and raise hemoglobin,7 and it is associated with increased survival rates in patients with SCD.8 However, it can also cause myelosuppression, or bone marrow suppression; therefore, hematology labs must be monitored regularly. When taking this hazardous agent, contraception is required for both males and females, and patients should avoid live vaccines.9 L-glutamine oral powder, or Endari®, is approved for adults and children older than 5 years with SCD who have failed hydroxyurea or for concurrent use. Glutamine can reduce acute complications related to SCD such as pain crises. Each 5g dose is mixed with 8 ounces of cold or room-temperature liquid or 6 ounces of food.10 Glutamine 1,500 mg is also available as an over-thecounter product, but this is generally not recommended since it has not been approved by the U.S. Food and Drug Administration (FDA).
Pain Management: Opioids and Other Pain Relievers VOCs usually require IV opioids or patient-controlled analgesia to tolerate the severe pain.6 Acute moderate pain is usually managed with a less potent opioid, such as codeine or hydrocodone, in combination with an NSAID, such as ibuprofen or acetaminophen. For acute severe pain, stronger opioids, such as intravenous morphine or fentanyl may be used.
Chelation Therapy SCD patients receive regular blood transfusions, and within each unit of packed red blood cells is about 250 mg of iron. After 20 of these transfusions, the body accumulates excess iron due to saturation of transferrin. Iron overload can cause organ damage. Chelation therapy is used to manage the iron load that patients receive from blood transfusions. Oral chelation therapy has recently become available: deferasirox (Exjade®, Jadenu®) and deferiprone (Ferriprox®).11
Transplantation Bone marrow or stem cell transplantation is currently the only real cure for SCD. Unfortunately, it is not utilized widely due to the high costs and risks that accompany such an invasive treatment. There is significant toxicity as well as serious side effects and even death associated with this procedure. Additionally, for these transplants to work effectively, the bone marrow must be a close match.1
New Pharmacotherapies The FDA recently approved two new drugs: crizanlizumab-tmca (Adakveo®) and voxelotor (Oxbryta™). Adakveo® is an intravenous infusion-administered monoclonal antibody developed by
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