As a frequent complication and the top cause of mortality among individuals with sickle cell disease (SCD), acute chest syndrome represents a key concern in the management of these patients. However, a range of additional complications and comorbidities may warrant increased attention in this population.

In a paper published in 2021 in the Journal of Blood Medicine, Ugochi Ogu and colleagues described the various issues affecting children with SCD, as highlighted below. These complications often persist into adulthood and intersect with the usual aging-related comorbidities that affect the general population.1

Central nervous system (CNS) complications. Due to “progressive inflammation and oxidative endothelial damage within intracranial vessels,” children with SCD may experience cerebral vasculopathy. This represents the most common CNS complication in children and can lead to mild cognitive or behavioral deficits as well as more severe neurologic morbidity and death. Accordingly, regular screening for vasculopathy is recommended for patients beginning at 2 years of age, along with “early initiation of disease modifying therapy to mitigate anemia and endothelial damage,” the authors noted in their report.1

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Cardiovascular complications. Hyperdynamic blood flow and left ventricular hypertrophy results in heart murmur in many children with SCD, and iron overload may lead to pulmonary hypertension and cardiomyopathy that become clinically apparent in adulthood. However, screening echocardiograms are generally only recommended if indicated by relevant clinical findings.1

Splenic complications. Patients may have an elevated risk of infections including pneumonia and meningitis due to impaired immunity to encapsulated organisms. Therefore, febrile illnesses in infants and underimmunized children should be treated as medical emergencies.1

Although the morbidity and mortality related to these infections have declined substantially with the initiation of prophylactic antibiotics in infancy in addition to routine vaccines, some findings have shown suboptimal prescription rates for prophylactic antibiotics in children with SCD.1-3

Other potential splenic complications include acute splenic sequestration of red cells or platelets and autoplenectomy, the latter of which is more common in older children and teens compared with children who started disease-modifying therapy early. “Reviewing splenic palpation and signs of anemia are an important component of anticipatory guidance for parents, with the need for urgent red cell transfusion if signs of circulatory failure,” the authors stated in the paper.1

Gastrointestinal complications. Vaso-occlusion in intra-abdominal vessels or acute vaso-occlusive events within hepatic parenchyma can lead to acute abdominal pain in SCD and are managed with hydration and pain control. Intense abdominal pain and transaminitis may also result from intrahepatic sequestration, and transfusion may be indicated for patients with symptomatic anemia.

Cholelithiasis and cholecystitis represent other differential diagnoses for acute abdominal pain in SCD. These cases require urgent evaluation by gastrointestinal and surgical teams to determine treatment needs.

Peptic ulcers related to nonsteroidal anti-inflammatory drug use and constipation due to the use of narcotic pain medications are frequent complaints in children with SCD and may warrant consultation with a gastroenterologist.