Sickle cell disease, a common genetic disorder in the

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1 CLINICAL ARTICLE Treating Sickle Cell Pain: An Update from The Georgia Comprehensive Sickle Cell Center * Authors: Allan Platt, PA-C, James R. Eckman, MD, JoAnn Beasley, RN, BS, and Gaynell Miller, RN, MS, CNS, Atlanta, Ga Allan Platt is Clinical Instructor, Physician Assistant Program, Emory University School of Medicine, and Program Coordinator, The Georgia Comprehensive Sickle Cell Center at Grady Health System, Atlanta, Ga. James R. Eckman is Professor of Hematology Oncology, Winship Cancer Institute, Emory University School of Medicine, and Medical Director, The Georgia Comprehensive Sickle Cell Center at Grady Health System, Atlanta, Ga. JoAnn Beasley is Newborn Screening Coordinator-Clinical Manager, The Georgia Comprehensive Sickle Cell Center at Grady Health System, Atlanta, Ga. Gaynell Miller is Assistant VP Patient Care, Grady Health System, Atlanta, Ga. *The Georgia Comprehensive Sickle Cell Center at Grady Health System was established in 1985 as the world s first 24-hour urgent care center dedicated to the needs of patients with sickle cell disease. Staffed by nurses, physicians, physician assistants, and clinic assistants, the Center provides 24-hour emergency care for patients older than 15 years without trauma and who are not pregnant. Staff use problemspecific guidelines published on the World Wide Web at org to evaluate and manage more than 3000 visits per year, and 80% of patients treated for acute pain in the Center are able to be discharged and return home after 8 hours of treatment. Questions about the care of patients with sickle cell disease can be sent by to: aplatt@emory.edu. For reprints, write: Allan Platt, PA-C, The Georgia Comprehensive Sickle Cell Center at Grady Health Center, 80 Jesse Hill Jr Dr, PO Box 109, Atlanta, GA 30303; aplatt@emory.edu. J Emerg Nurs 2002;28: Copyright 2002 by the Emergency Nurses Association /2002 $ /1/ doi: /men Sickle cell disease, a common genetic disorder in the United States, is characterized by chronic hemolytic anemia, increased severity of infections, end-organ damage, and intermittent episodes of acute and chronic pain. In this disease, crystallized hemoglobin elongates and deforms the red cell into a rigid sickle shape, causing early red cell hemolysis and blockage of small blood vessels. 1 It is estimated that 70,000 people in the United States of different ethnic backgrounds have sickle cell disease. The disease is most common in persons whose ancestors came from areas of the world where Plasmodium falciparum malaria is endemic. Inheriting one sickle gene, or sickle cell trait, provides some protection from death in young children infected with this red cell parasite. In the United States, sickle cell syndromes are present in persons of many different ethnic backgrounds, including Greek, Italian, Saudi, South American, and those from India, but they are most common in African Americans. 1 Sickle cell disease occurs in about 1 in 400 African Americans, and 8% are carriers of the sickle gene, having the sickle trait. Clinical manifestations The clinical manifestations of sickle cell disease include hemolytic anemia, increased frequency and severity of infections, tissue and organ damage, and recurrent pain episodes. Damage to the red cell from the crystallized hemoglobin reduces the survival of the cells from a normal 120 days to 12 to 15 days. The resulting hemolytic anemia causes moderate to severe reduction in the hemoglobin level, increased production of young red cells (reticulocytes) by the bone marrow, jaundice from increases in indirect bilirubin, and elevated lactic dehydrogenase. Anemia can August :4 JOURNAL OF EMERGENCY NURSING 297

2 become life-threatening when infection or other complications reduce red cell production or there is increased trapping of red cells in the spleen or liver. Bacterial infections are causes of significant morbidity and mortality in patients with sickle cell disease. Children younger than 3 years of age are at great risk for fatal sepsis and meningitis. Early loss of splenic function contributes to a greater risk for severe infections such as meningitis, pneumonia, sepsis, osteomyelitis, and salmonella. 2 Other complications are thought to result from reduced delivery of oxygen to tissues and organs. Anemia, increased blood viscosity from changes in red blood cell deformability, adherence of blood cells to vascular walls, and activation of the coagulation system all reduce the delivery of oxygen and other nutrients by the blood. Reduced blood oxygen levels, dehydration, fever, and acidosis are treatable factors that contribute to complications from crystallization of the sickle hemoglobin, causing vascular occlusion, infarctions, ischemic necrosis, and hemorrhage. 1 Damage may occur to any organ in the body, including the brain, lungs, liver, spleen, kidneys, bones, and eyes. Recurrent pain episodes are caused by ischemia resulting from reduced blood flow and the inflammation from bone marrow and muscle necrosis. These unpredictable episodes of intense pain are the most distressing symptoms for most patients. Pain episodes Self-limited, reversible pain episodes involve the extremities, back, chest, and abdomen. Many patients have mild pain episodes of short duration that can be managed at home. More severe episodes, however, may last hours, days, or weeks. 3 Patients characterize the pain as being similar to breaking all of one s bones at the same time. The frequency of pain episodes varies with each individual and is generally related to the hemoglobin phenotype, level of fetal hemoglobin, concurrent illness, physical condition, psychological factors, and social variables. Chronic damage to bones, nerves, and other tissues with inflammation and fibrosis can lead to chronic pain syndromes as well. Management of pain episodes Pain episodes are diagnosed by patient history alone because no physical or laboratory manifestations occur during uncomplicated pain episodes. The best question to ask the patient is: Is this pain typical for your pain episodes? If the pain is typical, the patient is treated for a routine pain episode. If the pain is atypical, a search for complications or other causes is started. Pain in the chest, head, and abdomen warrants a careful evaluation for complications and non sickle-related causes. A detailed history and physical examination are important for identifying correctable precipitating factors such as infection, dehydration, increased anemia, or other precipitating complications. Laboratory evaluation does not help in the diagnosis of pain episodes, but it does help identify anemia, infection, acidosis, hypoxia, and other complications. 5 Pain assessment Pain intensity is typically assessed and recorded at the beginning of treatment and at set intervals to document the maximal response to treatment. It is helpful to use patient self-report including a Visual Analog Scale, a verbal rating from 0 to 10, or the Wong Baker Faces Scale, which are rapid tools for pain assessment. 3-4 Pain therapy The treatment of pain episodes includes control of pain, intracellular hydration with hypotonic oral or intravenous fluids, correction of hypoxia and acidosis, bed rest, and treatment of underlying infection or other complications. 3 Control of pain requires accurate pain assessment and provision of adequate analgesia by giving sufficient doses of medications on a regular schedule based on the effective half-life of the medication. Pain therapy requires choosing agents that provide rapid and sustained analgesia. Pain medication should be administered in a dose that reduces the pain to a level that is acceptable to the patient and on a fixed time schedule at an interval that provides adequate analgesia without return of pain. At the Georgia Comprehensive Sickle Cell Center, we find that most patients present with an average pain level of 8 out of 10 and are ready to go home with a supply of oral analgesics after reducing their pain intensity below 4. We use this approach to maintain a steady drug level, thereby improving control of pain, minimizing complications, and 298 JOURNAL OF EMERGENCY NURSING 28:4 August 2002

3 decreasing anxiety. Most patients presenting to the hospital have failed to control the pain with oral therapy and should therefore be treated with parenteral analgesics. Intravenous therapy is the route of choice because of the rapid onset of action, predicable distribution, and minimal pain or local tissue damage from the drugs. Morphine is the opiate of choice because of flexibility in dosing forms, a predictable adverse effect profile, and proven effectiveness. 3,5 Dosing instructions for morphine are provided in Table 1. Patientcontrolled analgesia (PCA) pumps that provide constant low-dose infusion of morphine with patient-controlled demand doses provide excellent pain treatment. Similar results can be obtained by using sustained-release morphine preparations on a fixed schedule to control pain by maintaining a constant blood level and providing supplemental doses of oral or parenteral short-acting preparations on demand for breakthrough pain. Frequent adverse effects of the narcotic analgesics include itching from histamine release, nausea, vomiting, and constipation. These effects respond to concurrent administration of antihistamines, antiemetics, stool softeners, and laxatives. Unusual adverse effects, usually from excessive blood levels, include respiratory depression, hypotension, increased bladder tone, urinary retention, and decreased seizure threshold. Most experts now discourage the use of meperidine to treat sickle cell pain. When meperidine is used, a metabolite of meperidine, normeperidine, accumulates, causing irritability, reduced pain control, central nervous system stimulation, and seizures with frequent or prolonged use. Nalbuphine, a synthetic agonist-antagonist opiate, is very useful in children and adults who require infrequent pain treatment. The benefits of nalbuphine include an excellent adverse effect profile with reduced nausea, less sedation, and minimal respiratory depression. A major disadvantage is the analgesic ceiling of 20 mg every 3 hours because of the antagonist action. If pain is not controlled by this dose, nalbuphine must be stopped and a pure agonist opiate such as morphine should be titrated until the pain is controlled. Nalbuphine is contraindicated in patients with frequent or regular narcotic usage because it can cause naloxone-like, acute withdrawal symptoms when physical dependency on opiates is present. The dosage is 0.3 mg/kg up to 20 mg administered intravenously every 3 hours. TABLE 1 Pain therapy dosing for patients with sickle cell disease Morphine sulfate, 0.05 to 0.08 mg/kg (3 to 5 mg) administered intravenously every 10 minutes until pain is controlled Give the dose required to control the pain every 3 hours intravenously (NOT prn) Add ketorolac, 30 mg intravenous every 6 hours, or ibuprofen, 600 mg by mouth every 6 hours if the patient has no history of renal disease, peptic ulcer disease, or other contraindications Adjust the every-3-hour dose based on pain level and sedation Morphine patient-controlled analgesia: Morphine concentration of 5 mg/ml is used to treat adults because they generally need relatively high doses to control the pain. Loading doses of morphine up to 0.05 mg/kg every 15 minutes times 4 may be given based on need. Demand doses of to 0.04 mg/kg/dose with a 5 to 15 minute lockout are a usual range for patients with sickle cell disease. Continuous infusion doses, if necessary, may be given at night or around the clock. The usual dose ranges are between 0.01 to 0.04 mg/kg/hour. One-hour or 4-hour limits should be calculated so that the patient can get treatment for the entire period. That is, the limits should be based on the sum of the maximum demand dose plus the continuous infusion dose. Adjuvants are hydroxyzine, 25 mg administered intramuscularly every 6 hours, or promethazine, 12.5 mg every 6 hours, for nausea or anxiety. Use diphenhydramine, 25 to 50 mg every 6 hours, with morphine to prevent itching. A large number of analgesics can be used to treat the pain of sickle cell disease reliably. The important principle that can make caring for a patient with a sickle pain episode much more successful is to titrate the patient s pain level to a level that they desire. The amounts and frequency of the agonist opiate analgesics can be increased in dosage and frequency until pain control is adequate or the patient experiences excessive sedation, respiratory depression, or hypotension. Once a dose and frequency is established, the medication can be given on a regular schedule at this dose to prevent the pain from recurring at a level that is unacceptable to the patient. The dose and frequency may be different for every patient and may differ for an individual patient, depending on the severity of the pain. 3,5 August :4 JOURNAL OF EMERGENCY NURSING 299

4 TABLE 2 The ABC s for managing sickle cell pain A Assessment of the pain (use a pain assessment tool) B Believe the patient s level of pain C Complications or cause of pain (look for complications) D Drugs and distraction Pain Medication (opiates and NSAIDs, if no contraindications) Distraction with music, TV, relaxation techniques E Environment, rest in quiet area with privacy F Fluids (Hypotonic-D 5 W or D5 with 0.25 normal saline solution) Use fixed dosing; give on a time schedule; no prn dosing for pain medications A number of important medical interventions may decrease the duration of the pain episode. Oral hydration with water and juices or intravenous hypotonic fluids such as intravenous dextrose 5% with water (D 5 W) is important for 2 reasons. First, decreasing the sodium concentration outside the red cell causes water to go into the red cell. This dilutes the intracellular hemoglobin concentration, which markedly reduces the tendency of the sickle hemoglobin to crystallize. 6 The second reason is that almost all persons with sickle cell anemia and many with hemoglobin SC and hemoglobin S β-thalassemia have a renal concentrating defect that causes them to continuously lose water from the kidneys. If they stop drinking water because of the pain or other illness, free water dehydration rapidly develops. By administering replacement fluids, the duration of pain can be reduced. 6 At our Center, patients are hydrated with D 5 W at 250 ml per hour (children receive 5 ml/kg/h) for 4 hours in the absence of congestive heart failure, renal failure, or hyponatremia. The fluids should be changed to D 5 W or D 5 W with 1 4 or 1 2 normal saline solution at a slower rate, 125 ml per hour (for children, 1 to 2 ml/kg/h), for second and subsequent liters, based on electrolytes. If venous access cannot be obtained, oral hydration should be used. We do not use lower extremities for hydration except in extreme emergencies because of the increased frequency of leg ulcers. It is important to administer oxygen to patients with hypoxia or dyspnea because deoxygenation is the primary cause of sickling. Pulse oximetry can be used to diagnose and monitor oxygen saturation if the patient s anemia is not too severe. Low oxygen saturation in symptomatic patients must be investigated with arterial blood gases (ABGs), chest radiographs, and pulmonary testing. Oxygen use for routine pain episodes is not beneficial. In our Center, we keep patients for 8 hours with ongoing treatment and pain assessments. If the patient is then able to manage their pain at home, a 48-hour supply of opiate and nonsteroidal antiinflammatory drugs (NSAIDs) is given, with instructions to return if the pain escalates or if fever or other new symptoms develop. We admit the patient if his or her pain level is not adequately resolved in 8 hours, or if a complication such as fever, sequestration, infiltrate, dropping hematocrit level, or priapism is present. The ABC s of sickle cell pain management are listed in Table 2. Complications Pain episodes may continue until underlying medical problems are completely resolved. A second concern is that the pain episode may be an antecedent to a more serious sickle complication. A number of potentially life-threatening complications can present as pain episodes, or can develop as a pain episode is being treated. Table 3 details common emergency patient presentations and laboratory, radiologic, and treatment options. Fever Fever in a patient with sickle cell disease is an emergent situation. It indicates sepsis until proven otherwise. All our patients with fever receive a septic work-up, including blood cultures, complete blood cell count with differential, reticulocyte count, urinalysis, and chest radiograph. After cultures are obtained, we give the patient a long-acting parenteral antibiotic with coverage against pneumococcus. Some patients may be allowed to go home from the emergency department with next day follow-up if no high-risk features appear on history, physical examination, or laboratory evaluation, but only if outpatient follow-up can be assured JOURNAL OF EMERGENCY NURSING 28:4 August 2002

5 TABLE 3 Common problems of patients with sickle cell disease in the emergency department Signs and symptoms Diagnostic tests and differential Treatment options Symmetric hand-foot Hand foot syndrome first manifestation of Fluids and pain management swelling, infant sickle cell disease Chills and fever Sepsis, pneumonia, osteomyelitis Empirically treat with antibiotics until cultures are do a CBC, WBC differential (check for known; prevent infections with immunization and elevated bands and total WBC count), prophylactic penicillin until age 6 years blood cultures Headache Stroke, aneurysm, meningitis Treat etiology CT scan, MRI-MRA, LP Chest Pain, dyspnea, Chest syndrome, pneumonia do a chest Treat empirically with antibiotics and transfusion cough radiograph, ABG Abdominal pain and Splenic or hepatic sequestration, gallstones Transfusion for sequestration; surgery for gallstones swelling ultrasound or CT, CBC and chemistry profile New weakness, Stroke do a CT or MRI-MRA Transfuse acutely and chronic transfusion program for paresthesias, prevention difficulty talking Pain in extremities, Pain episode look for precipitating causes Hydration with oral or IV water; good pain managelow back typical such as infection, dehydration, acidosis; ment; hydrea may prevent pain episodes pain episode pain do a CBC, reticulocyte count, chemistry profile, and UA Weakness, lethargy, Aplastic episodes, do a CBC with reticulocyte Transfusion support until bone marrow responds pallor count (low hematocrit with low reticulocyte count) Acute decline after Multi-organ system failure evidenced by renal, Transfusions can be life saving routine pain hepatic, failure, ARDS, DIC episodes multiple organ failure Increasing jaundice Increased hemolysis, hepatitis, or bile duct Treat etiology; administer folate, 1 mg daily, to all obstruction; do a CBC, reticulocyte count, patients for red cell production demands chemistry profile, hepatitis screen; consider a gallbladder ultrasound Increasing ED visits Consider inadequate pain management, Provide good pain management; consider hydroxyurea for pain infection, increased anemia; assess psycho- therapy to prevent pain; case management social aspects Focal bone pain Consider bone infarction or osteomyelitis; For bone infarction and AVN, treat with long acting if hip or shoulder pain, consider AVN; NSAIDs and decreased weight bearing do a CBC and a radiograph of the area ABG, Arterial blood gas; ARDS, acute respiratory distress syndrome; AVN, avascular necrosis; CBC, complete blood cell count; CT, computed tomography; DIC, disseminated intravascular coagulation; LP, lumbar puncture; MRI-MRA, magnetic resonance imaging-magnetic resonance angiography; UA, urinalysis; WBC, white blood cell count. Abdominal pain Abdominal pain may be caused by splenic sequestration. This serious complication is second only to infections as a cause of death in infants with sickle cell disease. This event usually occurs between the ages of 4 months and 3 years, but it may happen at any age with hemoglobin SC or S β-thalassemia disease. During sequestration episodes, sickle cells are trapped in the spleen, causing rapid fall in the hemoglobin level and enlargement of the spleen. Sequestration events may be triggered by infection or can occur with no apparent antecedent. The onset of signs and symptoms is very rapid and consists of weakness, left upper quadrant August :4 JOURNAL OF EMERGENCY NURSING 301

6 Case scenario You are the nurse on duty in the emergency department when a 19-year-old man with severe pain in his arms, legs, and back is brought in by his college roommate. You do the initial triage and find out that the student has sickle cell anemia and the pain had begun 6 hours earlier. He also has a 24-hour history of coughing, fever, and chills. His last pain episode was 8 months ago. Physical evaluation shows a well-developed man holding his arms and legs, writhing in pain. His vital signs are as follows: pulse, 110; respirations, 20; blood pressure, 130/85; temperature, 39.0 C; and pulse oximetry SaO 2, 90%. His tongue is furrowed, showing dehydration. His pain intensity is 9 out of 10 on a visual analog scale. You ask if his pain is typical for his usual episodes, and he states that it is more severe than usual and the cough is unusual. The patient has been taking ibuprofen, 600 mg every 6 hours, without much relief. You immediately assess the patient as high priority, and care for his sickle cell pain episode is started immediately. The ED physician evaluates the patient quickly. You review the history, physical examination, and orders for treatment and diagnostic evaluation. The laboratory orders include a complete blood cell count and reticulocyte count to determine the bone marrow response to the chronic hemolytic anemia. A chemistry profile, sputum for culture, blood culture, and urinalysis also are ordered. A chest radiograph and nasal oxygen at 2 L per hour are ordered because of the cough, fever, and hypoxia. A venous line is inserted and D 5 W is administered at 250 ml per hour for the first liter, then 125 ml per hour. The pain management orders are as follows: nalbuphine (Nubain), 20 mg administered intravenously every 3 hours. Nalbuphine is an agonist/antagonist that has respiratory depression ceiling. Nonsteroidal antiinflammatory drugs are held to observe for fever. You assess the patient s pain score and administer the medication slowly, intravenously. You come back in 15 minutes to reassess the pain score and ask about adverse effects, and make the patient as comfortable as possible. Your patient returns from radiology with a negative chest radiograph. His pain level has decreased during the past hour to a 5 out of possible 10 on the visual analog pain scale. His laboratory results are typical of the patient s baseline values. The physician decides to admit the patient for intravenous administration of antibiotics, pain management, and monitoring. The ED nurse has contributed significantly to the care of this patient in recognizing the patient s need for pain relief and supporting him throughout his episode. abdominal pain, fatigue, dyspnea, pending shock, tachycardia, pallor, enlarging spleen, and a falling hemoglobin and hematocrit. The diagnosis is based on the enlarging spleen and a reticulocyte count that is equal to or higher than the patient s baseline. Treatment consists of admission to an ICU with aggressive blood transfusions. Emergent splenectomy is occasionally required. Other sickle cell causes of abdominal pain include hepatic sequestration and gallstones. Chest pain Chest pain, cough, fever, or hypoxia in a patient with sickle cell disease requires evaluation with a chest radiograph and ABGs. The presence of an infiltrate is the hallmark of the acute chest syndrome (ACS), an important cause of morbidity and mortality. The etiology of ACS includes infection with viruses and bacteria, bone marrow necrosis with fat embolism, and de novo sickling in the pulmonary blood vessels. 7 Pneumonia caused by atypical bacteria is a common infectious cause of ACS. Bronchoscopy is often diagnostic and may occasionally be therapeutic. Patients should be treated with transfusions and antibiotics that cover atypical bacteria and Streptococcus pneumoniae. The occurrence of ACS in the postoperative period may be reduced by simple transfusion to a hemoglobin of 10 g/dl preoperatively and use of incentive spirometry postoperatively. Headache Acute headache or transient neurologic symptoms could indicate a hemorrhagic or thrombotic stroke or meningitis. A rapid neurologic evaluation with consideration of a computed tomography scan and/or lumbar puncture is essential. 5 Atypical pain The worst pain episode ever with other evidence of multiorgan failure is best treated with rapid blood transfusions or exchange transfusion. Transfusion of packed red cells is not indicated for the treatment of routine pain episodes. Patients may require transfusions if symptomatic anemia develops during treatment for a pain episode. Patients with sickle cell disease who have strokes, transient ischemic episodes, chest infarction, multi-organ system failure, or 302 JOURNAL OF EMERGENCY NURSING 28:4 August 2002

7 Resources for patients with sickle cell disease: Web sites, books, and CD-ROMs The Sickle Cell Information Center Web site at is a comprehensive site for providers and patients. The book Problem Oriented Management of Sickle Cell Syndromes by James Eckman, MD, and Allan Platt, PA-C, is available at this site. Ballas, SK. Sickle Cell Pain: Progress in Pain Management (vol. 11). Seattle (WA): International Association for the Study of Pain; 1998, 398 pp, hardbound, ISBN Guideline for the Management of Acute and Chronic Pain in Sickle Cell Disease, Glenview (IL): American Pain Society; Address: American Pain Society, 4700 W Lake Ave, Glenview, IL 60025; phone (847) ; fax (847) ; info@ ampainsoc.org; Web site: CD-ROM: The Wellcome Trust Presents: Topics in International Health Sickle Cell Disease. Available at: or sequestration of red cells in the spleen or liver are candidates for exchange transfusions. Preventive care and patient education Prophylactic daily penicillin is instituted from birth to 6 years for all patients with sickle cell disease to prevent pneumococcal sepsis. 8 Guidelines for discharge instructions in Table 4 can help prevent other pain episodes. To prevent pain episodes, the only approved preventive strategy is oral hydroxyurea. Be sure to mention it to your patients. Opiate addiction myths and issues Narcotic addiction is a rare occurrence, usually affecting 2% to 5% of the sickle cell patient population. It takes several days of continuous narcotics to cause physical dependence and tolerance to opiate medications. Severe pain requiring narcotics should not be confused with a true addiction, where the patient s life adversely revolves around a favorite drug. Pseudo-addiction occurs when the patient is continually undertreated and returns frequently with unresolved pain. This situation can be managed with adequate doses of long-acting agents. TABLE 4 Discharge teaching points for sickle cell patients 1. Encourage daily oral folate supplementation, which will help the bone marrow keep up red cell production. 2. Encourage follow-up care with sickle cell experts (see the Sickle Cell Information Center at for clinic locations). 3. Encourage increased water intake to prevent dehydration (this should be 8 glasses a day for adults). 4. Advise patients to avoid temperature extremes of too hot or too cold. 5. Advise patients to rest and not to overdo it. 6. Advise patients that bone pain should be treated with daily NSAIDs. 7. Advise patients on how to manage the sedation and constipation accompanying the use of opiates. 8. Advise patients to slowly taper opiates when they are taken for several days. Otherwise, they will experience withdrawal symptoms. 9. Offer patient education handouts. Several are available for printing and distribution at REFERENCES 1. Steinberg M. Management of sickle cell disease. N Engl J Med 1999;340: Ander DS, Vallee PA. Diagnostic evaluation for infectious etiology of sickle cell pain crisis. Am J Emerg Med 1997;15: Benjamin LJ, Dampier CD, Jacox A, Odesina V, Phoenix D, Shapiro, BS, et al. Guideline for the management of acute and chronic pain in sickle cell disease (American Pain Society clinical practice guidelines series, No. 1). Glenview (IL): American Pain Society; Beyer JE, Platt AF, Kinney T, Treadwell M. Practice guidelines for the assessment of children with sickle cell pain. J Soc Pediatr Nurse 1999;4: Eckman JR, Platt AF. Problem oriented management of sickle cell syndromes (1991, revised 2001). Available at: URL: SCInfo.org/prod04.htm 6. Rosa RM, Bierer BE, Thomas R, Stoff JS, Kurskall M, Robinson S, et al. A study of induced hyponatremia in the prevention and treatment of sickle cell crisis. N Engl J Med 1980;303: Vichinsky EP, Styles LA, Colangelo LH, Wright EC, Castro O, Nickerson B. Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative study of sickle cell disease. Blood 1997;89: Overturf GD. American Academy of Pediatrics. Committee on Infectious Diseases. Technical report: prevention of pneumococcal infections, including the use of pneumococcal conjugate and polysaccharide vaccines and antibiotic prophylaxis. Pediatrics 2000;106(2 Pt 1): August :4 JOURNAL OF EMERGENCY NURSING 303