Sickle Cell Disease: Treatment

In the acute pain setting, analgesics, warm compresses, and oral and intravenous fluids are appropriate interventions. Complementary therapies, such as hypnosis, relaxation techniques, and biofeedback, may also be helpful.

Preventive Strategies

Comprehensive and multidisciplinary care is essential. Education of both patient and family may help prevent complications of the disease.

Influenza and pneumococcus vaccines should routinely be used. Pneumococcal prophylaxis (oral penicillin V 125-250 mg twice daily) should be taken continuously by children with SCD until age 5. Children with a history of splenectomy or severe pneumonia may need further prophylaxis.

Folic acid should be taken in doses of 1 mg daily.

Transcranial Doppler may identify children at risk for stroke. Those at higher risk should receive blood exchange transfusions.²

Routine eye exams should monitor for proliferative retinopathy.

Assessment for chronic complications, including chronic lung and kidney disease, should be performed periodically, especially in older children and adults.

Analgesia

Narcotics are often required for pain relief. Initial boluses with patient-controlled anesthesia for later pain control are appropriate strategies.

Morphine sulfate and hydromorphone are first-line agents. Hydromorphone is more concentrated, and therefore beneficial in fluid-restricted patients. Morphine synthetics, such as fentanyl, can also be used. Meperidine is not recommended.

Nonnarcotic analgesia may also be helpful. Ketorolac is especially helpful for bone pain and is as effective as meperidine.³ Note: Ulcer prophylaxis is needed. Tramadol can be used for outpatient management and is less likely than narcotics to lead to dependence.

Lesser-potency analgesics, including nonsteroidal anti-inflammatory drugs (NSAIDs), are likely important adjuncts to the above narcotic agents, but they have not been well-studied in this context.

Antibiotics

Infections cause nearly 50% of SCD-induced mortality. Patients with febrile episodes, without other symptoms, need broad-spectrum antibiotic coverage (eg, ceftriaxone). Depending on the severity of the fever and prophylactic penicillin status (in children), antibiotics can be administered intravenously or intramuscularly, and on an inpatient or outpatient basis.

Meningitis, bacteremia, osteomyelitis, urinary tract infections, and acute chest syndrome require specific antibiotic regimens.

Blood Transfusions

Transfusions may be of the simple or exchange type. It is important that patients are not transfused acutely above a hemoglobin of 10 g/dL, which can lead to increased blood viscosity. Strategies and formulas have been devised to help calculate appropriate volumes to be transfused in both children and adults.

Simple transfusions restore blood volume and oxygen-carrying capacity in individuals with SCD.

Partial-exchange transfusions may be required during a severe acute complication (eg, acute chest syndrome) to prevent increased blood viscosity. As mentioned previously, exchange therapy lowers the risk of stroke2 and may also prevent other end-organ damage and reduce iron loading, as compared with simple transfusion.

Transfusion therapy is not indicated for uncomplicated SCD pain events.

Alloimmunization, antibody formation after blood transfusion, is approximately 6 times more common in persons with SCD compared with other anemias, and the cost to ensure more strictly matched blood can be high. A racially and ethnically diverse blood supply can help reduce the likelihood of alloimmunization.

Other Treatments

Hydroxyurea stimulates the production of hemoglobin F. In addition, hydroxyurea may reduce the number of acute pain episodes and acute chest syndrome attacks.

Erythropoietin's ability to stimulate production of hemoglobin F is less clear, but if hydroxyurea produces a less than adequate stimulus, substitution or addition of erythropoietin may be tried empirically.⁴

Hematopoietic cell transplantation and gene therapy are potentially curative treatment strategies, but remain experimental.

Magnesium supplementation may reduce the number of acute pain episodes, though more thorough study of its role is underway. Inhaled nitric oxide may have a role in treatment of SCD, and poloxamer 188 is promising for relief of acute pain episodes,⁵ but both of these treatments need further study.