eMedicine Specialties > Emergency Medicine > Hematology & Oncology
Anemia, Sickle Cell: Treatment & Medication
Updated: Dec 5, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Prehospital Care
- When severity of the patient's crisis is assessable, self-treatment at home with bed rest, oral analgesia, and hydration is possible.
- Individuals with sickle cell anemia often present to the ED after failing self-treatment. Do not underestimate the patient's pain.
- If patients with sickle cell anemia are in crisis and are being transported by EMS, they should receive supplemental oxygen and intravenous hydration en route to the hospital.
Emergency Department Care
Some areas have specialized facilities that offer emergency care of acute pain associated with sickle cell disease; many emergency departments (EDs) have a standardized treatment plan in place. Failure to treat acute pain aggressively and promptly may lead to chronic pain syndrome. Pain management should include 4 stages: assessment, treatment, reassessment, and adjustment. While considering the severity of pain and the patient's past response, follow consistent protocols to relieve the patient's pain.
Treatment of pain crises is primarily pharmacologic in nature, and opioids represent the mainstay of therapy. Hydration is another mainstay of treatment. For mild-to-moderate pain, acetaminophen with codeine or a nonsteroidal anti-inflammatory drug (NSAID) is usually enough. Patients with severe pain should be given a parenteral opiate in full therapeutic doses at fixed intervals (and not as needed) till pain diminishes at which time the opiate is tapered and then stopped and oral analgesic therapy is instituted. If more frequent doses are needed, patient-controlled analgesia (PCA) can be used. For all types of pain, incentive spirometry is recommended. For frequent and severe pain, long-term hydroxyurea (HU) is presently the accepted treatment. For HU nonresponders, chronic transfusions for a limited period may be an option. Management of constant pain is extremely difficult, and expert advice should be obtained.
Treatment of acute chest syndrome consists of oxygen, antibiotics, incentive spirometry, simple transfusion, and bronchodilators. Exchange transfusion may be indicated for severe cases. Adults, in general, need a higher rate of transfusions and longer hospitalization as compared to children. Overhydration must be avoided.
As for stroke, blood transfusion therapy, aimed at keeping HgbS at less than 30%, is now considered standard care for primary and secondary stroke prevention in children with sickle cell disease. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) showed that regular blood transfusions produced a marked (90%) reduction in first stroke in asymptomatic high-risk children who had 2 abnormal transcranial Doppler (TCD) studies with velocities of 200 cm/s or greater.8 During the transfusion period, most of the TCD studies reverted to or toward normal, but, once transfusion was stopped, there was an unacceptably high rate of TCD reversion to high risk, as well as to actual strokes.9
Prompt recognition and treatment of acute splenic sequestration (ASS) with immediate transfusion have reduced the number of deaths attributed to this life-threatening medical emergency. All new mothers should be educated about symptoms of this potentially life-threatening event and how to do splenic palpation on their infant.
As for priapism, early exchange transfusion is indicated. Epidural neuraxial blockade offers superior analgesia to the often painful conservative treatments. Surgical intervention is the last therapeutic option and often results in significant long-term morbidity.10,11 Intermittent treatment with phosphodiesterase 5 (PDE5) inhibitors is hypothesized to increase PDE5 protein expression, which relieves priapism in pilot studies in patients with sickle cell disease.
Oxygen supplementation is only beneficial if the patient has hypoxia. Intubation and mechanical ventilation may be required in patients in whom strokes have occurred and in patients with acute chest syndrome.
Transfusions are not needed for the usual anemia or episodes of pain associated with sickle cell disease. Urgent replacement of blood is often required for sudden severe anemia due to ASS, parvovirus B19 infection, or in hyperhemolytic crises. Transfusion is helpful in acute chest syndrome, perioperatively and during pregnancy. Acute red cell exchange transfusion is indicated in acute infarctive strokes, severe acute chest syndrome and the multi-organ failure syndromes, the right upper quadrant syndrome, and possibly priapism. Transfusions, simple or exchange, are unlikely to speed up resolution of an acute pain episode. Exchange blood transfusions are indicated in cases of strokes and acute chest syndrome. They are performed occasionally in patients with acute sequestration crisis or in cases of priapism that do not resolve after adequate hydration and analgesia.Consultations
- Consultation with a hematologist may be necessary.
- If retinopathy or hyphema is suspected and visual symptoms are present, consultation with an ophthalmologist is warranted.
- In case of priapism that does not resolve after 6 hours of hydration and analgesia, consult a urologist for aspiration of corpus cavernosum or shunting.
- If avascular necrosis of the hip is suspected in a patient with hip pain and difficulty in walking, consult an orthopedist for possible hip joint replacement. Consult an orthopedist if osteomyelitis is suspected.
Medication
Medications involved in the treatment of sickle cell anemia include analgesics for pain and antibiotics for infections.
Analgesics
Pain control is essential to quality patient care. Analgesics ensure patient comfort, promote pulmonary toilet, and have sedating properties.
Codeine
Binds to opiate receptors in CNS, causing inhibition of ascending pain pathways, altering perception and response to pain.
Adult
15-60 mg PO/IV/IM/SC q4-6h; not to exceed 120 mg/d
Pediatric
0.5 mg/kg PO/IM/SC q4-6h
Phenothiazines may decrease analgesic effect; conversely, acetaminophen toxicity can increase when administered concurrently with CNS depressants or tricyclic antidepressants
May potentiate CNS effects of barbiturates
Documented hypersensitivity; HACE diagnosis; elevated intercostal pain
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Use to treat cough in patients with HAPE only if absolutely necessary; may depress hypoxic ventilatory rate and respiratory drive during sleep
Aspirin (Anacin, Ascriptin, Bayer)
Treats mild to moderate pain. Inhibits prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2.
Adult
325-600 mg PO q4h
Pediatric
10-15 mg/kg/dose PO q4-6h; not to exceed 60-80 mg/kg/d
Effects may decrease with antacids and urinary alkalinizers; corticosteroids decrease salicylate serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur with coadministration of anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses >2 g/d may potentiate glucose-lowering effect of sulfonylurea drugs
Documented hypersensitivity; liver damage; hypoprothrombinemia; vitamin K deficiency; bleeding disorders; asthma; because of association of aspirin with Reye syndrome, do not use in children (<16 y) with flu
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
May cause transient decrease in renal function and aggravate chronic kidney disease; avoid use in patients with severe anemia, with history of blood coagulation defects, or who are taking anticoagulants
Acetaminophen (Tylenol, Panadol, Aspirin Free Anacin)
DOC for pain in patients with documented hypersensitivity to aspirin or NSAIDs, with upper GI disease, or who are taking oral anticoagulants.
Adult
325-650 mg PO q4-6h; not to exceed 4 g/d
Pediatric
<12 years: 10-15 mg/kg/dose PO q4-6h prn; not to exceed 2.6 g/d
>12 years: 325-650 mg PO q4h; not to exceed 5 doses/d
Rifampin can reduce analgesic effects of acetaminophen; coadministration with barbiturates, carbamazepine, hydantoins, and isoniazid may increase hepatotoxicity
Documented hypersensitivity; known G-6-P deficiency
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Hepatotoxicity possible in chronic alcoholism following various dose levels; severe or recurrent pain or high or continued fever may indicate a serious illness; acetaminophen is contained in many OTC products and combined use with these products may result in cumulative acetaminophen doses exceeding recommended maximum dose
Ibuprofen (Ibuprin, Advil, Motrin)
Usually the DOC for treatment of mild to moderate pain, if no contraindications exist. Inhibits inflammatory reactions and pain by decreasing the activity of the enzyme cyclo-oxygenase, resulting in inhibition of prostaglandin synthesis.
Adult
200-800 mg PO qd
Pediatric
Children's Motrin
2-3 years: 1 tsp
4-5 years: 1 1/2 tsp
6-8 years: 2 tsp
9-10 years: 2 1/2 tsp
12 years: 3 tsp
>12 years: Administer as in adults
Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently
Documented hypersensitivity; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency; high risk of bleeding
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in congestive heart failure, hypertension, and decreased renal and hepatic function; caution in anticoagulation abnormalities or during anticoagulant therapy
Oxycodone and acetaminophen (Percocet, Roxicet, Roxilox)
Drug combination indicated for the relief of moderate to severe pain. DOC for patients who are hypersensitive to aspirin.
Adult
1 tab PO q4-6h prn
Pediatric
0.05-0.15 mg/kg/dose oxycodone PO; not to exceed 5 mg/dose oxycodone
Phenothiazines may decrease analgesic effects of this medication; toxicity increases with coadministration of either CNS depressants or tricyclic antidepressants
Documented hypersensitivity; CNS injuries
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Duration of action may increase in elderly persons; be aware of total daily dose of acetaminophen patient is receiving; do not exceed 4,000 mg/24 h of acetaminophen; higher doses may cause liver toxicity
Meperidine (Demerol)
Analgesic with multiple actions similar to those of morphine; may produce less constipation, smooth muscle spasm, and depression of cough reflex than similar analgesic doses of morphine.
Adult
50-150 mg PO/IV/IM q3-4h prn
Pediatric
1-1.8 mg/kg IM q1-3h
Monitor for increased respiratory and CNS depression with coadministration of cimetidine; hydantoins may decrease effects of meperidine; avoid with protease inhibitors
Documented hypersensitivity; MAOIs; upper airway obstruction or significant respiratory depression; during labor when premature delivery of infant is anticipated
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in patients with head injuries since meperidine may increase respiratory depression and CSF pressure (use only if absolutely necessary); caution when using postoperatively and with history of pulmonary disease (suppresses cough reflex)
Substantially increased dose levels, due to tolerance, may aggravate or cause seizures even if no prior history of convulsive disorders exists; monitor closely for morphine-induced seizure activity if prior seizure history
Morphine sulfate (Duramorph, Astramorph, MS Contin)
DOC for analgesia due to reliable and predictable effects, safety profile, and ease of reversibility with naloxone. Various IV doses are used; commonly titrated until desired effect obtained.
Adult
2- to 5-mg increments IV titrated q10-30min to pain response
30 mg PO q8-12h
10 mg/70 kg IM q4h
12-25 mg/70 kg in 5 mL of water over 5-min continuous infusion 0.1-1 mg/mL in 5% dextrose
Pediatric
0.1-0.2 mg/kg IV q4h; not to exceed 15 mg
Phenothiazines may antagonize analgesic effects of opiate agonists; tricyclic antidepressants, MAOIs, and other CNS depressants may potentiate adverse effects of morphine
Documented hypersensitivity; hypotension; potentially compromised airway in which establishing rapid airway control would be difficult
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Avoid in hypotension, respiratory depression, nausea, emesis, constipation, and urinary retention; caution in atrial flutter and other supraventricular tachycardias; has vagolytic action and may increase ventricular response rate
Oxycodone and aspirin (Percodan, Roxiprin, Codoxy)
Drug combination indicated for the relief of moderate to severe pain.
Adult
1-2 tab or cap PO q4-6h prn pain
Pediatric
0.05-0.15 mg/kg/dose oxycodone PO; not to exceed 5 mg/dose of oxycodone q4-6h prn
Phenothiazines may decrease analgesic effects; conversely, toxicity increases when administered concurrently with, CNS depressants or tricyclic antidepressants; may also potentiate anticoagulant effects of warfarin
Documented hypersensitivity; liver damage; hypoprothrombinemia; vitamin K deficiency; bleeding disorders; asthma; children <16 y with the flu (potential risk of Reye syndrome)
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Duration of action may increase in elderly persons; caution in renal or liver impairment, peptic ulcer disease, and erosive gastritis
Methadone (Dolophine)
Used in the management of severe pain. Inhibits ascending pain pathways, diminishing the perception of and response to pain.
Adult
2.5-10 mg PO/IM/SC q3-8h prn; increase to a maintenance dose of 5-20 mg q6-8h
Pediatric
0.7 mg/kg/d PO/IM/SC divided q4-6h prn; not to exceed 10 mg/dose
Phenytoin, rifampin, and pentazocine may decrease blood levels of methadone; phenothiazines, tricyclic antidepressants, MAOIs, and CNS depressants may increase the toxicity of methadone
Documented hypersensitivity; bronchial asthma; increased intracranial pressure
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in severe liver disease; due to its relatively long half-life, titrate dose slowly
Antibiotics
These agents are used for treatment of suspected or confirmed infections.
Cefuroxime (Ceftin)
Second-generation cephalosporin that maintains gram-positive activity of first-generation cephalosporins and adds activity against P mirabilis, H influenzae, E coli, K pneumonia, and M catarrhalis. Condition of patient, severity of infection, and susceptibility of the microorganism should determine proper dose and route of administration.
Adult
250 mg PO q12h or 750-1500 mg IV/IM q8h
Pediatric
125 mg PO q12h
50-100 mg/g/d IV/IM divided q6-8h
Disulfiramlike reactions may occur when alcohol is consumed within 72 h after taking cefuroxime; may increase hypoprothrombinemic effects of anticoagulants; may increase nephrotoxicity in patient receiving potent diuretics such as loop diuretics; coadministration with aminoglycosides increases nephrotoxic potential
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Administer half dose if CrCl is 10-30 mL/min and one quarter dose if <10 mL/min; fungal and microorganism overgrowth may occur with prolonged therapy
Amoxicillin and clavulanate (Augmentin)
Drug combination that extends antibiotic spectrum of this penicillin to include bacteria normally resistant to beta-lactam antibiotics. Indicated for skin and skin structure infections caused by beta-lactamase-producing strains of S aureus. Administer treatment for a minimum of 10 d.
Adult
250-500 mg PO q8h
Pediatric
<40 kg: 40 mg/kg PO divided tid
>40 kg: Administer as in adults
Coadministration with warfarin or heparin increases risk of bleeding
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Increases risk of rash in patients taking allopurinol or with infectious mononucleosis
Perform bacteriologic studies to determine causative organisms and their susceptibility so that appropriate therapy is administered
Use therapy for a minimum of 10 d to eliminate organism; otherwise, sequelae such as endocarditis and rheumatic fever may ensue; cultures should be taken following treatment to confirm that the streptococci have been eradicated
Ceftriaxone (Rocephin)
Third-generation cephalosporin with broad gram-negative spectrum, lower efficacy against gram-positive organisms, and higher efficacy against resistant organisms.
By binding to one or more of the penicillin-binding proteins, arrests bacterial cell wall synthesis and inhibits bacterial growth.
Adult
1-2 g IV/IM qd
Pediatric
50-75 mg/kg/d IV divided q12h; not to exceed 2 g/d
Probenecid may increase ceftriaxone levels; coadministration with ethacrynic acid, furosemide, and aminoglycosides may increase nephrotoxicity
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adjust dose in renal impairment; caution in breastfeeding women and in those with penicillin allergy
Cefaclor (Ceclor)
Second-generation cephalosporin indicated for infections caused by susceptible gram-positive cocci and gram-negative rods.
Adult
250-500 mg PO q8h
Pediatric
20-40 mg/kg/d PO divided q8-12h; not to exceed 2 g/d
Alcoholic beverages consumed <72 h after taking cefaclor may produce disulfiramlike reactions; may increase hypoprothrombinemic effects of anticoagulants; coadministration with potent diuretics and aminoglycosides (eg, loop diuretics) may increase nephrotoxicity
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Reduce dosage by 1/2 if creatinine clearance is 10-30 mL/min and by 3/4 if <10 mL/min; bacterial or fungal overgrowth of nonsusceptible organisms may occur with prolonged or repeated therapy
Antiemetics
These agents are useful in the treatment of symptomatic nausea.
Promethazine (Phenergan)
Used for symptomatic treatment of nausea in vestibular dysfunction. Antidopaminergic agent effective in the treatment of emesis. Blocks postsynaptic mesolimbic dopaminergic receptors in the brain and reduces stimuli to brainstem reticular system.
Adult
25 mg PO q4-6h prn
Pediatric
<2 years: Contraindicated
>2 years: 1 mg/kg PO q4-6h
May have additive effects when used concurrently with other CNS depressants or anticonvulsants; coadministration with epinephrine may cause hypotension
Documented hypersensitivity; children <2 y (incidences of death due to respiratory depression)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in cardiovascular disease, impaired liver function, seizures, sleep apnea, and asthma
More on Anemia, Sickle Cell |
| Overview: Anemia, Sickle Cell |
| Differential Diagnoses & Workup: Anemia, Sickle Cell |
 Treatment & Medication: Anemia, Sickle Cell |
| Follow-up: Anemia, Sickle Cell |
| References |
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References
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Further Reading
Keywords
sickle cell disease, sickle cell anemia, blood disorder, crescent cell anemia, sickle cell autosomal recessive genetic disease, hemoglobin S, HbS, vasoocclusive crisis, avascular necrosis, isosthenuria, acute chest syndrome, hypertransfusion programs, hematologic crises, aplastic crisis, parvovirus B19 infection, infectious crises, acute sequestration crisis, syncope
