Sickle cell disease: The need for a public health agenda

American Journal of Preventive Medicine

Volumn 41, Issue 6 SUPPL.4, 2011, Pages

  Yusuf, Hussain R ^a   Lloyd Puryear, Michele A ^b   Grant, Althea M ^a   Parker, Christopher S ^a   Creary, Melissa S ^a   Atrash, Hani K ^a  

^a NATIONAL CENTER ON BIRTH DEFECTS AND DEVELOPMENTAL DISABILITIES   (United States)

^b Maternal and Child Health Bureau   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

HAEMOPHILUS INFLUENZAE TYPE B VACCINE; HEMOGLOBIN S; PENICILLIN G; PNEUMOCOCCUS VACCINE;

ARTICLE; AWARENESS; COMPLICATION; DISABILITY; HAEMOPHILUS INFLUENZAE; HEALTH CARE ACCESS; HEALTH CARE UTILIZATION; HEALTH SERVICE; HUMAN; INFECTION PREVENTION; INFLUENZA; KNOWLEDGE; MORTALITY; NEWBORN SCREENING; OUTCOME ASSESSMENT; PROPHYLAXIS; PUBLIC HEALTH; SICKLE CELL ANEMIA; SICKLE CELL TRAIT; STREPTOCOCCUS PNEUMONIA; STREPTOCOCCUS PNEUMONIAE;

AFRICAN AMERICANS; ANEMIA, SICKLE CELL; HEALTH KNOWLEDGE, ATTITUDES, PRACTICE; HEALTH PROMOTION; HEALTH SERVICES; HUMANS; NEEDS ASSESSMENT; PUBLIC HEALTH PRACTICE; UNITED STATES;

EID: 81355150282     PISSN: 07493797     EISSN: 18732607     Source Type: Journal    
DOI: 10.1016/j.amepre.2011.09.007     Document Type: Article

References (76)

1. B. Modell, and M. Darlison Global epidemiology of haemoglobin disorders and derived service indicators Bull World Health Organ 86 2008 480 487 (Pubitemid 351821761)
2. D.J. Weatherall, and J.B. Clegg Inherited haemoglobin disorders: an increasing global health problem Bull World Health Organ 79 2001 704 712 (Pubitemid 32776838)
3. Sickle Cell Disease Guideline Panel Sickle cell disease: screening, diagnosis, management, and counseling in newborns and infants April 1993 Agency for Health Care Policy and Research, Public Health Service, USDHHS Rockville MD Clinical Practice Guideline No. 6. AHCPR Pub. No. 93 0562
4. H.F. Bunn Pathogenesis and treatment of sickle cell disease N Eng J Med 337 1997 762 769 (Pubitemid 27417977)
5. P.A. Lane Sickle cell disease Pediatr Clin N Am 43 1996 639 664 (Pubitemid 26181472)
6. G. Tsaras, A. Owusu-Ansah, F.O. Boateng, and Y. Amoateng-Adjepong Complications associated with sickle cell trait: a brief narrative review Am J Med 122 2009 507 512
7. B.L. Mitchell Sickle cell trait and sudden deathbringing it home J Natl Med Assoc 99 2007 300 305 (Pubitemid 46399389)
8. J.N. Makaryus, J.N. Catanzaro, and K.C. Katona Exertional rhabdomyolysis and renal failure in patients with sickle cell trait: is it time to change our approach? Hematology 12 2007 349 352 (Pubitemid 47180664)
9. CDC National Public Health Performance Standards Program: 10 essential public health services www.cdc.gov/od/ocphp/nphpsp/essentialphservices.htm
10. R.S. Olney Newborn screening for sickle cell disease: public health impact and evaluation M.J. Khoury, W. Burke, E.J. Thomson, Genetics and public health in the 21st century: using public genetic information to improve health and prevent disease 2000 Oxford University Press New York NY http://www.cdc.gov/genomics/resources/books/21stcent/index.htm
11. N.B. Lerner, B.L. Platania, and S. LaBella Newborn sickle cell screening in a region of Western New York State J Pediatr 154 2009 121 125
12. M.S. Harris, and J.R. Eckman Georgia's experience with newborn screening: 1981 to 1985 Pediatrics 83 5 Pt 2 1989 858 860 (Pubitemid 19125974)
13. B.L. Therrell, J.L. Simmank, and M. Willborn Experiences with sickle hemoglobin testing in the Texas Newborn Screening Program Pediatrics 83 1989 864 867 (Pubitemid 19125976)
14. M.A. Lloyd-Puryear, B.L. Therrell, M.Y. Mann, J.R. Eckman, and J. Telfair The role of the federal government in supporting state newborn screening programs M.A. Baily, T.H. Murray, Ethics and newborn genetic testing - new technologies, new challenges 2009 Johns Hopkins Press Baltimore MD
15. K.A. Pass, P.A. Lane, and P.M. Fernhoff U.S. newborn screening system guidelines II: follow-up of children, diagnosis, management, and evaluation Statement of the Council of Regional Networks for Genetic Services J Pediatr 137 4S 2000 S1 S46
16. NIH The management of sickle cell disease 4th ed. 2002 NIH Bethesda MD National Heart, Lung, and Blood Institute, Division of Blood Diseases and Resources, NIH Publication No. 02-2117
17. P. Kavanagh, C.J. Wang, B.L. Therrell, P.G. Sprinz, and H. Bauchner Communication of positive newborn screening results for sickle cell disease and sickle cell trait: variation across states Am J Med Genet C Semin Med Genet 148C 2008 15 22 (Pubitemid 351294848)
18. B.L. Therrell, A. Johnson, and D. Williams Status of newborn screening programs in the United States Pediatrics 117 5 Pt 2 2006 S212 S252
19. T. Frempong, and H.A. Pearson Newborn screening coupled with comprehensive follow-up reduced early mortality of sickle cell disease in Connecticut Conn Med 71 2007 9 12 (Pubitemid 46190764)
20. L. King, R. Fraser, M. Forbes, M. Grindley, S. Ali, and M. Reid Newborn sickle cell disease screening: the Jamaican experience (19952006) J Med Screen 14 2007 117 122 (Pubitemid 47511120)
21. J. Bardakdjian-Michau, M. Guilloud-Bataille, and M. Maier-Redelsperger Decreased morbidity in homozygous sickle cell disease detected at birth Hemoglobin 26 2002 211 217 (Pubitemid 35191300)
22. M.C. Rahimy, A. Gangbo, G. Ahouignan, and E. Alihonou Newborn screening for sickle cell disease in the Republic of Benin J Clin Pathol 62; 2009 46 48
23. D.C. Brousseau, J.A. Panepinto, M. Nimmer, and R.G. Hoffmann The number of people with sickle-cell disease in the United States: national and state estimates Am J Hematol 85 1 2010 77 78
24. K.L. Hassel Population estimates of sickle cell disease in the U.S. Am J Prev Med 38 4S 2010 S512 S521
25. F.W. Lorey, J. Arnopp, and G.C. Cunningham Distribution of hemoglobinopathy variants by ethnicity in a multiethnic state Genet Epidemiol 13 1996 501 512 (Pubitemid 26359326)
26. B. Therrell, F. Lorey, and R. Eaton Impact of expanded newborn screeningUnited States, 2006 MMWR 57 2008 1012 1015
27. F.M. Gill, L.A. Sleeper, and S.J. Weiner Clinical events in the first decade in a cohort of infants with sickle cell disease Cooperative study of sickle cell disease Blood 86 1995 776 783
28. G.D. Overturf Prevention of invasive pneumococcal infection in sickle cell disease: on the threshold of a new era of successes? J Pediatr 143 2003 423 425 (Pubitemid 37352494)
29. S.R. Leikin, D. Gallagher, and T.R. Kinney Mortality in children and adolescents with sickle cell disease Pediatrics 84 1989 500 508 (Pubitemid 19216350)
30. W.-Y. Wong, G.D. Overturf, and D.R. Powars Infection caused by Streptococcus pneumoniae in children with sickle cell disease: epidemiology, immunologic mechanism, prophylaxis, and vaccination Clin Infect Dis 14 5 1992 1124 1136
31. D. Powars, G. Overturf, J. Weiss, S. Lee, and L. Chan Pneumococcal septicemia in children with sickle cell anemia JAMA 245 1981 1839 1842 (Pubitemid 11098795)
32. M.H. Gaston, J.I. Verter, and G. Woods Prophylaxis with oral penicillin in children with sickle cell anemia N Engl J Med 314 1986 1593 1599 (Pubitemid 16118215)
33. H.S. Zarkowski, D. Gallagher, and F.M. Gill Bacteremia in sickle hemoglobinopathies J Pediatr 109 4 1986 579 585 (Pubitemid 16028601)
34. C.H. Pegelow, F.D. Armstrong, S. Light, S.R. Toledano, and J. Davis Experience with the use of prophylactic penicillin in children with sickle cell anemia J Pediatr 118 1991 736 738
35. T.V. Adamkiewicz, B.J. Silk, and J. Howgate Effectiveness of the 7-valent pneumococcal conjugate vaccine in children with sickle cell disease in the first decade of life Pediatrics 121 2008 562 569
36. E. Yanni, S.D. Grosse, Q. Yang, and R.S. Olney Trends in pediatric sickle cell disease-related mortality in the United States, 19832002 J Pediatr 154 2008 541 545
37. G.R. Buchanan, J.D. Siegel, S.J. Smith, and B.M. DePasse Oral penicillin prophylaxis in children with impaired splenic function: a study of compliance Pediatrics 70 6 1982 926 930 (Pubitemid 13247245)
38. G.R. Buchanan, and S.J. Smith Pneumococcal septicemia despite pneumococcal vaccine and prescription of penicillin prophylaxis in children with sickle cell anemia Am J Dis Child 140 1986 428 432 (Pubitemid 16114734)
39. T.V. Adamkiewicz, S. Sarnaik, and G.R. Buchanan Invasive pneumococcal infections in children with sickle cell disease in the era of penicillin prophylaxis, antibiotic resistance, and 23-valent pneumococcal polysaccharide vaccination J Pediatr 143 4 2003 438 444 Erratum in: J Pediatr 2004;144(3):412 (Pubitemid 37352499)
40. V. Elliot, S. Morgan, S. Day, L.S. Mollerup, and W. Wang Parental health beliefs and compliance with prophylactic penicillin administration in children with sickle cell disease J Pediatr Hematol Oncol 23 2001 112 116 (Pubitemid 32162673)
41. J. Grimshaw, M. Eccles, and R. Thomas Toward evidence-based quality improvement Evidence (and its limitations) of the effectiveness of guideline dissemination and implementation strategies 19661998 J Gen Intern Med 21 S2 2006 S14 S20
42. C. Haywood, M.C. Beach, and S. Lanzkron A systematic review of barriers and interventions to improve appropriate use of therapies for sickle cell disease J Natl Med Assoc 101 2009 1022 1033
43. K. Simon, M.L. Lobo, and S. Jackson Current knowledge in the management of children and adolescents with sickle cell disease: part 1, physiological issues J Pediatr Nurs 14 1999 281 295
44. J.C. Wood Cardiac iron across different transfusion-dependent diseases Blood Rev 22 S2 2008 S14 S21
45. J.B. Porter Concepts and goals in the management of transfusional iron overload Am J Hematol 82 2007 1136 1139 (Pubitemid 350221244)
46. O. Alvarez, H. Rodriquez-Cortes, and N. Robinson Adherence to deferasirox in children and adolescents with sickle cell disease during 1-year of therapy J Pediatr Hematol Oncol 31 10 2009 739 744
47. C.A. Steiner, and J.L. Miller Sickle cell disease patients in U.S. hospitals, 2004 2006 Dec Agency for Healthcare Research and Quality Rockville MD HCUP Statistical Brief #21 www.hcup-us.ahrq.gov/reports/statbriefs/sb21.pdf
48. C.D. Fitzhugh, N. Lauder, and J.C. Jonassaint Cardiopulmonary complications leading to premature deaths in adult patients with sickle cell disease Am. J. Hematol 85 2010 36 40
49. M.B. Fosdal, and A.W. Wojner-Alexandrov Events of hospitalization among children with sickle cell disease J Pediatr Nurs 22 2007 342 346
50. H.R. Yusuf, H.K. Atrash, S.D. Grosse, C.S. Parker, and A.M. Grant Emergency department visits made by patients with sickle cell disease: a descriptive study Am J Prev Med 38 4S 2010 S536 S541
51. S. Lanzkron, C.P. Carroll, and C. Haywood Jr The burden of emergency department use for sickle-cell disease: an analysis of the national emergency department sample database Am J Hematol 85 2010 797 799
52. C. Haywood Jr. , M.C. Beach, and S. Lanzkron A systematic review of barriers and interventions to improve appropriate use of therapies for sickle cell disease J Natl Med Assoc 101 2009 1022 1033
53. K. Hassell, B. Pace, and W. Wang Sickle cell disease summit: from clinical and research disparity to action Am J Hematol 84 2009 39 45
54. S.K. Ballas, R.L. Bauserman, and W.F. McCarthy Utilization of analgesics in the multicenter study of hydroxyurea in sickle cell anemia: effect of sex, age, and geographical location Am J Hematol 85 2010 611 613
55. J.H. Boyd, E.A. Macklin, R.C. Strunk, and M.R. DeBaun Asthma is associated with increased mortality in individuals with sickle cell anemia Haematologica 92 2007 1115 1118 (Pubitemid 350144219)
56. J.J. Field, and M.R. DeBaun Asthma and sickle cell disease: two distinct diseases or part of the same process? Hematology Am Soc Hematol Educ Program 2009 45 53
57. A.D. Slonim, J.G. Joseph, W.M. Turenne, A. Sharangpani, and N.L. Luban Blood transfusions in children: a multi-institutional analysis of practices and complications Transfusion 48 2008 73 80
58. H.J. Fullerton, R.J. Adams, S. Zhao, and S.C. Johnston Declining stroke rates in Californian children with sickle cell disease Blood 104 2 2004 336 339 (Pubitemid 38900012)
59. D.L. Wethers Sickle cell disease in childhood: part I laboratory diagnosis, pathophysiology, and health maintenance Am Fam Physician 62 2000 1013 1020
60. M. Creary, D. Williamson, and R. Kulkarni Sickle cell disease: current activities, public health implications, and future directions J Womens Health 16 2007 575 582 (Pubitemid 47093903)
61. M.H. Farrell, L.K. Certain, and P.M. Farrell Genetic counseling and risk communication services of newborn screening programs Arch Pediatr Adolesc Med 155 2001 120 126 (Pubitemid 32128379)
62. G. Mahat, M.A. Scoloveno, and C.B. Donnelly Written educational materials for families of chronically ill children J Am Acad Nurse Pract 19 2007 471 476
63. S.D. Grosse, M.S. Schechter, R. Kulkarni, M.A. Lloyd-Puryear, B. Strickland, and E. Trevathan Models of comprehensive multidisciplinary care for individuals in the United States with genetic disorders Pediatrics 123 2009 407 412
64. I.P. Aisiku, L.T. Penberthy, and W.R. Smith Patient satisfaction in specialized vs. non-specialized adult sickle cell care centers J Natl Med Assoc 99 2007 886 890 (Pubitemid 47255131)
65. American Academy of Pediatrics, Section on Hematology/Oncology, Committee on Genetics Health supervision for children with sickle cell disease Pediatrics 109 2002 526 535
66. J.L. Raphael, A. Kamdar, and T. Wang Day hospital versus inpatient management of uncomplicated vaso-occlusive crises in children with sickle cell disease Pediatr Blood Cancer 51 2008 398 401
67. L.J. Benjamin, G.I. Swinson, and R.L. Nagel Sickle cell anemia day hospital: an approach for the management of uncomplicated painful crises Blood 95 2000 1130 1136
68. M. Mayer, T.R. Konrad, and C.C. Dvorak Hospital resource utilization among patients with sickle cell disease J Health Care Poor Underserved 14 2003 122 135 (Pubitemid 38543354)
69. K. Woods, T. Karrison, M. Koshy, A. Patel, P. Friedmann, and C. Cassel Hospital utilization patterns and costs for adult sickle cell patients in Illinois Public Health Rep 112 1 1997 44 51 (Pubitemid 27053114)
70. N.E. Kass, A.M. Medley, and M.R. Natowicz Access to health insurance: experiences and attitudes of those with genetic versus non-genetic medical conditions Am J Med Genet 143 2007 707 717
71. M.A. Hall, J.E. McEwan, and J.C. Barton Concerns in a primary care population about genetic discrimination by insurers Genet Med 7 2005 311 316 (Pubitemid 40827579)
72. M.T. Roth, and R.B. Painter Genetic discrimination in health insurance: an overview and analysis of the issues Nurs Clin North Am 35 2000 731 756
73. J.L. Raphael, C.L. Dietrich, D. Whitmire, D.H. Mahoney, B.U. Meuller, and A.P. Giardino Health care utilization and expenditures for low income children with sickle cell disease Pediatr Blood Cancer 52 2009 263 267
74. K. Kuhlthau, T.G. Ferris, A.C. Beal, S.L. Gortmaker, and J.M. Perrin Who cares for Medicaid-enrolled children with chronic conditions? Pediatrics 108 2001 906 912 (Pubitemid 32947660)
75. S.M. Shankar, P.G. Arbogast, E. Mitchel, W.O. Cooper, W.C. Wang, and M.R. Griffin Medical care utilization and mortality in sickle cell disease: a population-based study Am J Hematol 80 2005 262 270 (Pubitemid 41746173)
76. A.M. Brandow, D.L. Jirovec, and J.A. Panepinto Hydroxyurea in children with sickle cell disease: practice patterns and barriers to utilization Am J Hematol 85 2010 611 613