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Review

Management of Sickle Cell Disease Complications Beyond Acute Chest Syndrome

ORCID Icon, , , &
Pages 101-114 | Published online: 25 Feb 2021

References

  • Fernandes Q. Therapeutic strategies in sickle cell anemia: the past present and future. Life Sci. 2017;178:100–108. doi:10.1016/j.lfs.2017.03.025
  • Vekilov PG. Sickle-cell haemoglobin polymerization: is it the primary pathogenic event of sickle-cell anaemia? Br J Haematol. 2007;139(2):173–184. doi:10.1111/j.1365-2141.2007.06794.x
  • Moerdler S, Manwani D. New insights into the pathophysiology and development of novel therapies for sickle cell disease. Hematology Am Soc Hematol Educ Program. 2018;2018(1):493–506. doi:10.1182/asheducation-2018.1.493
  • Piccin A, Murphy C, Eakins E, et al. Insight into the complex pathophysiology of sickle cell anaemia and possible treatment. Eur J Haematol. 2019;102(4):319–330. doi:10.1111/ejh.13212
  • Zhang D, Xu C, Manwani D, Frenette PS. Neutrophils, platelets, and inflammatory pathways at the nexus of sickle cell disease pathophysiology. Blood. 2016;127(7):801–809. doi:10.1182/blood-2015-09-618538
  • Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018–2031. doi:10.1016/S0140-6736(10)61029-X
  • Thein MS, Igbineweka NE, Thein SL. Sickle cell disease in the older adult. Pathology. 2017;49(1):1–9. doi:10.1016/j.pathol.2016.10.002
  • Payne R. Sickle cell anemia and pain: will data prevail over beliefs? Ann Emerg Med. 2009;53(5):596–597. doi:10.1016/j.annemergmed.2008.10.022
  • Shah F, Dwivedi M. Pathophysiology and recent therapeutic insights of sickle cell disease. Ann Hematol. 2020;99(5):925–935. doi:10.1007/s00277-020-03977-9
  • Adebiyi MG, Manalo JM, Xia Y. Metabolomic and molecular insights into sickle cell disease and innovative therapies. Blood Adv. 2019;3(8):1347–1355. doi:10.1182/bloodadvances.2018030619
  • Singh PC, Ballas SK. Emerging drugs for sickle cell anemia. Expert Opin Emerg Drugs. 2015;20(1):47–61. doi:10.1517/14728214.2015.985587
  • Power-Hays A, McGann PT. When actions speak louder than words - racism and sickle cell disease. N Engl J Med. 2020;383:1902–1903. doi:10.1056/NEJMp2022125
  • Bundy DG, Strouse JJ, Casella JF, Miller MR. Urgency of emergency department visits by children with sickle cell disease: a comparison of 3 chronic conditions. Acad Pediatr. 2011;11(4):333–341. doi:10.1016/j.acap.2011.04.006
  • Centers for Disease Control and Prevention. Data and statistics: sickle cell disease; 2019. Available from: https://www.cdc.gov/ncbddd/sicklecell/data.html. Accessed November 2, 2020.
  • National Organization for Rare Disorders. Cystic Fibrosis; 2017. Available from: https://rarediseases.org/rare-diseases/cystic-fibrosis/. Accessed November 2, 2020.
  • Farooq F, Mogayzel PJ, Lanzkron S, Haywood C, Strouse JJ. Comparison of US federal and foundation funding of research for sickle cell disease and cystic fibrosis and factors associated with research productivity. JAMA Netw Open. 2020;3(3):e201737. doi:10.1001/jamanetworkopen.2020.1737
  • Haywood C, Lanzkron S, Bediako S, et al. Perceived discrimination, patient trust, and adherence to medical recommendations among persons with sickle cell disease. J Gen Intern Med. 2014;29(12):1657–1662. doi:10.1007/s11606-014-2986-7
  • Lee L, Smith-Whitley K, Banks S, Puckrein G. Reducing health care disparities in sickle cell disease: a review. Public Health Rep. 2019;134(6):599–607. doi:10.1177/0033354919881438
  • Pulte D, Lovett PB, Axelrod D, Crawford A, McAna J, Powell R. Comparison of emergency department wait times in adults with sickle cell disease versus other painful etiologies. Hemoglobin. 2016;40(5):330–334. doi:10.1080/03630269.2016.1232272
  • Hsu LL, Green NS, Donnell Ivy E, et al. Community health workers as support for sickle cell care. Am J Prev Med. 2016;51(1 Suppl 1):S87–S98. doi:10.1016/j.amepre.2016.01.016
  • Adams-Graves P, Bronte-Jordan L. Recent treatment guidelines for managing adult patients with sickle cell disease: challenges in access to care, social issues, and adherence. Expert Rev Hematol. 2016;9(6):541–552. doi:10.1080/17474086.2016.1180242
  • Whiteman LN, Lanzkron S, Stewart RW, Haywood C, Strouse JJ, Feldman L. Quality improvement process in a sickle cell infusion center. Am J Med. 2015;128(5):541–544. doi:10.1016/j.amjmed.2014.11.020
  • Corvest V, Blais S, Dahmani B, et al. [Cerebral vasculopathy in children with sickle cell disease: key issues and the latest data]. Arch Pediatr. 2018;25(1):63–71. French. doi:10.1016/j.arcped.2017.11.015
  • Kassim AA, Galadanci NA, Pruthi S, DeBaun MR. How I treat and manage strokes in sickle cell disease. Blood. 2015;125(22):3401–3410. doi:10.1182/blood-2014-09-551564
  • Adams RJ. TCD in sickle cell disease: an important and useful test. Pediatr Radiol. 2005;35(3):229–234. doi:10.1007/s00247-005-1409-7
  • Medepalli V, Bajaj S, Bajaj M, Badamosi N Recurrent multifocal calvarial bony infarcts and intracranial hematomas in a patient with sickle cell anemia; 2020. Available from: https://www.eurorad.org/case/16842. Accessed November 2, 2020.
  • Hamm J, Rathore N, Lee P, et al. Cranial epidural hematomas: a case series and literature review of this rare complication associated with sickle cell disease. Pediatr Blood Cancer. 2017;64(3):e26237. doi:10.1002/pbc.26237
  • Liem RI, Lanzkron S, Coates T, et al. American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease. Blood Adv. 2019;3(23):3867–3897. doi:10.1182/bloodadvances.2019000916
  • Wyrick-Glatzel J, Conway-Klaassen J. Clinical utility of the IRF: assessment of erythroid regeneration following parvo B19 infection. Clin Lab Sci. 2002;15(4):208–212.
  • Shah R, Taborda C, Chawla S. Acute and chronic hepatobiliary manifestations of sickle cell disease: a review. World J Gastrointest Pathophysiol. 2017;8(3):108–116. doi:10.4291/wjgp.v8.i3.108
  • Anele UA, Le BV, Resar LMS, Burnett AL. How I treat priapism. Blood. 2015;125(23):3551–3558. doi:10.1182/blood-2014-09-551887
  • Martí‐Carvajal AJ, Solà I, Agreda‐Pérez LH. Treatment for avascular necrosis of bone in people with sickle cell disease. Cochrane Database Syst Rev. 2016;8.
  • Lebensburger JD, Bemrich-Stolz CJ, Howard TH. Barriers in transition from pediatrics to adult medicine in sickle cell anemia. J Blood Med. 2012;3:105–112. doi:10.2147/JBM.S32588
  • Ogu UO, Billett HH. Comorbidities in sickle cell disease: adult providers needed! Indian J Med Res. 2018;147(6):527–529. doi:10.4103/ijmr.IJMR_1019_18
  • Jain S, Bakshi N, Krishnamurti L. Acute chest syndrome in children with sickle cell disease. Pediatr Allergy Immunol Pulmonol. 2017;30(4):191–201. doi:10.1089/ped.2017.0814
  • Ballas SK, Lieff S, Benjamin LJ, et al. Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol. 2010;85(1):6–13. doi:10.1002/ajh.21550
  • Vichinsky EP, 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. doi:10.1056/NEJM200006223422502
  • Dampier C, Palermo TM, Darbari DS, Hassell K, Smith W, Zempsky W. AAPT diagnostic criteria for chronic sickle cell disease pain. J Pain. 2017;18(5):490–498. doi:10.1016/j.jpain.2016.12.016
  • Edwards LY, Edwards CL. Psychosocial treatments in pain management of sickle cell disease. J Natl Med Assoc. 2010;102(11):1084–1094. doi:10.1016/S0027-9684(15)30737-9
  • Williams H, Tanabe P. Sickle cell disease: a review of nonpharmacological approaches for pain. J Pain Symptom Manage. 2016;51(2):163–177. doi:10.1016/j.jpainsymman.2015.10.017
  • Shet AS, Wun T. How I diagnose and treat venous thromboembolism in sickle cell disease. Blood. 2018;132(17):1761–1769. doi:10.1182/blood-2018-03-822593
  • Olatunya OS, Albuquerque DM, Adekile AD, Costa FF. Evaluation of sociodemographic, clinical, and laboratory markers of sickle leg ulcers among young Nigerians at a tertiary health institution. Niger J Clin Pract. 2018;21(7):882–887. doi:10.4103/njcp.njcp_4_18
  • Serjeant GR, Serjeant BE, Mohan JS, Clare A. Leg ulceration in sickle cell disease: medieval medicine in a modern world. Hematol Oncol Clin North Am. 2005;19(5):943–ix. doi:10.1016/j.hoc.2005.08.005
  • Minniti CP, Kato GJ. Critical reviews: how we treat sickle cell patients with leg ulcers. Am J Hematol. 2016;91(1):22–30. doi:10.1002/ajh.24134
  • Vasconcelos A, Prior AR, Ferrão A, Morais A. An adolescent with sickle cell anaemia experiencing disease-related complications: priapism and leg ulcer–a management challenge. BMJ Case Rep. 2012;2012:bcr1120115146–bcr1120115146. doi:10.1136/bcr.11.2011.5146
  • Smith-Whitley K. Reproductive issues in sickle cell disease. Blood. 2014;124(24):3538–3543. doi:10.1182/blood-2014-07-577619
  • Taddesse A, Woldie IL, Khana P, et al. Hypogonadism in patients with sickle cell disease: central or peripheral? Acta Haematol. 2012;128(2):65–68. doi:10.1159/000337344
  • Madu AJ, Ubesie A, Ocheni S, et al. Priapism in homozygous sickle cell patients: important clinical and laboratory associations. Med Princ Pract. 2014;23(3):259–263. doi:10.1159/000360608
  • Serjeant GR, Singhal A, Hambleton IR. Sickle cell disease and age at menarche in Jamaican girls: observations from a cohort study. Arch Dis Child. 2001;85(5):375–378. doi:10.1136/adc.85.5.375
  • Smith-Whitley K. Complications in pregnant women with sickle cell disease. Hematology Am Soc Hematol Educ Program. 2019;2019(1):359–366. doi:10.1182/hematology.2019000039
  • Nnoli A, Seligman NS, Dysart K, Baxter JK, Ballas SK. Opioid utilization by pregnant women with sickle cell disease and the risk of neonatal abstinence syndrome. J Natl Med Assoc. 2018;110(2):163–168. doi:10.1016/j.jnma.2017.04.002
  • Johns Hopkins University & Medicine. COVID-19 map; 2020. Available from: https://coronavirus.jhu.edu/map.html. Accessed November 2, 2020.
  • Sickle Cell Society. Coronavirus (COVID-19) & sickle cell disorder; 2020. Available from: https://www.sicklecellsociety.org/coronavirus-and-scd/. Accessed November 2, 2020.
  • Medical College of Wisconsin. SECURE-SCD registry; 2020. Available from: https://covidsicklecell.org/updates-data/. Accessed November 2, 2020.
  • Arlet JB, de Luna G, Khimoud D, et al. Prognosis of patients with sickle cell disease and COVID-19: a French experience. Lancet Haematol. 2020;7(9):e632–e634. doi:10.1016/S2352-3026(20)30204-0
  • Hussain FA, Njoku FU, Saraf SL, Molokie RE, Gordeuk VR, Han J. COVID-19 infection in patients with sickle cell disease. Br J Haematol. 2020;189(5):851–852. doi:10.1111/bjh.16734
  • Sahu KK, Siddiqui AD, Cerny J. Managing sickle cell patients with COVID-19 infection: the need to pool our collective experience. Br J Haematol. 2020;190(2):e86–e89. doi:10.1111/bjh.16880
  • American Society of Hematology. COVID-19; 2020. Available from: https://www.hematology.org/covid-19. Accessed November 2, 2020.
  • Baker JP. Sickle Cell Anemia. Trans Am Clin Climatol Assoc. 1941;57:203–212.
  • Howard J. Sickle cell disease: when and how to transfuse. Hematology Am Soc Hematol Educ Program. 2016;2016(1):625–631. doi:10.1182/asheducation-2016.1.625
  • Adams RJ, McKie VC, Hsu L, 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. 1998;339(1):5–11. doi:10.1056/NEJM199807023390102
  • Adams RJ, Brambilla D. Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. N Engl J Med. 2005;353(26):2769–2778.
  • Ware RE, Helms RW. Stroke with transfusions changing to hydroxyurea (SWiTCH). Blood. 2012;119(17):3925–3932. doi:10.1182/blood-2011-11-392340
  • DeBaun MR, Gordon M, McKinstry RC, et al. Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia. N Engl J Med. 2014;371(8):699–710. doi:10.1056/NEJMoa1401731
  • Ware RE, Davis BR, Schultz WH, et al. Hydroxycarbamide versus chronic transfusion for maintenance of transcranial doppler flow velocities in children with sickle cell anaemia-TCD With Transfusions Changing to Hydroxyurea (TWiTCH): a multicentre, open-label, Phase 3, non-inferiority trial. Lancet. 2016;387(10019):661–670. doi:10.1016/S0140-6736(15)01041-7
  • Howard J, Malfroy M, Llewelyn C, et al. The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: a randomised, controlled, multicentre clinical trial. Lancet. 2013;381(9870):930–938. doi:10.1016/S0140-6736(12)61726-7
  • Koshy M, Burd L, Wallace D, Moawad A, Baron J. Prophylactic red-cell transfusions in pregnant patients with sickle cell disease. A randomized cooperative study. N Engl J Med. 1988;319(22):1447–1452. doi:10.1056/NEJM198812013192204
  • Charache S, Terrin ML, Moore RD, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the multicenter study of hydroxyurea in sickle cell anemia. N Engl J Med. 1995;332(20):1317–1322. doi:10.1056/NEJM199505183322001
  • U.S. Food and Drug Administration. FDA approves hydroxyurea for treatment of pediatric patients with sickle cell anemia; 2020. https://www.fda.gov/drugs/resources-information-approved-drugs/fda-approves-hydroxyurea-treatment-pediatric-patients-sickle-cell-anemia. Accessed October 18, 2020.
  • McGann PT, Ware RE. Hydroxyurea therapy for sickle cell anemia. Expert Opin Drug Saf. 2015;14(11):1749–1758. doi:10.1517/14740338.2015.1088827
  • Verma HK, Lakkakula S, Lakkakula BVKS. Retrospection of the effect of hydroxyurea treatment in patients with sickle cell disease. Acta Haematol Pol. 2018;49(1):1. doi:10.2478/ahp-2018-0001
  • Niihara Y, Miller ST, Kanter J, et al. A phase 3 trial of l-glutamine in sickle cell disease. N Engl J Med. 2018;379(3):226–235. doi:10.1056/NEJMoa1715971
  • Ogu UO, Thomas M, Chan F, et al. L-glutamine use in adults with sickle cell disease: clinical trials where success meets reality. Am J Hematol. 2020.
  • Matsui NM, Borsig L, Rosen SD, Yaghmai M, Varki A, Embury SH. P-selectin mediates the adhesion of sickle erythrocytes to the endothelium. Blood. 2001;98(6):1955–1962. doi:10.1182/blood.V98.6.1955
  • Ataga KI, Kutlar A, Kanter J, et al. Crizanlizumab for the prevention of pain crises in sickle cell disease. N Engl J Med. 2017;376(5):429–439. doi:10.1056/NEJMoa1611770
  • Oksenberg D, Dufu K, Patel MP, et al. GBT440 increases haemoglobin oxygen affinity, reduces sickling and prolongs RBC half-life in a murine model of sickle cell disease. Br J Haematol. 2016;175(1):141–153. doi:10.1111/bjh.14214
  • Vichinsky E, Hoppe CC, Ataga KI, et al. A phase 3 randomized trial of voxelotor in sickle cell disease. N Engl J Med. 2019;381(6):509–519. doi:10.1056/NEJMoa1903212
  • Johnson FL, Look AT, Gockerman J, Ruggiero MR, Dalla-Pozza L, Billings FT. Bone-marrow transplantation in a patient with sickle-cell anemia. N Engl J Med. 1984;311(12):780–783. doi:10.1056/NEJM198409203111207
  • Vermylen C, Fernandez Robles E, Ninane J, Cornu G. Bone marrow transplantation in five children with sickle cell anaemia. Lancet. 1988;331(8600):1427–1428. doi:10.1016/S0140-6736(88)92239-8
  • Ferster A, De Valck C, Azzi N, Fondu P, Toppet M, Sariban E. Bone marrow transplantation for severe sickle cell anaemia. Br J Haematol. 1992;80(1):102–105. doi:10.1111/j.1365-2141.1992.tb06407.x
  • Vermylen C, Cornu G. Bone marrow transplantation for sickle cell disease. The European experience. Am J Pediatr Hematol Oncol. 1994;16(1):18–21.
  • Johnson FL, Mentzer WC, Kalinyak KA, Sullivan KM, Abboud MR. Bone marrow transplantation for sickle cell disease. The United States experience. Am J Pediatr Hematol Oncol. 1994;16(1):22–26.
  • Walters MC, Patience M, Leisenring W, et al. Bone marrow transplantation for sickle cell disease. N Engl J Med. 1996;335(6):369–376. doi:10.1056/NEJM199608083350601
  • Walters MC, De Castro LM, Sullivan KM, et al. Indications and results of HLA-identical sibling hematopoietic cell transplantation for sickle cell disease. Biol Blood Marrow Transplant. 2016;22(2):207–211. doi:10.1016/j.bbmt.2015.10.017
  • Gluckman E, Cappelli B, Bernaudin F, et al. Sickle cell disease: an international survey of results of HLA-identical sibling hematopoietic stem cell transplantation. Blood. 2017;129(11):1548–1556. doi:10.1182/blood-2016-10-745711
  • Kato GJ, Piel FB, Reid CD, et al. Sickle cell disease. Nat Rev Dis Primers. 2018;4:18010.
  • Demirci S, Uchida N, Tisdale JF. Gene therapy for sickle cell disease: an update. Cytotherapy. 2018;20(7):899–910. doi:10.1016/j.jcyt.2018.04.003
  • Ribeil JA, Hacein-Bey-Abina S, Payen E, et al. Gene therapy in a patient with sickle cell disease. N Engl J Med. 2017;376(9):848–855. doi:10.1056/NEJMoa1609677
  • Magrin E, Semeraro M, Magnani A, et al. Results from the Completed Hgb-205 Trial of Lentiglobin for Β-Thalassemia and Lentiglobin for Sickle Cell Disease Gene Therapy. Washington, DC: American Society of Hematology; 2019.
  • Kanter J, Tisdale JF, Mapara MY, et al. Resolution of Sickle Cell Disease Manifestations in Patients Treated with Lentiglobin Gene Therapy: Updated Results from the Phase 1/2 Hgb-206 Group C Study. Washington, DC: American Society of Hematology; 2019.
  • Rai P, Ataga KI. Drug Therapies for the Management of Sickle Cell Disease. F1000Res. 2020; 9.