Sickle cell information center protocols (excerpt)

Edited by James Eckman, MD, and Allan Platt, PA-C

Emergency Department, Ambulance, and Triage Guidelines

Sickle cell patients are frequently seen in emergency departments (EDs) for evaluation of new symptoms and for pain management. These patients are at high risk for life-threatening events, and their triage level should be high. Patients’ level of pain should be assessed and believed. Ambulance staff should transport the patient to an emergency facility with a knowledgeable staff. Time should not be used in the field obtaining an IV unless there is a long transport. Nasal oxygen can be administered if the patient is dyspneic. ED staff performing triage and evaluation should be aware of high-priority problems:

Fever. Fever is a high-priority problem that could potentially be fatal sepsis. Patients with Hb SS are functionally asplenic and at very high risk for bacterial infections. This should not be masked with antipyretics until the source is known. Patients should be brought into the clinic immediately for a full evaluation. Early treatment with empiric parenteral antibiotics after cultures are obtained can be life-saving. All patients should receive pneumococcal vaccine at ages 2 and 6, and every 10 years. Prophylactic penicillin should be instituted at birth and should continue until the child is 6 years old or longer in individual cases.

Chest pain or dyspnea. Could be potentially fatal acute chest syndrome and needs a full evaluation with a chest X-ray. Administer oxygen if the patient is dyspneic or hypoxic. Order incentive spirometers for all hospitalized patients to prevent chest syndrome. Chest syndrome may need treatment with blood transfusions.

Acute headache. Could be a hemorrhagic or thrombotic stroke, or meningitis. A rapid evaluation with consideration of a CT scan and/or lumbar puncture.

Acute abdominal pain. Could be splenic/hepatic sequestration, cholecystitis, bowel infarction, or any other cause of acute abdominal pain. Narcotic analgesics may mask the signs and pain of an acute abdominal problem.

Transient neurologic symptoms, even in children, should be considered a stroke and early transfusion can prevent further episodes. A painless limp may indicate hemiparesis.

The "worst" pain crisis ever with other evidence of multiorgan failure should be treated immediately with blood transfusions or exchange transfusion.

Weakness, dizziness, and increasing fatigue can indicate an increasing anemia from aplastic crisis, sequestration of red cells in the spleen or liver, and from increased hemolysis. Chronic hemolysis will cause an elevated indirect bilirubin, lactate dehydrogenase, and reticulocyte count. A "normal" or low reticulocyte count and a falling hematocrit is an indicator of aplastic crisis. A reticulocyte count is the best indicator of the bone marrow effectiveness in making new red cells.

Atypical pain. Always ask the patients if the pain they are experiencing is normal pain crisis. If it is atypical, suspect another cause of the pain or a complication causing the pain episode. Focal bone pain and tenderness may be a bone infarction or osteomyelitis.

Priapism. Priapism is a painful sustained penile erection, and if it is not treated promptly, it can result in permanent impotence.

IV fluids and access. Hypotonic IV fluids such as D5W should be used to treat pain events to drive free water into red cells. Never use the foot or lower leg as an IV site because of the potential for leg ulcers. Many patients have ports for venous access. Use the proper Huber needle and strict aseptic technique to access a port.

Addiction. Narcotic addiction is a rare occurrence, usually effecting 2%-5% of the sickle cell population. Negative attitudes should not be generalized to all sickle cell patients. (It takes several days of continuous narcotics to cause physical dependence and tolerance. This should not be a concern in the ED.)

Routine labs. Emergency evaluation should include a complete blood count with white blood cell differential, a reticulocyte count, lactate dehydrogenase, direct/indirect bilirubin, and urinalysis. Other lab values may be required, depending on the presenting problem.

References

• Barrett-Connor E. Bacterial infection and sickle cell anemia. Medicine 1971; 50:97-112.

• Embury SH, Hebble RP, Mohandas N, et al. (eds). Sickle Cell Disease: Basic Principles and Clinical Practice. New York City: Raven Press Ltd.; 1991.

• Embury SH, Garcia JF, Mohandas N, et al. Effects of oxygen inhalation on endogenous erythropoietin kinetics, erythropoiesis, and properties of blood cells in sickle cell anemia. N Engl J Med 1984; 311:291-295.

• Gaston MH, Verter JI, Woods G, et al. Prophylaxis with oral penicillin in children with sickle cell anemia. N Engl J Med 1986; 314:1,593-1,599.

• Guy RB, Gavrilis PK, Rothenberg SP. In vitro and in vivo effect of hypotonic saline on the sickling phenomenon. Am J Med Sci 1973; 266:267-277.

• Powars D, Overturf G, Weiss J, et al. Pneumococcal septicemia in children with sickle cell anemia. JAMA 1981; 245:1,839-1,842.

• Reid CD, Charache S, Lubin B, et al. Management and Therapy of Sickle Cell Disease. NIH Publication No. 95-2117. Bethesda, MD; 1995.

• Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regiments in the perioperative management of sickle cell disease. N Engl J Med 1995; 222:206-213.