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<< Sickle Cell Disease: A Comprehensive Review Of Inpatient Management


Evidence-based medicine requires a critical appraisal of the literature based upon study methodology and number of subjects. Not all references are equally robust. The findings of a large, prospective, randomized, and blinded trial should carry more weight than a case report.

To help the reader judge the strength of each reference, pertinent information about the study, such as the type of study and the number of patients in the study, will be included in bold type following the reference, where available.

  1. Serjeant G R. One hundred years of sickle cell disease. Br J Haematol. 2010;151(5), 425-429. (Review)
  2. Prabhakar H, Haywood C, Molokie R. Sickle cell disease in the United States: looking back and forward at 100 years of progress in management and survival. Am J Hematol. 2010:85(5), 346-353. (Review)
  3. U.S. Department of Health and Human Services. National Institutes of Health, National Heart, Lung, and Blood Institute. The management of sickle cell disease. Fourth Edition. NIH Publication No. 02-2117. 2002:1-181. (Guideline)
  4. Kauf TL, Coates TD, Huazhi L, et al. The cost of health care for children and adults with sickle cell disease. Am J Hematol. 2009:84(6);323-327. (Retrospective; 4294 patients)
  5. Tsaras G, Owusu-Ansah A, Boateng FO, et al. Complications associated with sickle cell trait: a brief narrative review. Am J Med. 2009:122(6), 507-512. (Review)
  6. Hakimi AA, Koi PT, Milhoua PM, et al. Renal medullary carcinoma: the Bronx experience. Urology. 200:70(5);878-892. (Retrospective; 9 patients)
  7. Kark A, Posey DM, Schumacher HR, et al. Sickle cell trait as a risk factor for sudden death in physical training. N Engl J Med. 1987;317(13):781-787. (Retrospective; 2 million patients)
  8. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994;330: 1639-1644. (Prospective randomized controlled study; 3674 patients)
  9. Powars DR, Hiti A, Ramicone E, et al. Outcome in hemoglobin SC disease: a four-decade observational study of clinical, hematologic, and genetic factors. Am J Hematol. 2002;70(3):206-215. (Retrospective study; 284 patients)
  10. Fitzhugh CD, Lauder N, Jonassaint JC, et al. Cardiopulmonary complications leading to premature deaths in adult patients with sickle cell disease. Am J Hematol. 2010;85(1):36. (Sickle Cell Center patient outcome review; 43 patients)
  11. Gladwin MT, Sachdev V. Cardiovascular abnormalities in sickle cell disease. J Am Coll Cardiol. 2012;59(13):1123. (Review)
  12. Voskaridou E, Christoulas D, Terpos E. Sickle-cell disease and the heart: review of the current literature. Br J Haematol. 2012;157(6):664. (Review)
  13. Covitz W, Espeland M, Gallagher D, et al. The heart in sickle cell anemia. The Cooperative Study of Sickle Cell Disease (CSSCD). Chest. 1995;108(5):1214. (Prospective multicenter study)
  14. Varat MA, Adolph RJ, Fowler NO. Cardiovascular effects of anemia. Am Heart J. 1972;83(3):415.
  15. Gerry JL, Bulkley BH, Hutchins GM. Clinicopathologic analysis of cardiac dysfunction in 52 patients with sickle cell anemia. Am J Cardiol. 1978;42(2):211. (Autopsy study; 52 patients)
  16. Zilberman MV, Du W, Das S, et al. Evaluation of left ventricular diastolic function in pediatric sickle cell disease patients. Am J Hematol. 2007;82(6):433. (Pediatric Sickle Cell Clinic patient review; 156 patients)
  17. Johnson MC, Kirkham FJ, Redline S, et al. Left ventricular hypertrophy and diastolic dysfunction in children with sickle cell disease are related to asleep and waking oxygen desaturation. Blood. 2010;116(1):16. (Observational study; 44 pediatric patients)
  18. Eddine AC, Alvarez O, Lipshultz SE, et al. Ventricular structure and function in children with sickle cell disease using conventional and tissue Doppler echocardiography. Am J Cardiol. 2012;109(9):1358. (Pediatric echocardiogram study; 55 controls, 54 SCD patients)
  19. Ng ML, Liebman J, Anslovar J, et al. Cardiovascular findings in children with sickle cell anemia. Dis Chest. 1967;52(6):788.
  20. Mueller BU, Martin KJ, Dreyer W, et al. Prolonged QT interval in pediatric sickle cell disease. Pediatr Blood Cancer. 2006;47(6):831. (Retrospective review; 142 patients)
  21. Liem RI, Young LT, Thompson AA. Prolonged QTc interval in children and young adults with sickle cell disease at steady state. Pediatr Blood Cancer. 2009;52(7):842. (Retrospective review; 76 patients aged 10 to 25 years)
  22. Klings ES, Wyszynski DF, Nolan VG, et al. Abnormal pulmonary function in adults with sickle cell anemia. Am J Respir Crit Care Med. 2006;173(11):1264. (Data analysis; 310 patients)
  23. Westwood MA, Shah F, Anderson LJ, et al. Myocardial tissue characterization and the role of chronic anemia in sickle cell cardiomyopathy. J Magn Reson Imaging. 2007;26(3):564- 568.
  24. Aessopos A, Farmakis D, Trompoukis C, et al. Cardiac involvement in sickle beta-thalassemia. Ann Hematol. 2009;88(6):557-564. (Echocardiography evaluation; 115 adult patients, 50 controls)
  25. Ballas SK. Current issues in sickle cell pain and its management. Hematology Am Soc Hematol Educ program. 2007:97-105. (Review)
  26. Rees DC, Olujohungbe AB, Parker NE, et al. Guidelines for the management of the acute painful crisis in sickle cell disease. Br J Hematol. 2003;120(5):744-752. (Guidelines)
  27. Dunlop RJ, Bennett KC. Pain management for sickle cell disease. Cochrane Database Syst Rev. 2014;4:CD003350. (Systematic review)
  28. Ballas S. Sickle cell pain: a critical appraisal. Blood. 2012:120;3647-56. (Review article)
  29. Platt O, Thorington BD, Brambilla DJ, et al. Pain in sickle cell disease rates and risk factors. N Engl J Med. 1991;325(1):11- 16. (Prospective randomized study; 3578 patients)
  30. van Beers EJ, van Tuijn CF, Nieuwkerk PT, et al. Patientcontrolled analgesia versus continuous infusion of morphine during vaso-occlusive crisis in sickle cell disease, a randomized controlled trial. Am J Hematol. 2007;82(11)82:955-960. (Prospective randomized study; 25 patients)
  31. Okomo U, Meremikwu MM. Fluid replacement therapy for acute episodes of pain in people with sickle cell disease (review). Cochrane Database Syst Rev. 2012;Jun 13;6:CD005406. (Systematic review)
  32. Vichinsky E, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med. 2000:342(25):1855-1865. (Retrospective study; 538 patients)
  33. Marti-Carvajal AJ, Conterno LO, Knight-Madden JM. Antibiotics for treating acute chest syndrome in sickle cell disease. Cochrane Database Syst Rev. 2013 Jan 31;1:CD006110. (Systematic review)
  34. Swerdlow PS. Red cell exchange in sickle cell disease. Hematology Am Soc Hematol Educ Program. 2006;48-53.
  35. Emre U, Miller ST, Gutierez M, et al. Effect of transfusion in acute chest syndrome of sickle cell disease. J Pediatr. 1995;127(6):901-904. (Prospective; 27 patients)
  36. Alhashimi D, Fedorowicz Z, Alhashimi F, et al. Blood transfusions for treating acute chest syndrome in people with sickle cell disease. Cochrane Database Syst Rev. 2010 Jan 20;(1):CD007843. (Systematic review)
  37. Bellet PS, Kalinyak KA, Shukla R, et al. Incentive spirometry to prevent acute pulmonary diseases in sickle cell diseases. N Engl J Med. 1995;333(11):699-703. (Prospective study; 29 patients)
  38. Knight-Madden JM, Hambleton IR. Inhaled bronchodilators for acute chest syndrome in people with sickle cell disease. Cochrane Database Syst Rev. 2012 Jul 11;7:CD003733. (Systematic review)
  39. Charache S, Terrin ML, Moore RD, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med. 1995;332(20):1317-1322. (Prospective randomized study; 299 patients)
  40. Steinberg MH, Barton F, Castro O, et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia. JAMA. 2003;289(13):1645-1651. (Prospective study; 299 patients)
  41. Platt OS. Hydroxyurea for the treatment of sickle cell anemia. N Engl J Med. 2008;358(13):1362-1369. (Review)
  42. Telen MJ. Principles and problems of transfusion in sickle cell disease. Semin Hematol. 2001;38(4)315-323. (Review article)
  43. Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. N Engl J Med. 1995;33(4):206-213. (Prospective; 604 patients)
  44. Adams RJ, McKie VC, Hsu L, et al. Prevention of a first stroke by transfusion in children with sickle cell anemia and abnormal results of transcranial Doppler ultrasonography. N Engl J Med. 1998;339(1):5-11. (Prospective study; 130 patients)
  45. Vichinsky E, Onyekwere O, Porter J, et al. A randomized comparison of deferasirox versus deferoxamine for the treatment of transfusional iron overload in sickle cell disease. Br J Haematol. 2006;136(3):501-508. (Randomized open-label phase II trial; 195 patients)
  46. Musallam KM, Taher AT. Iron chelation therapy for transfusional iron overload: a swift evolution. Hemoglobin. 2011;35(5-6):565-573. (Review)
  47. The National Institute for Children’s Health Quality (NICHQ) website. Available at: http://www.nichq.org/. Accessed June 3, 2013. (Website)
  48. Benjamin LJ, Swinson GI, Nagel RL. Sickle cell anemia day hospital: an approach for the management of uncomplicated pain crises. Blood. 2000;95(4):1130-1136. (Prospective; 2574 patients)
  49. Johnson FL, Look AT, Gockerman J, et al. Bone-marrow transplantation in a patient with sickle-cell anemia. N Eng J Med. 1984;311(12);780-783. (Case report; 1 patient)
  50. Kharbanda S, Smith AR, Hutchinson SK, et al. Unrelated donor allogeneic hematopoietic stem cell transplantation for patients with hemoglobinopathies using a reduced-intensity conditioning regimen and third-party mesenchymal stromal cells. Biol Blood Marrow Transplant. 2014;20(4):581-586. (Pilot study; 6 patients)
  51. Bernaudin F, Souillet G, Vannier JP, et al. Bone marrow transplantation (BMT) in 14 children with severe sickle disease (SCD); the French experience. Bone Marrow Transplant. 1993;12 Suppl 1:118-121. (Prospective; 14 patients)
  52. Walters MC, Storb R, Patience M, et al. Impact of bone marrow transplantation for symptomatic sickle cell disease: an interim report. Multicenter investigation of bone marrow transplantation for sickle cell disease. Blood. 2000;95(6):1918- 1924. (Prospective study, 50 patients)
  53. Bernaudin F, Socie G, Kuentz M, et al. Long-term results of related myeloablative stem-cell transplantation to cure sickle cell disease. Blood. 2007;110(7):2749-2756. (Prospective study; 70 patients)
  54. Hsieh MM, Kang EM, Practice, Fitzhugh CD, et al. Allogenic hematopoietic stem-cell transplantation for sickle cell disease. N Engl J Med. 2009;361(24):2309-2317. (Prospective study; 10 patients)
  55. Anie KA, Steptoe A. Pain, mood and opioid medication use in sickle cell disease. Hematol J. 2003;4(1):71-73. (Prospective study; 21 patients)
  56. Lottenberg R, Hassell KL. An evidence-based approach to the treatment of adults with sickle cell disease. Hematology Am Soc Hematol Educ Program. 2005:58-65. (Review)
  57. Anie KA. Psychological complications in sickle cell disease. Br J Hematol. 2005;129(6):723-729. (Review)
  58. McClish DK, Penberthy LT, Bovbjerg VE, et al. Health related quality of life in sickle cell patients: The PiSCES project. Health Qual Life Outcomes. 2005 Aug 29;3:50. (Prospective study; 308 patients)
  59. Dampier C, LeBeau P, Rhee S, et al. Health-related quality of life in adults with sickle cell disease (SCD): a report from the comprehensive sickle cell centers clinical trial consortium. Am J Hematol. 2011;86(2):203-205. (Retrospective study; 1046 patients)
  60. Gil KM, Abrams MR, Phillips G, et al. Sickle cell disease pain: relation of coping strategies to adjustment. J Consult Clin Psychol. 1989;57(6):725-731. (Prospective study; 79 patients)
  61. Anie KA, Green J. Psychological therapies for sickle cell disease and pain. Cochrane Database Syst Rev. 2002;(2):CD001916. (Systematic review)
  62. Moore RD, Charache S, Terrin ML, et al. (2000) Cost-effectiveness of hydroxyurea in sickle cell anemia. Am J Hematol. 2000;64(1):26-31. (Prospective study; 299 patients)

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Last Modified: 07/23/2017
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