Treating Adult Patients With Sickle Cell Anemia

Morey Blinder, MD, professor of medicine at Washington University in St. Louis, is an adult hematologist whose practice is focused on patients with nonmalignant hematologic disorders. A large percentage of these patients have sickle cell anemia and require frequent transfusions, ultimately resulting in iron overload in approximately one-third.

Transitioning from a pediatric to adult sickle-cell anemia program is associated with some challenges, which require a personalized approach, explains Blinder. This is even more important as patients with sickle cell anemia begin to live longer.

Pain management in adults with sickle cell disease is a large focus of care and is much more complicated than in pediatric patients, as there are more causes of pain. Opioid dependence is more common, as is chronic bone disease from avascular necrosis, a serious complication. In addition, vaso-occlusive crisis can occur.

Iron overload associated with a lifetime of heavy transfusion-dependence results in organ system dysfunction, adds Blinder. Early on, excessive iron storage has no symptoms. However, at some point, the patient will develop abnormal iron results.

Clinical symptoms often don’t begin until the problem is more severe, such as when the patient shows evidence of liver dysfunction. Right upper quadrant pain is an underappreciated symptom in these patients, says Blinder. In this situation, iron chelation therapy should be considered to reduce ferritin levels.