Treatment

Management of patients with SCD focuses on both acute and chronic illness. It is important to remember that progressive end-organ involvement occurs over time. Of primary importance are ocular, pulmonary, cardiac, hepatic, and renal changes. These—as well as issues of growth, development, and sexual maturation—should be routinely monitored at well-patient visits to the pediatrician/hematologist. In the ED, it is the acute illness that confronts the physician. Complications arising from SCD are managed based upon the nature of the pathology. The ED care of patients with SCD presenting in crisis is generally supportive in nature. The mainstays of therapy have been hydration/fluid replacement, analgesia, supplemental oxygen, and blood transfusion.

FLUID REPLACEMENT All types of sickle cell crisis can be precipitated or exacerbated by dehydration. If the episode is relatively mild, oral fluid replacement may

be effective. Patients with severe pain, orthostasis, or change in vital signs will likely require intravenous hydration. The rate of hydration is generally at 1 2; times maintenance (about 2250 mL/M2 per day). Patients need to be carefully monitored to avoid fluid overload and iatrogenic cardiac failure, especially those with cardiopulmonary crisis/acute chest syndrome. Electrolytes should be checked and fluid choice adjusted appropriately.

ANALGESIA Analgesia is of paramount importance and should always be sufficient to control the pain. "Standard doses" of narcotic medication may not be enough for the individual patient with SCD. Although narcotic dependence or enhanced narcotic-seeking behavior may be a problem for rare patients, this should never affect the decision to control severe pain. The establishment of treatment guidelines and protocols for the ED management of patients with SCD can help minimize manipulative behavior and change expectations. The approach should be consistent. With the advent of patient-controlled analgesia (PCA) pumps, narcotic usage and dosing can be carefully controlled and reviewed. In the ED, narcotics should be chosen with potential side effects, drug interactions, and serum half-lives in mind.

Oral narcotic preparations such as Tylenol #3, Vicodin, or Percocet are usually adequate for moderate types of pain. Parenteral narcotics are usually required for more severe pain. Demerol (meperidine) in often used, in combination with an antiemetic, as an intramuscular injection. This may be useful, but only in limited doses, since meperidine can lower the seizure threshold. Also, repeated intramuscular injections run the risk of forming sterile abscesses and scarring, which can further exacerbate painful crises; they are therefore to be avoided in treatment of prolonged, painful crises. Intravenous narcotic administration is preferable, and a PCA pump is ideal. Morphine (at 0.15 mg/kg per dose to a maximum of 10 mg) every 2 to 3 h and hydromorphone (Dilaudid, at about 0.02 to 0.05 mg/kg per dose) are good choices. Dilaudid has a slightly more rapid onset and slightly shorter duration of action as compared with morphine. Approximately 1.3 mg of Dilaudid is considered equivalent to about 10 mg of morphine. Both medications should be adjusted to provide about 3 to 4 h of pain relief. The addition of nonsteroidal anti-inflammatory drugs has been shown to be of some benefit; however, these are to be used cautiously or not at all in patients with renal compromise.

OXYGEN Supplemental oxygen has long been considered an important part of treating SCD crises. More recent investigations have shown that it is beneficial only if the patient has demonstrable hypoxia.

CARDIAC MONITORING Cardiac monitoring is necessary if the patient has a history of known cardiac disease, signs and symptoms of acute chest syndrome, or cardiopulmonary compromise. When aggressive fluid replacement is warranted, cardiac monitoring also becomes important.

TRANFUSION THERAPY Blood transfusions are often necessary in children undergoing splenic sequestration crisis and severe aplastic crisis. In addition, transfusions may be needed in the management of cerebrovascular accident (CVA), priapism, or as perioperative management prior to surgery. Naturally, transfusion carries risk: alloimmunization, HIV infection, hepatitis, volume overload, and iron toxicity (secondary to repeated transfusions). Transfusion can also markedly decrease erythropoiesis.

The decision to institute transfusion therapy can be difficult. Clearly, in splenic sequestration crisis with hypotension, blood should be transfused. A hematologist should be consulted emergently to help direct therapy. An initial transfusion of 10 mL/kg of packed RBCs can be started in the ED.

Aplastic crisis may also require a transfusion of packed RBCs. When aplastic, splenic sequestration, or hemolytic crises occur, exchange transfusion is preferable. If the serum hemoglobin is greater than 6 g/dL, transfusion is rarely indicated.

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