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Sickle cell disease can lead to multiple health complications and an increased risk of death. Of these complications, acute chest syndrome is the top cause of mortality, and the complication providers are most familiar with.

Due to health advances, patients with sickle cell disease are living longer, meaning more patients are experiencing these health complications and facing increased morbidity and mortality.

A new article in the Journal of Blood Medicine highlights the different issues that affect children with sickle cell disease—issues that often continue into adulthood. The authors aim to better educate providers on managing systemic complications of sickle cell disease beyond acute chest syndrome to reduce suffering and harm among this patient population.

They stress that multidisciplinary management of health complications should be emphasized, while routine care provides opportunities to prevent and identify sickle cell disease complications. For example, a routine ophthalmologic exam can rule out sickle cell retinopathy, and updated vaccinations can provide added protection against encapsulated organisms.

Below are some of the complications that the authors discussed.

Splenic Complications
Splenic complications in children can include acute splenic sequestration, impaired immunity to encapsulated organisms, and life-threatening infections such as meningitis, osteomyelitis, pneumonia, and sepsis. Splenic palpation and signs of anemia are important components of anticipatory guidance for parents, with the need for urgent red cell transfusion if signs of circulatory failure appear.

Reproductive Health
Patients with sickle cell disease usually have delayed puberty. Sickle cell disease can also contribute to particularly painful menstrual cycles. In addition, patients with sickle cell disease can face high rates of sperm abnormalities and erectile dysfunction.

Central Nervous System (CNS) Complications
Cerebral vasculopathy is the most common CNS complication in children with sickle cell disease. A less common complication that may present with acute neurologic symptoms is acute cranial bony infarction associated with intracranial hemorrhage and thrombosis [1].


[1] Ogu, U. O., Badamosi, N. U., Camacho, P. E., Freire, A. X., & Adams-Graves, P. (2021). Management of sickle cell disease complications beyond acute chest syndrome. Journal of Blood Medicine, Volume 12, 101–114.