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Sickle cell disease (SCD) management in children focuses on acute complications. This review details handling acutely unwell pediatric SCD patients, improving survival rates.
Area of Science:
- Hematology
- Pediatrics
- Genetics
Background:
- Sickle cell disease (SCD) is an inherited blood disorder affecting hemoglobin synthesis.
- It primarily impacts individuals of African, Caribbean, Indian, Mediterranean, and Middle Eastern descent.
- Characterized by anemia, infections, pain, and organ dysfunction, childhood is a critical period for SCD morbidity and mortality.
Purpose of the Study:
- To discuss acute complications of sickle cell disease in children.
- To provide guidance on managing acutely unwell pediatric patients with SCD.
Main Methods:
- Literature review focusing on acute complications and management strategies for pediatric sickle cell disease.
Main Results:
- While life expectancy has improved, childhood remains a high-risk period for mortality and morbidity in SCD.
- Acute complications require prompt and specific management to prevent severe outcomes.
Conclusions:
- Effective management of acute complications is crucial for improving outcomes in children with sickle cell disease.
- Focusing on the acutely unwell child is essential for reducing childhood mortality and morbidity in SCD.
Abstract:
Sickle cell disease is a recessively inherited condition in which synthesis of haemoglobin is abnormal. The disease, which occurs mainly in people of African, African-Caribbean, Indian, Mediterranean and Middle Eastern descent, is characterised by chronic anaemia, susceptibility to infection, bouts of severe pain and organ dysfunction. While the life expectancy for patients has improved, from a median survival age of 14 years in the 1970s among those homozygous for the sickle haemoglobin gene to survival into the mid-40s, childhood remains a period of peak mortality and morbidity. Here, we discuss the acute complications of sickle cell disease in children, concentrating on the management of the acutely unwell child.