified postmortem. At the hospital, this patient had metabolic acidosis, rhabdomyolysis, acute kidney injury, and exertional heat stroke. His condition worsened while he was treated with fluids and received a laparotomy for abdominal compartment syndrome. He ultimately developed multiorgan system failure, and his death occurred within 48 hours of first showing symptoms.
“All 3 of the athletes in our case series died within 48 hours of symptom onset, demonstrating how lethal ECAST can be,” Dr Cools and colleagues concluded in their report. They also mentioned the importance of recognizing the difference between ECAST and exertional heat stroke, as the treatment approaches for each differ.
Disclosures: Some authors have declared affiliations with or received grant support from the pharmaceutical industry. Please refer to the original study for a full list of disclosures.
Cools KS, Crowder MD, Kucera LK, et al. Sudden death in high school athletes: a case series examining the influence of sickle cell trait. Pediatr Emerg Care. 2022;38(2):e497-e500. doi:10.1097/PEC.0000000000002632
This article originally appeared on Hematology Advisor