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The National Institutes of Health advises that optimal care for patients with sickle cell disease (SCD), including preventive care, is best achieved through treatment in clinics that specialize in the care of SCD. All patients with SCD should have a principal health care provider, who should either be a hematologist or be in frequent consultation with one. ^[39]

When severity of the patient’s crisis is assessable, self-treatment at home with bed rest, oral analgesia, and hydration is possible. Individuals with SCD often present to the emergency department (ED) after self-treatment fails. Do not underestimate the patient’s pain. Failure to treat acute pain aggressively and promptly may lead to chronic pain syndrome. According to the 2003 BCHS acute painful crisis guidelines, analgesia should be administered with 30 minutes of entering the hospital, with the goal of achieving effective pain control by 60 minutes. ^[32]

If patients with SCD crisis are being transported by emergency medical services (EMS), they should receive supplemental oxygen and intravenous hydration en route to the hospital. Some areas have specialized facilities that offer emergency care of acute pain associated with SCD; many EDs have a standardized treatment plan in place.

Pain management should include four stages: assessment, treatment, reassessment, and adjustment. While considering the severity of pain and the patient’s past response, follow consistent protocols to relieve the patient’s pain.

The goals of treatment are symptom control and management of disease complications. Treatment strategies include the following seven goals:

• Management of vaso-occlusive crisis
• Management of chronic pain syndromes
• Management of chronic hemolytic anemia
• Prevention and treatment of infections
• Management of the complications and the various organ damage syndromes associated with the disease
• Prevention of stroke
• Detection and treatment of pulmonary hypertension

Allogeneic bone marrow transplantation (BMT) can cure SCD, but it is difficult to decide which patients should be offered BMT. Many risks are associated with BMT, and the risk-to-benefit ratio must be assessed carefully. With the advent of cord blood stem cell transplantation and with the development of less immunoablative conditioning regimens, perhaps BMT will gain wider acceptance and use. The lack of availability of a matched donor may limit the utility of BMT.

An expert panel has released evidence-based guidelines for the treatment of SCD, including a strong recommendation that hydroxyurea and long-term, periodic blood transfusions should be used more often to treat patients. Other recommendations include the following ^{[40, 41]} :

• Use of daily oral prophylactic penicillin up to age 5
• Annual transcranial Doppler examinations between the ages of 2 and 16 years in patients with sickle cell anemia
• Long-term transfusion therapy to prevent stroke in children with abnormal transcranial Doppler velocity (≥200 cm/s)
• In patients with sickle cell anemia, preoperative transfusion therapy should be used to increase hemoglobin levels to 10 g/dL
• Rapid initiation of opioids for the treatment of severe pain associated with a vasoocclusive crisis
• Use of analgesics and physical therapy for the treatment of avascular necrosis