Sickle cell disease: Management options and challenges in developing countries

Mediterranean Journal of Hematology and Infectious Diseases

Volumn 5, Issue 1, 2013, Pages 1-6

  Ansong, Daniel ^a,b   Akoto, Alex Osei ^a,b   Ocloo, Delaena ^b   Ohene Frempong, Kwaku ^c  

^a KWAME NKRUMAH UNIVERSITY OF SCIENCE AND TECHNOLOGY   (Ghana)

^b KOMFO ANOKYE TEACHING HOSPITAL   (Ghana)

^c CHILDREN S HOSPITAL OF PHILADELPHIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ANTIBIOTIC AGENT; HYDROXYUREA; NONSTEROID ANTIINFLAMMATORY AGENT; OPIATE; OXYGEN; PARACETAMOL; PNEUMOCOCCUS VACCINE; ANALGESIC AGENT; CODEINE; IRON CHELATING AGENT; MORPHINE; NARCOTIC ANALGESIC AGENT; PENICILLIN DERIVATIVE; PETHIDINE;

ACUTE CHEST SYNDROME; ANEMIA; ARTICLE; BACTERIAL INFECTION; BLOOD CELL COUNT; BLOOD TRANSFUSION; BONE NECROSIS; CEREBROVASCULAR ACCIDENT; CONTINUING EDUCATION; DEVELOPING COUNTRY; DISEASE ASSOCIATION; DISEASE COURSE; DISEASE SEVERITY; DOPPLER ECHOGRAPHY; ERYTHROCYTE CONCENTRATE; ERYTHROCYTE TRANSFUSION; GHANA; HEALTH CARE PERSONNEL; HIP ARTHROPLASTY; HIP DISEASE; HIP NECROSIS; HUMAN; INFECTION; INFECTION PREVENTION; MEDICAL DECISION MAKING; MORBIDITY; NEWBORN SCREENING; NUCLEAR MAGNETIC RESONANCE IMAGING; PAIN; PATIENT CARE; PATIENT COMPLIANCE; PATIENT COUNSELING; PATIENT EDUCATION; PHYSIOTHERAPY; PNEUMOCOCCAL INFECTION; RESPIRATION DEPRESSION; SICKLE CELL ANEMIA; STOMACH EROSION; STOMACH ULCER; VACCINATION; VEIN OCCLUSION; ANTIBIOTIC PROPHYLAXIS; BLOOD VESSEL OCCLUSION; CAREGIVER; FEMUR HEAD NECROSIS; IRON OVERLOAD; MEDICAL EDUCATION; NEUROSURGERY; PATHOGENESIS; PHLEBOTOMY; PREVALENCE; UNSPECIFIED SIDE EFFECT;

EID: 84890523280     PISSN: None     EISSN: 20353006     Source Type: Journal    
DOI: 10.4084/MJHID.2013.062     Document Type: Article

References (47)

1. Weatherall DJ, Clegg JB. Inherited haemoglobin disorders: an increasing global health problem. Bull World Health Organ. 2001;79(8):704-12. PMid:11545326 PMCid:PMC2566499
2. Angastiniotis M, Modell B. Global Epidemiology of Hemoglobin Disorders. Annals of the New York Academy of Sciences. 1998 Jun;850(1 COOLEY'S ANEM):251-69.
3. Diallo D, Tchernia G. Sickle cell disease in Africa. Curr Opin Hematol. 2002 Mar;9(2):111-6. http://dx.doi.org/10.1097/00062752-200203000-00005 PMid:11844993
4. Bunn HF. Pathogenesis and treatment of sickle cell disease. N Engl J Med. 1997 Sep 11;337(11):762-9. http://dx.doi.org/10.1056/NEJM199709113371107 PMid:9287233
5. Marziali M, Isgrò A, Gaziev J, Lucarelli G. Hematopoietic stem cell transplantation in thalassemia and sickle cell disease. Unicenter experience in a multi-ethnic population. Mediterr J Hematol Infect Dis. 2009;1(1):e2009027. PMid:21415995 PMCid:PMC3033167
6. Walters MC. Stem cell therapy for sickle cell disease: transplantation and gene therapy. Hematology Am Soc Hematol Educ Program. 2005;66-73. http://dx.doi.org/10.1182/asheducation-2005.1.66 PMid:16304361
7. Shapiro BS, Dinges DF, Orne EC, Bauer N, Reilly LB, Whitehouse WG, et al. Home management of sickle cell-related pain in children and adolescents: natural history and impact on school attendance. Pain. 1995 Apr;61(1):139-44. http://dx.doi.org/10.1016/0304-3959(94)00164-A
8. Smith WR, Scherer M. Sickle-cell pain: advances in epidemiology and etiology. Hematology Am Soc Hematol Educ Program. 2010;2010:409-15. http://dx.doi.org/10.1182/asheducation-2010.1.409 PMid:21239827
9. Steinberg MH. Management of sickle cell disease. N Engl J Med. 1999 Apr 1;340(13):1021-30. http://dx.doi.org/10.1056/NEJM199904013401307 PMid:10099145
10. Ballas SK. Sickle cell disease: clinical management. Baillieres Clin Haematol. 1998 Mar;11(1):185-214. http://dx.doi.org/10.1016/S0950-3536(98)80075-9
11. 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. 1994 Jun 9;330(23):1639-44. http://dx.doi.org/10.1056/NEJM199406093302303 PMid:7993409
12. Ahmed SG. The role of infection in the pathogenesis of vaso-occlusive crisis in patients with sickle cell disease. Mediterr J Hematol Infect Dis. 2011;3(1):e2011028. http://dx.doi.org/10.4084/mjhid.2011.028 PMid:21869914 PMCid:PMC3152450
13. Robieux IC, Kellner JD, Coppes MJ, Shaw D, Brown E, Good C, et al. Analgesia in children with sickle cell crisis: comparison of intermittent opioids vs. continuous intravenous infusion of morphine and placebo-controlled study of oxygen inhalation. Pediatr Hematol Oncol. 1992 Dec;9(4):317-26. http://dx.doi.org/10.3109/08880019209016603 PMid:1467165
14. Stinson J, Naser B. Pain management in children with sickle cell disease. Paediatr Drugs. 2003;5(4):229-41. PMid:12662119
15. Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, 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 May 18;332(20):1317-22. http://dx.doi.org/10.1056/NEJM199505183322001 PMid:7715639
16. Koren A, Segal-Kupershmit D, Zalman L, Levin C, Abu Hana M, Palmor H, et al. Effect of hydroxyurea in sickle cell anemia: a clinical trial in children and teenagers with severe sickle cell anemia and sickle cell beta-thalassemia. Pediatr Hematol Oncol. 1999 Jun;16(3):221-32. http://dx.doi.org/10.1080/088800199277272 PMid:10326220
17. Thornburg CD, Files BA, Luo Z, Miller ST, Kalpatthi R, Iyer R, et al. Impact of hydroxyurea on clinical events in the BABY HUG trial. Blood. 2012 Nov 22;120(22):4304-4310; quiz 4448. http://dx.doi.org/10.1182/blood-2012-03-419879 PMid:22915643
18. Drayer RA, Henderson J, Reidenberg M. Barriers to better pain control in hospitalized patients. J Pain Symptom Manage. 1999 Jun;17(6):434-40. http://dx.doi.org/10.1016/S0885-3924(99)00022-6
19. Williams TN, Uyoga S, Macharia A, Ndila C, McAuley CF, Opi DH, et al. Bacteraemia in Kenyan children with sickle-cell anaemia: a retrospective cohort and case-control study. Lancet. 2009 Oct 17;374(9698):1364-70. http://dx.doi.org/10.1016/S0140-6736(09)61374-X
20. Komba AN, Makani J, Sadarangani M, Ajala-Agbo T, Berkley JA, Newton CRJC, et al. Malaria as a cause of morbidity and mortality in children with homozygous sickle cell disease on the coast of Kenya. Clin Infect Dis. 2009 Jul 15;49(2):216-22. http://dx.doi.org/10.1086/599834 PMid:19514855 PMCid:PMC2727464
21. Ramakrishnan M, Moïsi JC, Klugman KP, Iglesias JMF, Grant LR, Mpoudi-Etame M, et al. Increased risk of invasive bacterial infections in African people with sickle-cell disease: a systematic review and meta-analysis. Lancet Infect Dis. 2010 May;10(5):329-37 http://dx.doi.org/10.1016/S1473-3099(10)70055-4
22. Makani J, Komba AN, Cox SE, Oruo J, Mwamtemi K, Kitundu J, et al. Malaria in patients with sickle cell anemia: burden, risk factors, and outcome at the outpatient clinic and during hospitalization. Blood. 2010 Jan 14;115(2):215-20. http://dx.doi.org/10.1182/blood-2009-07-233528 PMid:19901265 PMCid:PMC2843825
23. Osei-Yeboah C, Rodrigues O, Enweronu-Laryea C. Nutritional status of children with sickle cell disease at korle bu teaching hospital, accra, ghana. West Afr J Med. 2011 Aug;30(4):262-7. PMid:22669830
24. Cober MP, Phelps SJ. Penicillin prophylaxis in children with sickle cell disease. J Pediatr Pharmacol Ther. 2010 Jul;15(3):152-9. PMid:22477807 PMCid:PMC3018247
25. Hirst C, Owusu-Ofori S. Prophylactic antibiotics for preventing pneumococcal infection in children with sickle cell disease. Cochrane Database Syst Rev. 2012;9:CD003427. PMid:22972063
26. Overturf GD. American Academy of Pediatrics. Committee on Infectious Diseases. Technical report: prevention of pneumococcal infections, including the use of pneumococcal conjugate and polysaccharide vaccines and antibiotic prophylaxis. Pediatrics. 2000 Aug;106(2 Pt 1):367-76. http://dx.doi.org/10.1542/peds.106.2.367 PMid:10920170
27. Serjeant GR. Sickle-cell disease. Lancet. 1997 Sep 6;350(9079):725-30. http://dx.doi.org/10.1016/S0140-6736(97)07330-3
28. Topley JM, Rogers DW, Stevens MC, Serjeant GR. Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease. Arch Dis Child. 1981 Oct;56(10):765-9. http://dx.doi.org/10.1136/adc.56.10.765 PMid:7305414 PMCid:PMC1627327
29. Fawibe AE. Managing acute chest syndrome of sickle cell disease in an African setting. Trans R Soc Trop Med Hyg. 2008 Jun;102(6):526-31. http://dx.doi.org/10.1016/j.trstmh.2008.03.018 PMid:18455745
30. Scothorn DJ, Price C, Schwartz D, Terrill C, Buchanan GR, Shurney W, et al. Risk of recurrent stroke in children with sickle cell disease receiving blood transfusion therapy for at least five years after initial stroke. J Pediatr. 2002 Mar;140(3):348-54. http://dx.doi.org/10.1067/mpd.2002.122498 PMid:11953734
31. Mazumdar M, Heeney MM, Sox CM, Lieu TA. Preventing stroke among children with sickle cell anemia: an analysis of strategies that involve transcranial Doppler testing and chronic transfusion. Pediatrics. 2007 Oct;120(4):e1107-1116. http://dx.doi.org/10.1542/peds.2006-2002 PMid:17768181
32. Ware RE, Helms RW, SWiTCH Investigators. Stroke With Transfusions Changing to Hydroxyurea (SWiTCH). Blood. 2012 Apr 26;119(17):3925-32. http://dx.doi.org/10.1182/blood-2011-11-392340 PMid:22318199 PMCid:PMC3350359
33. Zimmerman SA, Schultz WH, Burgett S, Mortier NA, Ware RE. Hydroxyurea therapy lowers transcranial Doppler flow velocities in children with sickle cell anemia. Blood. 2007 Aug 1;110(3):1043-7. http://dx.doi.org/10.1182/blood-2006-11-057893 PMid:17429008
34. Ohene-Frempong K. Stroke in sickle cell disease: demographic, clinical, and therapeutic considerations. Semin Hematol. 1991 Jul;28(3):213-9. PMid:1887247
35. Pegelow CH, Macklin EA, Moser FG, Wang WC, Bello JA, Miller ST, et al. Longitudinal changes in brain magnetic resonance imaging findings in children with sickle cell disease. Blood. 2002 Apr 15;99(8):3014-8. http://dx.doi.org/10.1182/blood.V99.8.3014 PMid:11929794
36. Hernigou P, Galacteros F, Bachir D, Goutallier D. Deformities of the hip in adults who have sickle-cell disease and had avascular necrosis in childhood. A natural history of fifty-two patients. J Bone Joint Surg Am. 1991 Jan;73(1):81-92. PMid:1985998
37. Hernigou P, Bachir D, Galacteros F. Avascular necrosis of the femoral head in sickle-cell disease. Treatment of collapse by the injection of acrylic cement. J Bone Joint Surg Br. 1993 Nov;75(6):875-80. PMid:8245075
38. Ware HE, Brooks AP, Toye R, Berney SI. Sickle cell disease and silent avascular necrosis of the hip. J Bone Joint Surg Br. 1991 Nov;73(6):947-9. PMid:1955442
39. Clarke HJ, Jinnah RH, Brooker AF, Michaelson JD. Total replacement of the hip for avascular necrosis in sickle cell disease. J Bone Joint Surg Br. 1989 May;71(3):465-70. PMid:2722941
40. Adekile AD, Gupta R, Yacoub F, Sinan T, Al-Bloushi M, Haider MZ. Avascular necrosis of the hip in children with sickle cell disease and high Hb F: magnetic resonance imaging findings and influence of alpha-thalassemia trait. Acta Haematol. 2001;105(1):27-31. http://dx.doi.org/10.1159/000046529 PMid:11340250
41. Fattoum S. Evolution of hemoglobinopathy prevention in Africa: results, problems and prospect. Mediterr J Hematol Infect Dis. 2009;1(1):e2009005.
42. Akodu S, Diaku-Akinwumi I, Njokanma O. Age at diagnosis of sickle cell anaemia in lagos, Nigeria. Mediterr J Hematol Infect Dis. 2013;5(1):e2013001.
43. Adamkiewicz TV, Silk BJ, Howgate J, Baughman W, Strayhorn G, Sullivan K, et al. Effectiveness of the 7-valent pneumococcal conjugate vaccine in children with sickle cell disease in the first decade of life. Pediatrics. 2008 Mar;121(3):562-9. http://dx.doi.org/10.1542/peds.2007-0018 PMid:18310206
44. Pneumococcal vaccines [Internet]. 11:31:41 [cited 2013 Sep 20]. Available from: http://www.who.int/vaccines/en/pneumococcus.shtml
45. Ministry of Health - Introduction of new vaccines [Internet]. 2013 [cited 2013 Sep 20]. Available from: http://www.moh-ghana.org/otherpages.aspx?id=3
46. MicrosoftWord-2013.02.13_PCVSupplyStatus_UNICEF_SD.v23.docx - PCV_Supply_Status_2013February_UNICEF_SD_Final.pdf [Internet]. [cited 2013 Oct 15]. Available from: http://www.unicef.org/supply/files/PCV_Supply_Status_2013February_UNICEF_SD_Final.pdf
47. Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. JAMA. 2003 Apr 2;289(13):1645-51. http://dx.doi.org/10.1001/jama.289.13.1645 PMid:12672732