Pharmacy & Therapeutics - April 2008 - (Page 240) CONTINUING EDUCATION CREDIT Table 2 Treatment Options for Complications from Acute Sickle Cell Disease Complication Pain Mild to moderate Therapy Codeine with acetaminophen or aspirin • orally every three to four hours Hydrocodone with acetaminophen • orally every three to four hours Morphine sulfate immediate-release (MSIR) • IV every two to four hours • orally every three to four hours Hydromorphone • IV or orally every three to four hours Empirical therapy coverage for: Streptococcus pneumoniae, Salmonella, Haemophilus influenzae, gram-negative enterics Streptococcus pneumoniae, Neisseria meningitides, H. influenzae S. pneumoniae, Legionella, Mycoplasma pneumoniae, respiratory syncytial virus, Chlamydia pneumoniae Salmonella, Staphylococcus aureus, S. pneumoniae Escherichia coli, other gram-negative enterics Moderate to severe Infection Fever without a source (rule out sepsis) Meningitis Chest syndrome Osteomyelitis/septic arthritis Urinary tract infection IV = intravenously. From National Institutes of Health. The Management of Sickle Cell Disease, 4th ed. revised, June 2002.22 Hydroxyurea’s potential benefits should be weighed against the risks of bone marrow suppression, which is reversible when the drug is discontinued. Complete blood counts are recommended every four to eight weeks after the hydroxyurea dose is stabilized. Pain Medications Pain, which is usually attributed to ischemia from the obstruction of blood vessels by sickled cells, is the most common symptom of SCD. It can be acute or chronic, and it varies among individuals in its frequency and intensity. Pain is the primary cause of hospitalization in patients with SCD, which is why proper management of pain in this population is essential.17 The general approach to the treatment of pain is to identify the causes, which include infection, extreme temperature, and emotional stress. Usually, however, there is not an identifiable cause, and the pain crisis occurs without warning. Milder pain is treated with general nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and ketorolac tromethamine (Toradol, Roche) or analgesics like acetaminophen and tramadol (Ultram, PriCara). Severe painful episodes should be treated with parenteral opiates at frequent intervals, not on an as-needed basis. In a study comparing intermittent intravenous (IV) injections and patient-controlled analgesia (PCA), PCA reduced the length of stay and was as efficacious as the injections.18 Acute episodes of pain may also be treated with IV hydration, and milder episodes may be treated with oral hydration— regardless of the patient’s state of hydration—to slow or stop the sickling process, which can be promoted by dehydration.19 After the pain has diminished and has tapered off, an oral analgesic can be given. The opiate drugs that have been studied to treat SCD include morphine, hydromorphone, fentanyl (Duragesic, PriCara), and codeine-related agents.20 Morphine is considered the drug of choice for the treatment of acute sickle cell pain (Table 2), whereas meperidine (Demerol, Sanofi-Synthelabo) should be avoided because of the increased risk of seizures in patients with renal dysfunction, which can occur in patients with SCD. Infection Prophylaxis Patients with SCD are at an increased susceptibility to pneumococcal infection primarily because of the development of functional asplenia, which can occur at as early as six months of age.21 In the absence of a functional spleen, the organ can no longer serve its immunological functions of clearing bacteria from the blood and synthesizing antibodies, circumstances that can lead to an increased frequency of infection.22 In the PRophylaxis with Oral Penicillin in children with Sickle Cell Anemia (PROPS) study, when infants received prophylactic penicillin between three months and three years of age, pneumococcal infection rates decreased by 84%.23 PROPS II evaluated the consequences of discontinuing penicillin prophylaxis at five years of age, and there was no difference in the rates of infection in the penicillin arm, compared with the placebo arm (relative risk = 0.5).21 On the basis of the PROPS and PROPS II results, children younger than three years of age should receive 125 mg of penicillin orally twice daily, and children between three and five years of age should receive 250 mg of penicillin orally twice a day. For patients who are allergic to penicillin, erythromycin 240 P&T® • April 2008 • Vol. 33 No. 4
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