PERIOPERATIVE MANAGEMENT OF SICKLE CELL DISEASE: A NARRATIVE REVIEW
Main Article Content
Keywords
Sickle cell disease, perioperative, transfusion
Abstract
An estimated 30 million people worldwide have sickle cell disease (SCD). Emergent and non-emergent surgical procedures in SCD have been associated with relatively increased risks of peri-operative mortality, vaso-occlussive (painful) crisis, acute chest syndrome, post-operative infections, congestive heart failure, cerebrovascular accident and acute kidney injury. Pre-operative assessment must include careful review of the patient’s known crisis triggers, baseline hematologic profile, usual transfusion requirements, pre-existing organ dysfunction and narcotic use. Use of preoperative blood transfusions should be selective and decisions individualized based on the baseline hemoglobin, surgical procedure and anticipated volume of blood loss. Intra- and post-operative management should focus on minimizing hypoxia, hypothermia, acidosis, and intravascular volume depletion. Pre- and post-operative incentive spirometry use should be encouraged.
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References
REFERENCES
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3. Stuart MJ, Nagel RL. Sickle Cell disease. Lancet 2004; 364:1343-1360.
4. Ashley-Koch A, Yang Q, Olney R S. Sickle hemoglobin (Hb S) allele and sickle cell disease: a HuGE review. American Journal of Epidemiology 2000; 151(9): 839-845.
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6. Hassell K L. Population estimates of sickle cell disease in the US. American journal of preventive medicine 2010; 38(4): S512-S521.
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8. Pearson HA. Sickle cell diseases: diagnosis and management in infancy and childhood. Pediatrics in review/American Academy of Pediatrics. 1987 Oct;9(4):121-30.
9. Noguchi CT, Schechter AN, Rodgers GP. Sickle cell disease pathophysiology. Baillière's clinical haematology 1993; 6(1): 57-91.
10. Kato GJ, Hebbel RP, Steinberg MH, Gladwin MT. Vasculopathy in sickle cell disease: Biology, pathophysiology, genetics, translational medicine, and new research directions. American journal of hematology 2009; 84(9): 618-625.
11. Manwani D, Frenette PS. Vaso-occlusion in sickle cell disease: pathophysiology and novel targeted therapies. Blood 2013; 122(24): 3892-3898.
12. Chirico EN, Pialoux V. Role of oxidative stress in the pathogenesis of sickle cell disease. IUBMB life 2012; 64(1): 72-80.
13. Kassim AA, DeBaun MR. Sickle cell disease, vasculopathy, and therapeutics. Annual review of medicine 2013; 64: 451-466.
14. Frenette PS. Sickle cell vaso-occlusion: multistep and multicellular paradigm. Current opinion in hematology 2002; 9(2): 101-106.
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45. Haynes J, Allison RC. Pulmonary edema: complication in the management of sickle cell pain crisis. The American journal of medicine 1986; 80(5): 833-840.
46. Kaul DK, Fabry ME, Costantini F, Rubin EM, Nagel RL. In vivo demonstration of red cell-endothelial interaction, sickling and altered microvascular response to oxygen in the sickle transgenic mouse. Journal of Clinical Investigation 1995; 96(6): 2845.
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48. Zipursky A, Robieux IC, Brown EJ, et al. Oxygen therapy in sickle cell disease. Journal of Pediatric Hematology/Oncology 1992; 14(3): 222-228.
49. Reinhard EH, Moore CV, Dubach R, Wade LJ. Depressant effects of high concentrations of inspired oxygen on erythrocytogenesis. Observations on patients with sickle cell anemia with a description of the observed toxic manifestations of oxygen. Journal of Clinical Investigation 1944; 23(5): 682.
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54. Alotaibi GS, Alsaleh K, Bolster L, Sean McMurtry M, Wu C. Preoperative transfusion in patients with sickle cell disease to prevent perioperative complications: A systematic review and meta-analysis. Hematology 2014; 19(8): 463-471.
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57. Davies SC, Roberts-Harewood M. Blood transfusion in sickle cell disease. Blood reviews 1997; 11(2): 57-71.
58. Yawn B P, Buchanan GR, Afenyi-Annan AN, et al. (2014). Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA 2014; 312(10):1033-1048.
59. Griffin TC, Buchanan GR. Elective surgery in children with sickle cell disease without preoperative blood transfusion. Journal of pediatric surgery 1993; 28(5): 681-685.
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62. Wali YA, al Okbi H, al Abri R. A comparison of two transfusion regimens in the perioperative management of children with sickle cell disease undergoing adenotonsillectomy. Pediatr Hematol Oncol. 2003; 20(1):7-13.
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64. Rubenstein E. Studies on the relationship of temperature to sickle cell anemia. The American journal of medicine 1961; 30(1): 95-98.
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1. Modell B, Darlison M. global epidemiology of haemoglobin disorders and derived service indicators. Bulletin of the World Health Organization 2008; 86(6):480-487.
2. Tsaras G, Owusu-Ansah A, Boateng FO, Amoateng-Adjepong Y. Complications associated with sickle cell trait: a brief narrative review. The American Journal of Medicine, 2009; 122(6): 507-512.
3. Stuart MJ, Nagel RL. Sickle Cell disease. Lancet 2004; 364:1343-1360.
4. Ashley-Koch A, Yang Q, Olney R S. Sickle hemoglobin (Hb S) allele and sickle cell disease: a HuGE review. American Journal of Epidemiology 2000; 151(9): 839-845.
5. Steinberg MH, Rodgers GP. Pathophysiology of sickle cell disease: role of cellular and genetic modifiers. Seminars in hematology 2001; 38 (4): 299-306.
6. Hassell K L. Population estimates of sickle cell disease in the US. American journal of preventive medicine 2010; 38(4): S512-S521.
7. Shafer FE, Lorey F, Cunningham GC, et al. Newborn screening for sickle cell disease: 4 years of experience from California's newborn screening program. Journal of pediatric hematology/oncology 1996; 18(1): 36-41.
8. Pearson HA. Sickle cell diseases: diagnosis and management in infancy and childhood. Pediatrics in review/American Academy of Pediatrics. 1987 Oct;9(4):121-30.
9. Noguchi CT, Schechter AN, Rodgers GP. Sickle cell disease pathophysiology. Baillière's clinical haematology 1993; 6(1): 57-91.
10. Kato GJ, Hebbel RP, Steinberg MH, Gladwin MT. Vasculopathy in sickle cell disease: Biology, pathophysiology, genetics, translational medicine, and new research directions. American journal of hematology 2009; 84(9): 618-625.
11. Manwani D, Frenette PS. Vaso-occlusion in sickle cell disease: pathophysiology and novel targeted therapies. Blood 2013; 122(24): 3892-3898.
12. Chirico EN, Pialoux V. Role of oxidative stress in the pathogenesis of sickle cell disease. IUBMB life 2012; 64(1): 72-80.
13. Kassim AA, DeBaun MR. Sickle cell disease, vasculopathy, and therapeutics. Annual review of medicine 2013; 64: 451-466.
14. Frenette PS. Sickle cell vaso-occlusion: multistep and multicellular paradigm. Current opinion in hematology 2002; 9(2): 101-106.
15. Kaul DK, Finnegan E, Barabino GA. Sickle red cell–endothelium interactions. Microcirculation 2009; 16(1): 97-111.
16. McCurdy PR, Sherman AS. Irreversibly sickled cells and red cell survival in sickle cell anemia: a study with both DF32P and 51CR. The American journal of medicine 1978; 64(2): 253-258.
17. Eadie GS, Brown IW, Analytical review: red blood cell survival studies. Blood 1953; 8(12): 1110-1136.
18. Holzmann L, Finn H, Lichtman HC, Harmel MH. Anesthesia in patients with sickle cell disease: a review of 112 cases. Anesthesia & Analgesia 1969; 48(4): 566-572.
19. Patterson RH, Wilson H, Diggs LW. Sickle-Cell Anemia: a Surgical Problem. II. Further Observation on the Surgical Implications of Sickle-Cell Anemia. Surgery 1950; 28(2): 393-402.
20. Browne RA. Anaesthesia in patients with sickle-cell anaemia. British journal of anaesthesia 1965; 37(3): 181-188.
21. Shapiro ND, Poe MF. Sickle-cell disease: an anesthesiological problem. Anesthesiology 1955; 16(5): 771.
22. Oduro KA. Anaesthesia and sickle cell haemoglobin. British journal of anaesthesia 1973; 45(1):123.
23. Oduro KA, Searle JF. Anaesthesia in sickle-cell states: a plea for simplicity. British medical journal 1972; 4(5840): 596-8.
24. Searle JF. Anaesthesia in sickle cell states. Anaesthesia 1973; 28(1): 48-58.
25. Firth PG, Head CA. Sickle cell disease and anesthesia. Anesthesiology 2004;101: 766-786.
26. Vichinsky EP, Neumayr LD, Haberkern C, et al. The perioperative complication rate of orthopedic surgery in sickle cell disease: report of the National Sickle Cell Surgery Study Group. American journal of hematology 1999, 62(3), 129-138.
27. Koshy M, Weiner SJ, Miller ST, et al. Surgery and anesthesia in sickle cell disease. Cooperative Study of Sickle Cell Diseases. Blood 1995; 86(10): 3676-3684.
28. Haberkern CM, Neumayr LD, Orringer EP, et al. Cholecystectomy in sickle cell anemia patients: perioperative outcome of 364 cases from the National Preoperative Transfusion Study. Blood 1997; 89(5):1533-1542.
29. Buck J, Davies SC. Surgery in sickle cell disease. Hematology/oncology clinics of North America 2005; 19(5): 897-902.
30. Ballas SK, Lieff S, Benjamin LJ, et al. Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol. 2010 Jan;85(1):6-13
31. Balfour IC, Covitz W, Davis H, et al. Cardiac size and function in children with sickle cell anemia. American heart journal 1984;108(2): 345-350.
32. Denenberg BS, Criner G, Jones R, Spann JF. Cardiac function in sickle cell anemia. The American journal of cardiology 1983; 51(10): 1674-1678.
33. Sutton LL, Castro O., Cross DJ, Spencer JE, Lewis JF. Pulmonary hypertension in sickle cell disease. The American journal of cardiology 1994; 74(6): 626-628.
34. Gerry JL, Bulkley BH, Hutchins GM. Clinicopathologic analysis of cardiac dysfunction in 52 patients with sickle cell anemia. The American journal of cardiology 1978; 42(2): 211-216.
35. Mansi IA, Rosner F . Myocardial infarction in sickle cell disease. Journal of the National Medical Association 2002; 94(6): 448.
36. Gladwin MT, Vichinsky E. Pulmonary complications of sickle cell disease. New England Journal of Medicine 2008; 359(21): 2254-2265.
37. Miller AC, Gladwin MT. Pulmonary complications of sickle cell disease. American journal of respiratory and critical care medicine 2012; 185(11): 1154-1165.
38. Vichinsky EP, Styles LA, Colangelo LH et al. Acute chest syndrome in sickle cell disease: clinical presentation and course. Blood 1997; 89(5), 1787-1792.
39. Raghuram N, Pettignano R, Gal AA, Harsch A, Adamkiewicz TV. Plastic bronchitis: an unusual complication associated with sickle cell disease and the acute chest syndrome. Pediatrics 1997; 100(1): 139-142.
40. Leong MA, Dampier C, Varlotta L, Allen J L. Airway hyperreactivity in children with sickle cell disease. The Journal of pediatrics 1997;131(2): 278-283.
41. Stein PD, Beemath A, Meyers FA, Skaf E, Olson RE. Deep venous thrombosis and pulmonary embolism in hospitalized patients with sickle cell disease. The American journal of medicine 2006; 119(10): 897-e7.
42. Platt OS, Thorington BD, Brambilla DJ, et al. Pain in sickle cell disease: rates and risk factors. New England Journal of Medicine 1991; 325(1): 11-16.
43. Coran AG, Ballantine TV, Horwitz DL, Herman CM. The effect of crystalloid resuscitation in hemorrhagic shock on acid-base balance: A comparison between normal saline and Ringer's lactate solutions. Surgery 1971; ,69(6): 874-880.
44. Guy RB, Gavrilis PK, Rothenberg SP. In vitro and in vivo effect of hypotonic saline on the sickling phenomenon. The American journal of the medical sciences 1973; 266(4): 267-277.
45. Haynes J, Allison RC. Pulmonary edema: complication in the management of sickle cell pain crisis. The American journal of medicine 1986; 80(5): 833-840.
46. Kaul DK, Fabry ME, Costantini F, Rubin EM, Nagel RL. In vivo demonstration of red cell-endothelial interaction, sickling and altered microvascular response to oxygen in the sickle transgenic mouse. Journal of Clinical Investigation 1995; 96(6): 2845.
47. Embury SH, Garcia JF, Mohandas N, Pennathur-Das R, Clark MR. Effects of oxygen inhalation on endogenous erythropoietin kinetics, erythropoiesis, and properties of blood cells in sickle-cell anemia. New England Journal of Medicine 1984; 311(5): 291-295.
48. Zipursky A, Robieux IC, Brown EJ, et al. Oxygen therapy in sickle cell disease. Journal of Pediatric Hematology/Oncology 1992; 14(3): 222-228.
49. Reinhard EH, Moore CV, Dubach R, Wade LJ. Depressant effects of high concentrations of inspired oxygen on erythrocytogenesis. Observations on patients with sickle cell anemia with a description of the observed toxic manifestations of oxygen. Journal of Clinical Investigation 1944; 23(5): 682.
50. Blaisdell CJ, Goodman S, Clark K, Casella JF, Loughlin GM. Pulse oximetry is a poor predictor of hypoxemia in stable children with sickle cell disease. Archives of pediatrics & adolescent medicine 2000; 154(9): 900-903.
51. Bellet PS, Kalinyak KA, Shukla R, Gelfand MJ, Rucknagel DL. Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases. New England Journal of Medicine 1995; 333(11): 699-703.
52. Jacob B, Amoateng-Adjepong Y, Rasakulasuriar S, Manthous CA, Haddad R. Preoperative pulmonary function tests do not predict outcome after coronary artery bypass. Connecticut medicine 1997; 61(6): 327-332.
53. Smetana G W. Preoperative pulmonary evaluation. New England Journal of Medicine 1999; 340(12): 937-944.
54. Alotaibi GS, Alsaleh K, Bolster L, Sean McMurtry M, Wu C. Preoperative transfusion in patients with sickle cell disease to prevent perioperative complications: A systematic review and meta-analysis. Hematology 2014; 19(8): 463-471.
55. Howard J, Malfroy M, Llewelyn C, et al. The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: a randomised, controlled, multicentre clinical trial.The Lancet 2013; 381(9870): 930-938.
56. Edwin F, Aniteye E, Tettey M, et al. Hypothermic cardiopulmonary bypass without exchange transfusion in sickle-cell patients: a matched-pair analysis. Interactive cardiovascular and thoracic surgery 2014; 19(5): 771-776.
57. Davies SC, Roberts-Harewood M. Blood transfusion in sickle cell disease. Blood reviews 1997; 11(2): 57-71.
58. Yawn B P, Buchanan GR, Afenyi-Annan AN, et al. (2014). Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA 2014; 312(10):1033-1048.
59. Griffin TC, Buchanan GR. Elective surgery in children with sickle cell disease without preoperative blood transfusion. Journal of pediatric surgery 1993; 28(5): 681-685.
60. Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. The Preoperative Transfusion in Sickle Cell Disease Study Group. N Engl J Med. 1995; 333(4):206-13.
61. Al-Samak ZM, Al-Falaki MM, Pasha AA. Assessment of perioperative transfusion therapy and complications in sickle cell disease patients undergoing surgery. Middle East J Anesthesiol. 2008;19(5):983-95.
62. Wali YA, al Okbi H, al Abri R. A comparison of two transfusion regimens in the perioperative management of children with sickle cell disease undergoing adenotonsillectomy. Pediatr Hematol Oncol. 2003; 20(1):7-13.
63. Firth PG. Anaesthesia for peculiar cells—a century of sickle cell disease. British journal of anaesthesia 2005; 95(3): 287-299.
64. Rubenstein E. Studies on the relationship of temperature to sickle cell anemia. The American journal of medicine 1961; 30(1): 95-98.
65. Edwin F, Aniteye E, Tamatey M, Frimpong-Boateng K. eComment: Cardiopulmonary bypass without exchange transfusion in sickle cell disease–An update. Interactive cardiovascular and thoracic surgery 2010; 10(1): 68-69.
66. Gross ML, Schwedler M, Bischoff RJ, Kerstein MD. Impact of anesthetic agents on patients with sickle cell disease. The American surgeon 1993; 59(4): 261-264.
67. Yaster M, Tobin JR, Billett C, Casella JF, Dover G. Epidural analgesia in the management of severe vaso-occlusive sickle cell crisis. Pediatrics 1994; 93(2): 310-315.
68. Haberkern CM, Neumayr LD, Orringer EP, et al. Cholecystectomy in sickle cell anemia patients: perioperative outcome of 364 cases from the National Preoperative Transfusion Study. Blood 1997, 89(5), 1533-1542.
69. Tagge EP, Othersen HB, Jackson SM, et al. Impact of laparoscopic cholecystectomy on the management of cholelithiasis in children with sickle cell disease. Journal of pediatric surgery 1994; 29(2): 209-213.
70. Al-Mulhim AS, Al-Mulhim A A. Laparoscopic cholecystectomy in 427 adults with sickle cell disease: a single-center experience. Surgical endoscopy 2009; 23(7): 1599-1602.
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