General recommendations will be reviewed here as well as some specific guidelines for the initial treatment of complications. Additional suggestions are included above under the types of crises and laboratory and radiologic evaluation.
The general management of patients with sickle cell disease who present to the ED is primarily supportive. Patients with unstable vital signs or neurologic compromise need emergency care. The basic care of patients who present with "crisis" include the following:
Hydration. Any type of crisis may be precipitated or exacerbated by dehydration. Oral rehydration can be attempted if the patient can tolerate fluids and the episode is relatively mild. Intravenous rehydration will be needed for patients who are orthostatic or in severe pain. Fluid overload needs to be avoided in light of potential underlying cardiopulmonary disease. One-half normal saline at a rate of one to one and a half times maintenance dose is a reasonable fluid choice.
Analgesia. Sickle cell patients need prompt pain relief with adequate analgesia. The presence of narcotic addiction or the potential for its development should not alter what is prescribed in the ED. Sickle cell patients who frequently seek help in the ED will benefit from a protocol treatment plan so they know what to expect and manipulative behavior is minimized. The emergency staff should have a consistent approach to the use of narcotics in these patients. It is reasonable to administer up to two doses of narcotics in the ED over a 4- to 6-h period; if patients still have significant pain, they should be admitted to the hospital. Nonnarcotic analgesics such as acetaminophen or ibuprofen can be used for mild pain, although most sickle cell patients do not come to the ED for mild pain. Although ketorolac will not manage the pain of a severe crisis, some have found it helpful as an adjunctive treatment. Oral narcotics such as acetaminophen-codeine or oxycodone may be adequate for moderate pain. Parenteral narcotics are necessary for severe pain. A combination of meperidine and promethazine is commonly used. A typical dose would be 100 mg of meperidine and 25 mg of promethazine intravenously or intramuscularly. Some patients will have developed tolerance to narcotics and require larger doses. Intravenous morphine, sometimes via patient-controlled analgesia is useful in selected patients.
Supplemental oxygen. Supplemental oxygen is only beneficial if the patient is hypoxemic.
Cardiac monitoring. Cardiac monitoring should be considered if the patient has a history of cardiopulmonary compromise or any acute complaints referable to those systems.
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