Sickle Cell Information Center Protocols

Edited by James Eckman, M.D. and Allan Platt, PA-C

Sickle cell patients are frequently seen in emergency rooms for evaluation of new symptoms and for pain management. These patients are 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 a emergency facility with 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. Emergency room staff performing triage and evaluation should be aware high priority problems:

Fever - Fever is 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 lifesaving. All patients should receive pneumococcal vaccine at age 2, 6, and every 10 years. Prophylactic penicillin should be instituted at birth and should continue until the child is six 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 any other cause of acute abdominal pain. Narcotic analgesics may mask the signs and pain of an acute abdomen

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 increase hemolysis. Chronic hemolysis will cause an elevated indirect bilirubin, LDH 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 patient 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 can result in permanent impotence.

IV Fluids and Access- Hypotonic IV fluids like 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 Emergency Room).

Routine Labs - For emergency evaluation should include a CBC with WBC differential, a reticulocyte count, LDH, Direct/Indirect Bilirubin, and Urinalysis. Other lab values may be required depending on the presenting problem.

For a full article on ER management see: Platt A, Eckman JR, Beasley J, Miller G. Treating sickle cell pain: An update from the Georgia comprehensive sickle cell center. J Emerg Nurs 2002 Aug;28(4):297-303 Full Text is available as PDF file for Adobe download by clicling here or see the article online at:  http://www2.us.elsevierhealth.com/scripts/om.dll/serve?action=searchDB&searchDBfor=art&artType=fullfree&id=a125268

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Copyright © 1997 Sickle Cell Information Center
Last modified: September 12, 2002