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Pediatrics, Sickle Cell Disease: Treatment & Medication

Author: Nedra R Dodds, MD, Medical Director, Opulence Aesthetic Medicine
Coauthor(s): Hosseinali Shahidi, MD, MPH, Assistant Professor, Departments of Emergency Medicine and Pediatrics, State University of New York and Health Science Center at Brooklyn
Contributor Information and Disclosures

Updated: Nov 21, 2007

Treatment

Emergency Department Care

The emergency physician must weigh all etiologies of possible precipitants when evaluating the presumed painful crisis.

• In the ED, the mainstay of therapy for vaso-occlusive crises is hydration and analgesia.
• Hydration should be administered to replace ongoing losses and meet maintenance needs, as well as correct preexisting deficits. Administer intravenous (IV) isotonic sodium chloride solution at a rate of 1-1.5 times the maintenance rate if oral hydration cannot be sustained.
• Base analgesia on the severity of symptoms and the response to therapy at home before the patient's presentation in the ED.
  • Mild pain can be controlled by giving ibuprofen or acetaminophen (with or without codeine).
  • If pain is more severe, patients can be given morphine, meperidine, hydromorphone, or oxycodone. Some pediatric patients have benefited from high-dose IV methylprednisolone. Ketorolac may also be considered. It is a potent NSAID indicated for <5-d management of moderately severe acute pain that requires analgesia at opioid level.
• Patients presenting with variable signs of respiratory distress suggestive of acute chest syndrome should be given early and aggressive therapy. Fluids should be monitored judiciously to avoid pulmonary edema. Use of an incentive spirometer may prove helpful.
  • Antibiotic therapy should cover Pneumococcus species, Haemophilus influenzae type b, and Mycoplasma pneumoniae, with drugs such as cefuroxime, ceftriaxone, and erythromycin, respectively.
  • Provide oxygen and respiratory support to relieve hypoxemia.
  • When respiratory distress is moderate to severe, especially with progressive anemia, transfusion is appropriate to increase the oxygen-carrying capacity and to decrease the percentage of sickle hemoglobin, thereby diminishing the tendency for intrapulmonary vaso-occlusion.
• For the patient with acute splenic sequestration, therapy is primarily directed at restoring intravascular volume with appropriate isotonic fluids. Severe anemia requires transfusion therapy. The size of the spleen tends to return to its baseline state with an associated increase in hemoglobin concentration over several days.
• Patients with suspected stroke require immediate exchange transfusion sufficient to decrease the percent of sickle hemoglobin to less than approximately 25%.
  • A single volume-exchange transfusion of packed RBCs, reconstituted with fresh-frozen plasma (FFP), to a hematocrit of approximately 40% is an appropriate emergency measure.
  • If the patient has occlusive disease of the large vessels, long-term transfusion therapy is required to prevent recurrent stroke.
  • If signs of increased intracranial pressure develop, hyperventilation is warranted, as are anticonvulsants to control seizure activity.
• Treat priapism with analgesia, hydration, and transfusion therapy sufficient to decrease the sickle hemoglobin level to less than 30%.
  • The choice between a simple RBC transfusion and exchange transfusion depends on the initial hematocrit.
  • This level should not be increased above 35% to avoid hyperviscosity and an increased tendency for sickling.
• Transfusions may be necessary for the patient with aplastic crisis, though the condition often spontaneously resolves within 5-10 days.
  • Caution should be exercised to isolate the child at the time of aplasia, because the human parvovirus B19 is responsible for more than 80% of crises and highly contagious.
  • The patient should be isolated from all vulnerable groups, such as immunocompromised children, children with hemolytic anemia, and pregnant women.

Consultations

• Always call the patient's primary pediatrician if the patient presents with severe complications of his or her sickle cell disease.
• Consult a pediatric hematologist for any patient requiring an exchange transfusion as therapy for their disease process.
• If the patient presents with neurological deficits, a pediatric neurologist may be consulted after the initial evaluation and workup.
• Multiple pediatric tertiary care centers equipped with specialized sickle cell services have emerged over the last few years. Children with sickle cell disease who become acutely ill may best be served by these facilities.

Medication

Analgesics

Medications used selectively for analgesia should be chosen based on the severity of pain. Maximum doses and frequencies of administration should be used for milder medications prior to using stronger pain medications.

Ibuprofen (Advil, Motrin)

DOC for patients with mild-to-moderate pain. Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis. Possesses anti-inflammatory, analgesic, and antipyretic properties. Do not use for abdominal pain.

Acetaminophen (Tylenol)

For temporary relief of mild pain due to vaso-occlusive crisis. DOC for pain in patients with documented hypersensitivity to aspirin or NSAIDs, with upper GI disease, or taking oral anticoagulants.

Acetaminophen and codeine (Tylenol with codeine)

About 10% administered codeine demethylated to morphine, which may account for analgesic activity. Adjust dose to severity of pain and response. Tolerance to codeine may develop with continued use; incidence of adverse effects is dose related. High doses in children fail to give commensurate relief of pain; merely prolongs analgesia and associated with increased incidence of adverse effects. Must determine number of tablets per dose and maximum tablets per day.
Tylenol #2 contains 15 mg codeine/325 mg acetaminophen; Tylenol #3, 30 mg codeine/325 mg acetaminophen; and Tylenol with codeine elixir, 12 mg codeine/120 mg acetaminophen per 5 mL. Respiratory depression indicates serious overdose.

Ketorolac (Toradol)

Potent NSAID indicated for <5-d management of moderately severe acute pain that requires analgesia at opioid level. Inhibits prostaglandin synthesis by decreasing activity of cyclooxygenase, decreasing formation of prostaglandin precursors.

Morphine sulphate (Duramorph, MS-IR)

DOC for analgesia because of reliable and predictable effects, safety profile, and ease of reversibility with naloxone.
Various IV doses used. Commonly titrated to desired effect. Analgesia achieved by action at CNS opioid receptors. Route determined by severity of pain and initial response. Nausea and vomiting prevalent due to stimulation of chemoreceptor trigger zone. Delayed onset of action when administered epidurally for PCA because of poor lipid solubility and slow access to receptor sites. Do not use rapid IV administration. Continuous infusion should be administered with 0.1-1 mg/mL.

Meperidine (Demerol)

Analgesic with multiple actions similar to those of morphine; may produce less constipation, smooth muscle spasm, and depression of cough reflex than similar analgesic doses of morphine.
In most institutions, DOC for vaso-occlusive pain. Adjust dose to severity of pain. IM preferred if repeated doses required. If IV required, decrease dose and give by slow injection. Use only for 3-4 d to avoid accumulation of the active metabolite, normeperidine, which may cause CNS toxicity (eg, seizures).

Oxycodone and acetaminophen (Percocet)

Drug combination indicated for relief of moderate to severe vaso-occlusive pain in sickle cell disease. Retains at least half its analgesic activity when administered orally; therefore, can be used as an adjunct to outpatient care. Adjusted dose to severity of pain and response.

Oxycodone and aspirin (Percodan)

Drug combination indicated for relief of moderate to severe vaso-occlusive pain in sickle cell disease. Retains at least half its analgesic activity when administered orally; therefore, can be used as an adjunct to outpatient care. Adjust dose to severity of pain and response.

Cytoreductive agent

Increases production of fetal hemoglobin.

Hydroxyurea (Droxia)

Chemotherapy agent that allows sickled shaped blood cells to assume the shape and characteristics of fetal hemoglobin by becoming larger and less adherent, thus allowing travel through the blood vessels to occur easier. Shown to reduce frequency of painful crises and to reduce need for blood transfusions in patients with recurrent moderate-to-severe painful crises (ie, 3 episodes in preceding year).

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