From the Research
Painful vaso-occlusive crisis (PVOC) is the most common complication of sickle cell disease in pediatric patients, accounting for approximately 60-80% of emergency visits, as supported by recent studies 1.
Common Complications of Sickle Cell Disease
The most prevalent complications of sickle cell disease in pediatric patients include:
- Painful vaso-occlusive crisis (PVOC): characterized by severe pain in the extremities, back, chest, or abdomen due to microvascular occlusion from sickled red blood cells, affecting approximately 60-80% of patients 1.
- Acute chest syndrome: occurring in about 15-20% of pediatric sickle cell patients, characterized by fever, chest pain, respiratory symptoms, and new pulmonary infiltrates 2.
- Splenic sequestration: affecting approximately 10-30% of children, primarily those under 5 years, presenting with rapid splenic enlargement, acute anemia, and potential circulatory collapse 3.
- Aplastic crisis: occurring in roughly 3-10% of patients, typically triggered by parvovirus B19 infection, resulting in severe anemia due to temporary cessation of red blood cell production 4.
Management and Prevention
Management of sickle cell disease focuses on:
- Hydration
- Pain control with appropriate analgesics
- Oxygen therapy when needed
- Transfusion therapy for severe cases Prevention strategies include:
- Hydroxyurea therapy, which reduces crisis frequency by increasing fetal hemoglobin production 3
- Regular comprehensive care
- Prompt treatment of infections It is essential to note that the management of sickle cell disease should be individualized, and the use of a clinical pathway can improve the acute management of vaso-occlusive crisis pain in pediatric patients, as shown in a study published in 2014 5.