Treatment

The treatment of sickle cell depends in large part on the severity of the phenotype. Patients with known sickle cell trait should undergo genetic counseling to help assess the risk to any offspring. Patients who exhibit clinical manifestations are managed expectantly. All symptomatic patients should have their care managed by a multidisciplinary hematology team.

Management of acute pain crisis is one of the more problematic areas of sickle cell treatment. As previously stated, these patients present with severe pain without a clear etiology. The mainstay of pain treatment is the use of anti-inflammatory medications and opioid analgesics. Nonpharmacologic treatment includes cutaneous stimulation, hot and cold packs, massage, and relaxation techniques. A consistent pain scale instrument (e.g., visual pain analog scale) is helpful to quantify the amount of pain present. Liberal use of analgesia is recommended. Side effects are the only limiting factor with opioid administration (16).

ACS is the second most common cause of hospital admission in sickle cell disease. In addition to adequate analgesia and broad-spectrum antibiotic therapy, exchange transfusion has been shown to modulate the severity of the disorder. Hydroxyurea has been shown to prevent recurrences of ACS as well as acute pain crisis. The drug appears to work by reducing the number of sickled RBCs in circulation (17). The indications for this therapy are controversial. Due to adverse reactions, use of hydroxyurea in children is also debated.

Treatment of CVA centers primarily on prevention. In the event of acute stroke, immediate transfusion therapy is undertaken. The goal of transfusion is to reduce the Hb-S concentration to less than 30%. Patients thereafter are maintained on continual transfusion therapies with chelation therapy for five years. CVA prevention utilizes noninvasive transcranial Doppler to monitor high-risk vasculature (e.g., internal carotids). Patients with abnormal Doppler velocities are treated with blood transfusions to reduce Hb-S concentrations. Patients aged 2 to 16 years with sickle cell should be screened yearly for vessel disease (11,12,14,15).

Other treatments for sickle cell disease include prophylactic transfusion therapy, glucocorticoids, nitric oxide, and bone marrow transplants. The efficacy of many of these treatments is still being evaluated.

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