Sickle Cell Information Center Protocols

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

by Lewis Hsu MD, PhD

Chest pain in patients with sickle syndromes can occur from pulmonary infarction, pneumonia, myocardial ischemia, chest wall pain from bone infarction, or as part of the diffuse pain of pain crisis. Esophageal disease, peptic ulcer disease, and gall bladder disease can occasionally cause chest pain. The Acute Chest Syndrome is defined as a new infiltrate on chest radiograph in a patient with sickle cell disease, with possible associated features of acute pleuritic chest pain, fever, prostration, or leukocytosis. This medical emergency, a common cause of hospitalization, is usually caused by pulmonary infarction from sickling in adults and infection in children.

Documentation of pulmonary infarction, pneumonia, or a history suggesting myocardial ischemia are all reasons for emergency admission and should be promptly excluded in patients presenting with a complaint of chest pain. Children probably do not need evaluation for myocardial infarction on the basis of chest pain alone.

Present Illness. Determine the onset of the chest pain, precipitating factors (exertion, breathing, cough, body movement, sitting, lying, swallowing), relieving factors (rest, leaning forward, antacids, nitroglycerine, heat, burping), change in pain with cough or respiration, radiation of the pain, associated nausea or vomiting, presence of cough, sputum production, hemoptysis, fever, chills, night sweats, presence of pain crisis, exposure to viral infections or tuberculosis.

Past Medical History. Is this pain typical of previous pain episodes? Past history of cardiac disease, angina, CHF, chest syndrome, asthma, pneumonia, phlebitis, recent surgery, prolonged sitting, trauma, ulcer disease, esophagitis, gallbladder disease, pancreatitis. Document present medications, allergies, recreational drugs, and smoking.

• Vital signs. Pulse, blood pressure, temperature, respiratory rate and quality (shallow, splinting, labored, retraction, accessory muscle use).
• General. Amount of distress, hydration and nutrition status.
• HEENT. Nasal congestion, rhinorrhea, injected throat, exudates. Nasal flaring in child.
• Neck. Tracheal deviation, lymphandenopathy, thyroid. Supraclavicular retractions in child.
• Chest wall. Check for point tenderness, swelling, erythema, retraction, asymmetry.
• Breasts. Asymmetry, discharge, tenderness, erythema, masses.
• Lungs. Percussion and palpation for dullness, increased or decreased resonance, fremitus. Auscultation for rales, wheezes, rhonchi, inspiratory:expiratory ratio, change in breath sounds, E to A. Listen to cough.
• Heart. Neck veins for venous pressure and pulse, point of maximum impulse, thrills, S1, S2, S3, S4, murmur, rubs, edema.
• Abdomen. Bowel sounds, hepatosplenomegaly, tenderness, rebound, masses. Subcostal retractions in child.
• Extremities. Cyanosis, clubbing, edema, calf swelling, calf tenderness, cords, erythema, pulses.
• Neurologic. Cranial nerves, coordination, muscle strength, sensation.
• Laboratory & Imaging studies
• Minimum Evaluation. CBC with differential, reticulocyte and platelet count. ECG if the pain appears to be cardiac or for patient over 30 years old. Chest x-ray. If sputum production is present, do smear and culture. If chest exam or chest x-ray is positive with dyspnea or tachypnea, examine arterial blood gas.
• Additional Evaluation. If chest x-ray is negative and pulmonary infarction is suspected, consider V/Q lung scan and blood gases. Echocardiogram for pericarditis. Blood cultures if pneumonia is present.

Acute Chest Syndrome - defined as a new chest infiltrate in a sickle cell patient- Etiologies include pneumonia, pulmonary infarction, bone pain from sickling, bone infarction, and fat embolism.. Differentiation of these disorders can be very difficult and all occur in high frequency in patients with sickle syndromes. Fat embolism, pneumonia, and pulmonary infarction cause pleuritic chest pain, fever, leukocytosis, pulmonary infiltrates, hemoptysis, and hypoxia. Pulmonary infarctions occur de novo without emboli from phlebitis. Pneumonia is more likely in children, with high fever, leukocytosis with left shift. Older children and adults have purulent sputum (younger children swallow theirs). Chest radiograph often reveals a small pleural effusion. Infarction from sickling is more common in adults, with hemoptysis, positive V/Q scan with negative chest x-ray, and in patients without fever or leukocytosis. If in doubt, always treat for pneumonia.

Chest Pain from Sickle Pain Episode. Some patients will have pain in chest as a part of their crisis. Pain is usually diffuse, typical of previous episodes, and often associated with chest wall tenderness. This is a diagnosis of exclusion and other causes of chest pain must be eliminated by appropriate testing.

Bone Infarctions in Ribs. Rib infarctions are not uncommon. Findings are localized pain varying with respiration, point tenderness over a rib, and there may be periosteal reaction or soft tissue swelling. Rib radiograph or bone scan may establish a diagnosis.

Acute Myocardial Infarction. Patients of all ages are at some risk for myocardial infarction. Older patients with pain suggestive of myocardial ischemia of recent onset or which has changed in character should be admitted to the CCU. Such pain is usually crushing, substernal with radiation to arms or jaw, accompanied by diaphoresis, nausea, vomiting.

Pericarditis. Causes pain which decreases in the sitting position. There may be fever, leukocytosis, and a history of a viral syndrome. Physical findings include increased neck veins, pulsus paradoxus, decreased heart sounds, and pericardial friction rub. Chest x-ray may show enlarged, globular cardiac shadow. ECG will often show diffuse ST&T abnormalities. Cardiac echo will reveal an effusion.

Abdominal Disease. Cholecystitis may present with complaints of chest pain. Rapid enlargement of the liver or spleen or splenic infarction may also cause pain localized over the chest that varies with respiration. Usually physical examination will reveal that the tenderness is below the ribs. ECG and chest x-ray should always be done before attributing the chest pain to an abdominal problem.

Esophagitis. Pain may be identical to cardiac ischemia, however, not caused by exercise, often relieved by burping or antacids, and may have sour taste in mouth or heartburn. Upper GI imaging and endoscopy may be necessary to diagnose gastroesophageal refluxl.

Pulmonary Embolism - Patients may develop pulmonary emboli from proximal deep venous thrombophlebitis with risk factors similar to those in patients without a sickle syndrome (such as prolonged immobilization, heart failure, hip or knee surgery and pregnancy). The lung scan may be characteristic. Detection of the venous disease may require plethysmography, nuclear venography, or contrast studies.

Acute Asthma Attack - Asthma attacks can cause deep chest pain. Physical exam is usually sufficient to establish the diagnosis because of prolonged expiratory phase of respiration and wheezing. Airway hyperreactivity appears to be common in children with sickle cell disease.

Fat Embolization - Fat emboli can occur as a complication of a severe pain episode. This is a relatively common cause of severe chest syndrome. The presentation is the onset of severe, diffuse bone pain, acute chest pain, cough, acute, severe dyspnea, renal insufficiency, confusion, agitation, altered mental status progressing to coma, and disseminated intravascular coagulation. Fat globules in the urine, refractile bodies on retinal exam, and head and neck petechiae establish the diagnosis, but bronchoscopy is necessary to exclude the diagnosis with bronchial alveolar lavage.

Acute Chest Syndrome – New infiltrate on chest radiograph - Pneumonia verses Pulmonary Infarction - all patients should be admitted to the hospital, at least for overnight observation. Oxygen therapy is indicated for hypoxia and monitoring with frequent blood gases or continuous transcutaneous oximetry. Empiric treatment with antibiotics is indicated after appropriate cultures have been obtained. Third generation cephalosporins with activity against S. pneumoniae and H. influenzae are good initial choices, however, penicillin or ampicillin may be appropriate based on sputum smear findings and local patterns of H. influenzae sensitivity. Empiric addition of a macrolide antibiotic (erythromycin, azithromycin, or clarithromycin) should also be considered because Chlamydia and Mycoplasma infections are common. Antibiotic changes are based on response to therapy and results of cultures and sensitivities.

Simple transfusion may halt progressive respiratory deterioration, such as increasing tachypnea and need for supplemental oxygen. Typical transfusion in the setting of moderate Acute Chest Syndrome would be two aliquots of 5 cc/kg of packed RBC (leukocyte depleted and matched for C, D, E, Kell antigens) infused over 3 hours each, not to exceed hemoglobin level 10 gm/dL. RBC transfusions should, however, be used judiciously because of the risk of alloimmunization or iron overload.

Analgesics will be required. Agents which do not suppress respirations including acetaminophen and non-steroidal anti-inflammatory medications can be used. Narcotic agents which are less respiratory suppressants such as nalbuphine may be used for more severe pain. Other narcotics may be needed to control severe pain, however, they must be used with caution to avoid respiratory depression. Sedative drugs should be avoided. Other support includes careful hydration ( e.g., 1 - 1.5 x maintenance rate, using D₅ 0.2 NaCl for children). Isotonic solutions or volume overload may contribute to pulmonary infiltrates and exacerbate hypoxia.

The role of corticosteroids in non-asthmatic patients with acute chest syndrome is still a topic of clinical research, but high-dose steroids reported to produce brisk improvement in mild to moderate acute chest syndrome. However, this beneficial effect may be transient, with rebound worsening a few days after a 48 hour pulse of intravenous dexamethasone (REF Bernini 1998). Tapering the steroid dosing over a week may be prudent, similar to steroid treatment for asthma.

For more severe episodes - rapid exchange transfusion is indicated for severe hypoxia, rapid progression, diffuse pulmonary involvement, and failure to improve. Intensive care is indicated for severe hypoxia or respiratory distress, because respiratory decompensation can soon require mechanical ventilation. Bronchoscopy can obtain diagnostic lavage specimens and sometimes therapeutic clearance of secretions or (rare) plastic bronchitis. Studies are exploring the role of nitric oxide, biphasic positive airway pressure, extracorporeal membrane oxygenation, and high-frequency oscillatory ventilation for managing severe acute chest syndrome.

Chest Pain from Sickle Pain Episode. Chest pain, which is a usual occurrence during pain episodes can be treated as an uncomplicated pain episode provided the chest film is normal and there are no other respiratory symptoms, fever, or physical findings on chest exam.

Bone Infarctions in Ribs. Bone infarctions in the rib may persist for prolonged periods requiring patient education and non-narcotic analgesics. The pain often responds well to non-steroidal anti-inflammatory medications.

Acute Myocardial Infarction. Patients with myocardial infarction need to be admitted to a coronary care unit with continuous monitoring of electrocardiogram and close observation for complications. Simple transfusion to a hemoglobin of 9 gm/dL with fresh red cells may improve myocardial oxygenation if severe anemia is present.

Pericarditis. Patients with pericarditis should be admitted for initial evaluation and treatment. Specific therapy depends on the etiology.

Abdominal Disease. Patient with splenic or hepatic sequestration should be admitted and considered for transfusion . Patients with acute cholecystitis should be admitted and considered for emergent surgery. Esophagitis is treated with antiacids, H2 blockers in adults, elevation of the bed, and perhaps metoclopramide or cisapride.

Pulmonary Embolism. Admission for treatment with heparin by continuous intravenous infusion followed by coumadin therapy. Low molecular weight heparin may be an alternative.

Acute Asthma Attack. Administration of inhaled bronchodilators and hydration. Consider 3 to 5 days of oral corticosteroids, followed by a week-long taper. Careful observation is indicated because epinephrine may cause cardiac decompensation if severe anemia is present. Bronchodilators can cause diuresis. Hypoxemia occurring during asthma attacks may precipitate sickle complications.

Fat Embolization. Patients usually develop this complication while hospitalized for treatment of a pain episode, although severe overexertion may precipitate an attack in outpatients. Patients require intensive support, rapid exchange transfusion, and often respiratory support with mechanical ventilation. Coagulation support with fresh frozen plasma, cryoprecipitate, and platelets may be required to treat bleeding. High dose corticosteroids are often given. Heparin and alcohol probably are not beneficial.

Explain procedures, listen to patient/caregiver’s concerns/questions, answer questions and reassure patient/caregivers about diagnosis.

 Explain to caregivers/parents that chest pain is a potential problem with sickle cell disease, upper respiratory infections, fever, and cough. Stress the importance of up to date immunizations and encourage administration of prophylactic penicillin to children.

Teach patient/caregivers signs/symptoms of acute chest syndrome/infection (wheezing, dyspnea on exertion, shortness of breath, petechia neck/head, fever, dizziness, weakness).

 Stress the importance of early treatment for cardiac symptoms such as chest pain which feels like "heavy pressure" and/or radiates to jaw, shoulder, or arms.

Explain that pain which gets worse with respiration usually resolves with NSAIDS.

 Teach patients/caregivers about medications, adverse rx, drug interactions and to report symptoms immediately.

Counsel patients about necessary lifestyle changes

-provide nutrition consult for dietary restrictions

-stress the need for patient to stop smoking and for others to avoid smoking around patient.

-refer to smoking cessation group.

-encourage weight loss as needed; and exercise

-stress importance of BP checks and compliance with medications, treatment regimen and follow up visits

Factors which predispose to the development of acute chest syndrome and pneumonia are still being defined. Incentive Spirometry should be used for all postoperative patients and hospitalized patients with pain in the thorax or back (REF Bellet 1995). Daily treatment with hydroxyurea decreases the rate of recurrent acute chest syndrome in adults and children. Smoking and exposure to passive smoking should be avoided. Children and adults should receive appropriate vaccination including H. influenzae, pneumococcal, and annual influenza vaccine. Patients with sickle syndromes and upper respiratory syndromes should be treated with antibiotics if fever, sinus drainage, or productive cough develops. Periodic evaluation of pulmonary functions may identify individuals at risk for chest syndrome, however, more research is indicated before this becomes standard practice.

Parents should be instructed to provide prophylactic penicillin, keep immunizations up to date, and take the child to the physician for evaluation of upper respiratory symptoms, cough and fever. Smoking by patients and around patients should be actively discouraged. All hospitalized patients should be issued an incentive spirometer.

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http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=10861320&dopt=Abstract 

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Last modified: November 03, 2000