Sickle Cell Anemia Medication

  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Jan 3, 2012
 

Medication Summary

The drugs used in treatment of sickle cell disease (SCD) include antimetabolites, analgesics, antibiotics, and vaccines.

Next

Antimetabolites

Class Summary

Hydroxyurea affects DNA, resulting in increased production of Hb F, which inhibits sickling. Considerable effort is being expended to identify agents whose ultimate effect interferes with the sickling process and prevents the many complications of sickle cell disease.

View full drug information

Hydroxyurea (Droxia)

 

Hydroxyurea inhibits deoxynucleotide synthesis. Its myelosuppressive effects last a few days to a week and are easier to control than those of alkylating agents.

Previous
Next

Opioid Analgesics

Class Summary

Opioid analgesics are used to control acute crisis and chronic pain.

View full drug information

Oxycodone and aspirin (Percodan, Roxiprin)

 

This drug combination is indicated for the relief of moderate to severe pain.

View full drug information

Methadone (Dolophine)

 

Methadone is used in the management of severe pain. It inhibits ascending pain pathways, diminishing the perception of and response to pain.

View full drug information

Morphine sulfate (Duramorph, Astramorph, MS Contin, Avinza, Kadian)

 

An opioid analgesic, morphine interacts with endorphin receptors in the CNS.

View full drug information

Oxycodone and acetaminophen (Percocet, Roxicet, Roxilox)

 

This drug combination is indicated for the relief of moderate to severe pain. It is the drug of choice for patients who are hypersensitive to aspirin.

Fentanyl citrate

 

A synthetic opioid analgesic that is primarily a mu receptor agonist, fentanyl is 50-100 times more potent than morphine. It has short duration of action (1-2 h) and minimal cardiovascular effects, such as hypotension. Respiratory depression is uncommon, but this effect lasts longer than its analgesic effect. Fentanyl is frequently used in patient-controlled analgesia for relief of pain. Unlike morphine, fentanyl is not commonly associated with histamine release.

View full drug information

Nalbuphine

 

An opioid agonist/antagonist, nalbuphine stimulates kappa opioid receptor in the CNS, which causes inhibition of ascending pain pathways. An antagonist at the opioid mu receptors, it is useful for moderate-to-severe pain in sickle cell disease.

View full drug information

Codeine

 

Codeine binds to opiate receptors in the CNS, causing inhibition of ascending pain pathways, altering perception and response to pain.

Acetaminophen with codeine (Tylenol-3)

 

This combination is a mild narcotic analgesic. Provide the family with a small supply for use when pain severity is greater than can be managed with acetaminophen alone. Counsel parents to use for severe pain only, not as the first medication for each symptom.

Previous
Next

Nonsteroidal Analgesics

Class Summary

Acetaminophen and nonsteroidal anti-inflammatory drugs (NSAIDs) add to the effects of opioids during painful crisis. This allows use of lower doses of narcotics.

View full drug information

Ketorolac

 

Ketorolac is an intravenously administered NSAID and a very powerful analgesic. It inhibits prostaglandin synthesis by decreasing activity of the enzyme cyclooxygenase, which results in decreased formation of prostaglandin precursors. In turn, this results in reduced inflammation.

View full drug information

Aspirin (Anacin, Ascriptin, Bayer Aspirin)

 

Aspirin treats mild to moderate pain. It inhibits prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2.

View full drug information

Acetaminophen (Tylenol, Aspirin-Free Anacin)

 

Acetaminophen is the drug of choice for pain in patients with documented hypersensitivity to aspirin or NSAIDs, with upper GI disease, or who are taking oral anticoagulants.

View full drug information

Ibuprofen (Advil, Motrin)

 

Ibuprofen is usually the drug of choice for treatment of mild to moderate pain, if no contraindications exist. It inhibits inflammatory reactions and pain by decreasing the activity of the enzyme cyclo-oxygenase, resulting in inhibition of prostaglandin synthesis.

Previous
Next

Vitamins

Class Summary

Folate is necessary for erythropoiesis. Supplemental folic acid replenishes depleted folate stores secondary to hemolysis.

View full drug information

Folic acid

 

Folic acid is nnecessary for proper nucleotide metabolism. It is an important cofactor for enzymes used in production of RBCs.

Previous
Next

Antibiotics

Class Summary

These agents are used for treatment of suspected or confirmed infections.

View full drug information

Cefuroxime (Ceftin, Zinacef)

 

Cefuroxime is a second-generation cephalosporin that maintains the gram-positive activity of first-generation cephalosporins and adds activity against Proteus mirabilis, Haemophilus influenzae, Escherichia coli, Klebsiella pneumoniae, and Moraxella catarrhalis. The condition of the patient, severity of infection, and susceptibility of the microorganism should determine proper dose and route of administration.

View full drug information

Amoxicillin and clavulanate (Augmentin, Augmentin XR, Augmentin ES-600)

 

This drug combination extends the antibiotic spectrum of this penicillin to include bacteria normally resistant to beta-lactam antibiotics. It is indicated for skin and skin structure infections caused by beta-lactamase-producing strains of Staphylococcus aureus. Administer treatment for a minimum of 10 d.

View full drug information

Penicillin VK

 

Penicillin inhibits the biosynthesis of cell wall mucopeptide.

View full drug information

Ceftriaxone (Rocephin)

 

A third-generation cephalosporin, ceftriaxone is the most common antibiotic used in the management of fever in patients with sickle cell disease in the acute care/ED setting. This agent is widely distributed throughout the body, including gallbladder, lungs, bone, bile, and cerebrospinal fluid (CSF); ceftriaxone diffuses into the CSF at higher concentrations when the meninges are inflamed.

View full drug information

Azithromycin (Zithromax)

 

Azithromycin is a macrolide antibiotic that is useful for treatment of

community-acquired pneumonia in sickle cell disease, as an adjunct to a cephalosporin to cover Chlamydia or Mycoplasma infections.

View full drug information

Cefaclor (Raniclor)

 

A second-generation cephalosporin, cefaclor is indicated for infections caused by susceptible gram-positive cocci and gram-negative rods.

Previous
Next

Phosphodiesterase Type 5 Inhibitors

Class Summary

Phosphodiesterase type 5 (PDE5) inhibitors are used to treat pulmonary hypertension associated with sickle cell disease. These agents are also used to prevent priapism associated with sickle cell disease.

View full drug information

Sildenafil (Revatio)

 

Sildenafil promotes selective smooth muscle relaxation in lung vasculature, possibly by inhibiting PDE5. This results in a subsequent reduction of blood pressure in pulmonary arteries and an increase in cardiac output.

Previous
Next

Endothelin Receptor Antagonists

Class Summary

These agents are used for pulmonary hypertension associated with sickle cell disease.

View full drug information

Bosentan (Tracleer)

 

Bosentan improves pulmonary arterial hemodynamics by competitively binding to ET-1 receptors endothelin-A and endothelin-B in pulmonary vascular endothelium and pulmonary vascular smooth muscle. This leads to a significant increase in cardiac index associated with a significant reduction in pulmonary artery pressure, pulmonary vascular resistance, and mean right atrial pressure.

Previous
Next

Iron Chelators

Class Summary

Iron overload is a consequence of the numerous transfusions required and may lead to complications such as heart or liver failure. Iron chelators help maintain hemoglobin levels within the desired range.

View full drug information

Deferoxamine mesylate (Desferal)

 

Deferoxamine helps prevent damage to the liver and bone marrow from iron deposition by promoting renal and hepatic excretion in urine and bile in feces. It readily chelates iron from ferritin and hemosiderin but not from transferrin. It does not affect iron in the cytochromes or hemoglobin. This agent is most effective when administered by continuous infusion. It gives urine a red discoloration.

View full drug information

Deferasirox (Exjade)

 

Deferasirox is an orally administered iron chelation agent that has been shown to reduce the liver iron concentration in adults and children who receive repeated RBC transfusions. It binds iron with high affinity in a 2:1 ratio.

View full drug information

Deferiprone (Ferriprox)

 

Deferiprone (1,2 dimethyl-3-hydroxypyridine-4-one) is a member of a family of hydroxypyridine-4-one (HPO) chelators that requires 3 molecules to fully bind iron (III), each molecule providing 2 coordination sites (bidentate chelation). It is designated as an orphan drug in the United States.

Previous
Next

Antiemetics

Class Summary

These agents are useful in the treatment of symptomatic nausea.

Promethazine (Phenergan)

 

Phenergan is used for symptomatic treatment of nausea in vestibular dysfunction. Antidopaminergic agent effective in the treatment of emesis. It blocks postsynaptic mesolimbic dopaminergic receptors in the brain and reduces stimuli to the brainstem reticular system.

Previous
 
Contributor Information and Disclosures
Author

Joseph E Maakaron, MD  Research Fellow, Department of Internal Medicine, American University of Beirut, Lebanon

Disclosure: Nothing to disclose.

Coauthor(s)

Ali Taher  MD, Professor of Medicine, Division of Hematology and Oncology, American University of Beirut Medical Center

Disclosure: Nothing to disclose.

Ulrich Josef Woermann, MD  Consulting Staff, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Roy Alson, MD, PhD, FACEP, FAAEM Associate Professor, Department of Emergency Medicine, Wake Forest University School of Medicine; Medical Director, Forsyth County EMS; Deputy Medical Advisor, North Carolina Office of EMS; Associate Medical Director, North Carolina Baptist AirCare

Roy Alson, MD, PhD, FACEP, FAAEM is a member of the following medical societies: Air Medical Physician Association, American Academy of Emergency Medicine, American College of Emergency Physicians, American Medical Association, National Association of EMS Physicians, North Carolina Medical Society, Society for Academic Emergency Medicine, and World Association for Disaster and Emergency Medicine

Disclosure: Nothing to disclose.

Jeffrey L Arnold, MD, FACEP Chairman, Department of Emergency Medicine, Santa Clara Valley Medical Center

Jeffrey L Arnold, MD, FACEP is a member of the following medical societies: American Academy of Emergency Medicine and American College of Physicians

Disclosure: Nothing to disclose.

Robert J Arceci, MD, PhD King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Wadie F Bahou, MD Chief, Division of Hematology, Hematology/Oncology Fellowship Director, Professor, Department of Internal Medicine, State University of New York at Stony Brook

Wadie F Bahou, MD is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

Dvorah Balsam, MD Chief, Division of Pediatric Radiology, Nassau University Medical Center; Professor, Department of Clinical Radiology, State University of New York at Stony Brook

Disclosure: Nothing to disclose.

Salvatore Bertolone, MD Director, Division of Pediatric Hematology/Oncology, Department of Pediatrics, Kosair Children's Hospital; Professor, University of Louisville School of Medicine

Salvatore Bertolone, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Education, American Association of Blood Banks, American Cancer Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Kentucky Medical Association

Disclosure: Nothing to disclose.

Barry E Brenner, MD, PhD, FACEP Professor of Emergency Medicine, Professor of Internal Medicine, Program Director, Emergency Medicine, Case Medical Center, University Hospitals, Case Western Reserve University School of Medicine

Barry E Brenner, MD, PhD, FACEP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Chest Physicians, American College of Emergency Physicians, American College of Physicians, American Heart Association, American Thoracic Society, Arkansas Medical Society, New York Academy of Medicine, New York Academy of Sciences, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group

Disclosure: No financial interests None None

Nedra R Dodds, MD Medical Director, Opulence Aesthetic Medicine

Nedra R Dodds, MD is a member of the following medical societies: American Academy of Anti-Aging Medicine, American Academy of Cosmetic Surgery, American College of Emergency Physicians, American Medical Association, National Medical Association, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

James L Harper, MD Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society

Disclosure: Nothing to disclose.

Adlette Inati, MD Head, Division of Pediatric Hematology-Oncology, Medical Director, Children's Center for Cancer and Blood Diseases, Rafik Hariri University Hospital; Research Associate, Balamand University; Head of Post Bone Marrow Transplant Clinic and Consultant Hematologist, Chronic Care Center; Founding Faculty, Lebanese American University School of Medicine, Lebanon

Adlette Inati, MD is a member of the following medical societies: Alpha Omega Alpha, American Society of Hematology, European Hematology Association, and International Society of Hematology

Disclosure: Nothing to disclose.

Ziad N Kazzi, MD Assistant Professor, Department of Emergency Medicine, Emory University; Medical Toxicologist, Georgia Poison Center

Ziad N Kazzi, MD is a member of the following medical societies: American Academy of Clinical Toxicology, American Academy of Emergency Medicine, American College of Emergency Physicians, and American College of Medical Toxicology

Disclosure: Nothing to disclose.

Richard S Krause, MD Senior Clinical Faculty/Clinical Assistant Professor, Department of Emergency Medicine, University of Buffalo State University of New York School of Medicine and Biomedical Sciences

Richard S Krause, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Ashok B Raj, MD Associate Professor, Section of Pediatric Hematology and Oncology, Department of Pediatrics, Kosair Children's Hospital, University of Louisville School of Medicine

Ashok B Raj, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and Kentucky Medical Association

Disclosure: Nothing to disclose.

Sharada A Sarnaik, MBBS Professor of Pediatrics, Wayne State University School of Medicine; Director, Sickle Cell Center, Attending Hematologist/Oncologist, Children's Hospital of Michigan

Sharada A Sarnaik, MBBS is a member of the following medical societies: American Association of Blood Banks, American Association of University Professors, American Society of Hematology, American Society of Pediatric Hematology/Oncology, New York Academy of Sciences, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Hosseinali Shahidi, MD, MPH Assistant Professor, Departments of Emergency Medicine and Pediatrics, State University of New York and Health Science Center at Brooklyn

Hosseinali Shahidi, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, and American Public Health Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Garry Wilkes MBBS, FACEM, Director of Emergency Medicine, Calvary Hospital, Canberra, ACT; Adjunct Associate Professor, Edith Cowan University; Clinical Associate Professor, Rural Clinical School, University of Western Australia

Disclosure: Nothing to disclose.

Mary L Windle, PharmD, Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Grace M Young, MD Associate Professor, Department of Pediatrics, University of Maryland Medical Center

Grace M Young, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Emergency Physicians

Disclosure: Nothing to disclose.

References

  1. Olujohungbe A, Howard J. The clinical care of adult patients with sickle cell disease. Br J Hosp Med (Lond). Nov 2008;69(11):616-9. [Medline].

  2. Johnson L, Carmona-Bayonas A, Tick L. Management of pain due to sickle cell disease. J Pain Palliat Care Pharmacother. 2008;22(1):51-4. [Medline].

  3. De D. Acute nursing care and management of patients with sickle cell. Br J Nurs. Jul 10-23 2008;17(13):818-23. [Medline].

  4. Zen Q, Batchvarova M, Twyman CA, Eyler CE, Qiu H, De Castro LM, et al. B-CAM/LU expression and the role of B-CAM/LU activation in binding of low- and high-density red cells to laminin in sickle cell disease. Am J Hematol. Feb 2004;75(2):63-72. [Medline].

  5. Morris CR. Asthma management: reinventing the wheel in sickle cell disease. Am J Hematol. Apr 2009;84(4):234-41. [Medline].

  6. National Institutes of Health. Introduction to Genes and Disease: Anemia, Sickle Cell. National Center for Biotechnology Information. Available at http://www.ncbi.nlm.nih.gov/books/NBK22238/. Accessed May 6, 2009.

  7. Centers for Disease Control and Prevention. Sickle Cell Disease: Health Care Professionals: Data & Statistics. Centers for Disease Control and Prevention. Department of Health and Human Services. Available at http://www.cdc.gov/ncbddd/sicklecell/hcp_data.htm. Accessed May 6, 2009.

  8. Abbott KC, Hypolite IO, Agodoa LY. Sickle cell nephropathy at end-stage renal disease in the United States: patient characteristics and survival. Clin Nephrol. Jul 2002;58(1):9-15. [Medline].

  9. Powars DR, Elliott-Mills DD, Chan L, Niland J, Hiti AL, Opas LM, et al. Chronic renal failure in sickle cell disease: risk factors, clinical course, and mortality. Ann Intern Med. Oct 15 1991;115(8):614-20. [Medline].

  10. Derebail VK, Nachman PH, Key NS, Ansede H, Falk RJ, Kshirsagar AV. High prevalence of sickle cell trait in African Americans with ESRD. J Am Soc Nephrol. Mar 2010;21(3):413-7. [Medline]. [Full Text].

  11. Audard V, Homs S, Habibi A, Galacteros F, Bartolucci P, Godeau B, et al. Acute kidney injury in sickle patients with painful crisis or acute chest syndrome and its relation to pulmonary hypertension. Nephrol Dial Transplant. Aug 2010;25(8):2524-9. [Medline].

  12. Scheinman JI. In: Holliday M, Barratt TM, Avner ED (Eds.). Sickle cell nephropathy. Pediatric Nephrology. Baltimore: Williams and Wilkins; 1994:908.

  13. Nissenson AR, Port FK. Outcome of end-stage renal disease in patients with rare causes of renal failure. I. Inherited and metabolic disorders. Q J Med. Nov 1989;73(271):1055-62. [Medline].

  14. Saborio P, Scheinman JI. Disease of the month - Sickle cell nephropathy. J Amm Soc Nephrol. 1999;10:187.

  15. Miller ST, Sleeper LA, Pegelow CH, Enos LE, Wang WC, Weiner SJ, et al. Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med. Jan 13 2000;342(2):83-9. [Medline].

  16. Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. Jun 9 1994;330(23):1639-44. [Medline].

  17. Quinn CT, Rogers ZR, Buchanan GR. Survival of children with sickle cell disease. Blood. Jun 1 2004;103(11):4023-7. [Medline]. [Full Text].

  18. Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med. Feb 26 2004;350(9):886-95. [Medline].

  19. Parent F, Bachir D, Inamo J, et al. A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med. Jul 7 2011;365(1):44-53. [Medline].

  20. Wright SW, Zeldin MH, Wrenn K, Miller O. Screening for sickle-cell trait in the emergency department. J Gen Intern Med. Aug 1994;9(8):421-4. [Medline].

  21. Chiang EY, Frenette PS. Sickle cell vaso-occlusion. Hematol Oncol Clin North Am. Oct 2005;19(5):771-84, v. [Medline].

  22. Rhodes M, Akohoue SA, Shankar SM, Fleming I, Qi An A, Yu C, et al. Growth patterns in children with sickle cell anemia during puberty. Pediatr Blood Cancer. Oct 2009;53(4):635-41. [Medline]. [Full Text].

  23. Telfer P, Coen P, Chakravorty S, Wilkey O, Evans J, Newell H, et al. Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. Haematologica. Jul 2007;92(7):905-12. [Medline].

  24. Nicholson GT, Hsu DT, Colan SD, et al. Coronary artery dilation in sickle cell disease. J Pediatr. Nov 2011;159(5):789-794.e1-2. [Medline].

  25. Dahoui HA, Hayek MN, Nietert PJ, et al. Pulmonary hypertension in children and young adults with sickle cell disease: evidence for familial clustering. Pediatr Blood Cancer. 2010;54(3):398-402.

  26. Onyekwere OC, Campbell A, Teshome M, Onyeagoro S, Sylvan C, Akintilo A, et al. Pulmonary hypertension in children and adolescents with sickle cell disease. Pediatr Cardiol. Mar 2008;29(2):309-12. [Medline].

  27. Parent F, Bachir D, Inamo J, et al. A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med. Jul 7 2011;365(1):44-53. [Medline].

  28. [Guideline] Goldstein LB, Bushnell CD, Adams RJ, et al. Guidelines for the primary prevention of stroke: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. Feb 2011;42(2):517-84. [Medline].

  29. [Guideline] Rees DC, Olujohungbe AD, Parker NE, Stephens AD, Telfer P, Wright J. Guidelines for the management of the acute painful crisis in sickle cell disease. Br J Haematol. Mar 2003;120(5):744-52. [Medline]. [Full Text].

  30. [Guideline] US Preventive Services Task Force. Screening for sickle cell disease in newborns: U.S. Preventive Services Task Force recommendation statement. Agency for Healthcare Research and Quality (AHRQ). Sep 2007;[Full Text].

  31. Bernard AW, Venkat A, Lyons MS. Best evidence topic report. Full blood count and reticulocyte count in painful sickle crisis. Emerg Med J. Apr 2006;23(4):302-3. [Medline]. [Full Text].

  32. [Guideline] Goldstein LB, Bushnell CD, Adams RJ, Appel LJ, Braun LT, Chaturvedi S, et al. Guidelines for the primary prevention of stroke: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. Feb 2011;42(2):517-84. [Medline]. [Full Text].

  33. Linguraru MG, Orandi BJ, Van Uitert RL, Mukherjee N, Summers RM, Gladwin MT, et al. CT and image processing non-invasive indicators of sickle cell secondary pulmonary hypertension. Conf Proc IEEE Eng Med Biol Soc. 2008;2008:859-62. [Medline]. [Full Text].

  34. Cerci SS, Suslu H, Cerci C, Yildiz M, Ozbek FM, Balci TA, et al. Different findings in Tc-99m MDP bone scintigraphy of patients with sickle cell disease: report of three cases. Ann Nucl Med. Jul 2007;21(5):311-4. [Medline].

  35. Roberts L, O'Driscoll S, Dick MC, Height SE, Deane C, Goss DE, et al. Stroke prevention in the young child with sickle cell anaemia. Ann Hematol. Oct 2009;88(10):943-6. [Medline].

  36. Lee MT, Piomelli S, Granger S, Miller ST, Harkness S, Brambilla DJ, et al. Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results. Blood. Aug 1 2006;108(3):847-52. [Medline]. [Full Text].

  37. [Best Evidence] Adams RJ, Brambilla D. Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. N Engl J Med. Dec 29 2005;353(26):2769-78. [Medline]. [Full Text].

  38. [Guideline] Brawley OW, Cornelius LJ, Edwards LR, Gamble VN, Green BL, Inturrisi C, et al. National Institutes of Health Consensus Development Conference statement: hydroxyurea treatment for sickle cell disease. Ann Intern Med. Jun 17 2008;148(12):932-8. [Medline]. [Full Text].

  39. Heeney MM, Ware RE. Hydroxyurea for children with sickle cell disease. Pediatr Clin North Am. Apr 2008;55(2):483-501, x. [Medline].

  40. [Best Evidence] Strouse JJ, Lanzkron S, Beach MC, Haywood C, Park H, Witkop C, et al. Hydroxyurea for sickle cell disease: a systematic review for efficacy and toxicity in children. Pediatrics. Dec 2008;122(6):1332-42. [Medline].

  41. Firth PG, Head CA. Sickle cell disease and anesthesia. Anesthesiology. Sep 2004;101(3):766-85. [Medline].

  42. Cappellini MD, Piga A. Current status in iron chelation in hemoglobinopathies. Curr Mol Med. Nov 2008;8(7):663-74. [Medline].

  43. Rienhoff HY Jr, Viprakasit V, Tay L, et al. A phase 1 dose-escalation study: safety, tolerability, and pharmacokinetics of FBS0701, a novel oral iron chelator for the treatment of transfusional iron overload. Haematologica. Apr 2011;96(4):521-5. [Medline]. [Full Text].

  44. Das BB, Sobczyk W, Bertolone S, Raj A. Cardiopulmonary stress testing in children with sickle cell disease who are on long-term erythrocytapheresis. J Pediatr Hematol Oncol. May 2008;30(5):373-7. [Medline].

  45. Rogovik AL, Li Y, Kirby MA, Friedman JN, Goldman RD. Admission and length of stay due to painful vasoocclusive crisis in children. Am J Emerg Med. Sep 2009;27(7):797-801. [Medline].

  46. Wang WC, Ware RE, Miller ST, et al. Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet. May 14 2011;377(9778):1663-72. [Medline].

  47. Gladwin MT, Kato GJ, Weiner D, et al. Nitric oxide for inhalation in the acute treatment of sickle cell pain crisis: a randomized controlled trial. JAMA. Mar 2 2011;305(9):893-902. [Medline].

  48. Rogers ZR. Priapism in sickle cell disease. Hematol Oncol Clin North Am. Oct 2005;19(5):917-28, viii. [Medline].

  49. Burnett AL, Bivalacqua TJ, Champion HC, Musicki B. Long-term oral phosphodiesterase 5 inhibitor therapy alleviates recurrent priapism. Urology. May 2006;67(5):1043-8. [Medline].

  50. [Guideline] Furie KL, Kasner SE, Adams RJ, Albers GW, Bush RL, Fagan SC, et al. Guidelines for the prevention of stroke in patients with stroke or transient ischemic attack: a guideline for healthcare professionals from the american heart association/american stroke association. Stroke. Jan 2011;42(1):227-76. [Medline]. [Full Text].

  51. Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E, Pegelow C, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med. Jul 2 1998;339(1):5-11. [Medline].

  52. Williams R, Olivi S, Li CS, Storm M, Cremer L, Mackert P, et al. Oral glutamine supplementation decreases resting energy expenditure in children and adolescents with sickle cell anemia. J Pediatr Hematol Oncol. Oct 2004;26(10):619-25. [Medline].

  53. Kar BC. Splenectomy in sickle cell disease. J Assoc Physicians India. Sep 1999;47(9):890-3. [Medline].

  54. Prevention of pneumococcal disease: recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Recomm Rep. Apr 4 1997;46:1-24. [Medline].

  55. Kazancioglu R, Sever MS, Yüksel-Onel D, Eraksoy H, Yildiz A, Celik AV, et al. Immunization of renal transplant recipients with pneumococcal polysaccharide vaccine. Clin Transplant. Feb 2000;14(1):61-5. [Medline].

  56. Fiore AE, Shay DK, Broder K, Iskander JK, Uyeki TM, Mootrey G, et al. Prevention and control of seasonal influenza with vaccines: recommendations of the Advisory Committee on Immunization Practices (ACIP), 2009. MMWR Recomm Rep. Jul 31 2009;58:1-52. [Medline].

 
Previous
Next
 
Molecular and cellular changes of hemoglobin S.
Skeletal sickle cell anemia. H vertebrae. Lateral view of the spine shows angular depression of the central portion of each upper and lower endplate.
Peripheral blood with sickled cells at 400X magnification. Image courtesy of Ulrich Woermann, MD.
Peripheral blood smear with sickled cells at 1000X magnification. Image courtesy of Ulrich Woermann, MD.
Peripheral blood smear with Howell-Jolly body, indicating functional asplenism. Image courtesy of Ulrich Woermann, MD.
Effects of therapy with hydroxyurea.
Skeletal sickle cell anemia. Bone-within-bone appearance. Following multiple infarctions of the long bones, sclerosis may assume the appearance of a bone within a bone, reflecting the old cortex within the new cortex.
Table. Schedule of Laboratory Tests for Patients With Sickle Cell Disease
TestsAgeFrequency
CBC count with WBC differential,



reticulocyte count



3-24 mo



>24 mo



every 3 mo



every 6 mo



Percent Hb F6-24 mo



>24 mo



every 6 mo



annually



Renal function (creatinine, BUN, urinalysis)≥ 12 moannually
Hepatobiliary function (ALT, fractionated bilirubin)≥ 12 moannually
Pulmonary function (transcutaneous O2 saturation)≥ 12 moevery 6 mo
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.