A primary care provider's guide to preventive and acute care management of adults and children with sickle cell disease

Journal of the American Academy of Nurse Practitioners

Volumn 21, Issue 5, 2009, Pages 250-257

  Pack Mabien, Ardie ^b   Haynes Jr , Johnson ^a,b  

^a Department of Medicine ^*

^b UNIVERSITY OF SOUTH ALABAMA   (United States)

Author keywords

Cerebrovascular disease; Pulmonary hypertension; Renal insufficiency; Splenic sequestration; Vaso occlusive pain episode

Indexed keywords

ACUTE DISEASE; ADULT; APLASTIC ANEMIA; ARTICLE; CHILD; CHOLELITHIASIS; DYSPNEA; FEMALE; FEVER; GENETICS; HOSPITALIZATION; HUMAN; INFECTION; MALE; METHODOLOGY; NURSE ATTITUDE; NURSE PRACTITIONER; ORGANIZATION AND MANAGEMENT; PAIN; PRACTICE GUIDELINE; PRIAPISM; PRIMARY HEALTH CARE; PRIMARY PREVENTION; PULMONARY HYPERTENSION; SICKLE CELL ANEMIA; STROKE;

ACUTE DISEASE; ADULT; ANEMIA, APLASTIC; ANEMIA, SICKLE CELL; CHILD; CHOLELITHIASIS; DYSPNEA; FEMALE; FEVER; HUMANS; HYPERTENSION, PULMONARY; INFECTION; MALE; NURSE PRACTITIONERS; NURSE'S ROLE; PAIN; PRACTICE GUIDELINES AS TOPIC; PRIAPISM; PRIMARY HEALTH CARE; PRIMARY PREVENTION; SEVERITY OF ILLNESS INDEX; STROKE;

EID: 65449190094     PISSN: 10412972     EISSN: 17457599     Source Type: Journal    
DOI: 10.1111/j.1745-7599.2009.00401.x     Document Type: Article

References (57)

1. Adams, R 2000). Lessons from the stroke prevention trial in sickle cell anemia (STOP) study. Journal of Child Neurology, 15 (5 344 349. (Pubitemid 30341366)
2. Adeyoju, A., Olujohungbe, A., Morris, J., Yardumian, A., Bareford, D., Akenova, A., et al 2002). Priapism in sickle cell disease: Incidence, risk factors and complications. An international multicenter study. BJU International, 90 (9 898 902. (Pubitemid 36693626)
3. Ambrusko, S., Gunawardena, S., Sakara, A., Windsor, B., Lanford, L., Michelson, P., et al 2006). Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell disease. Pediatric Blood & Cancer, 47 (7 907 913. (Pubitemid 44656113)
4. Ataga, K., Sood, N., De Gent, G., Kelly, E., Henderson, A., Jones, S., et al 2004). Pulmonary hypertension in sickle cell disease. American Journal of Medicine, 117 (9 665 669. (Pubitemid 40288173)
5. Ataga, K., Moore, C., Jones, S., Olajide, O., Strayhorn, D., Hinderliter, A., et al 2006). Pulmonary hypertension in patients with sickle cell disease: A longitudinal study. British Journal of Haematology, 134 (1 109 115.
6. Bonds, D 2005). Three decades of innovation in the management of sickle cell disease: The road to understanding the sickle cell disease clinical phenotype. Blood Reviews, 19, 99 110. (Pubitemid 39643115)
7. Castagnola, E. Fioredda, F 2003). Prevention of life-threatening infections due to encapsulated bacteria in children with hyposplenia or asplenia: A brief review of current recommendations for practical purposes. European Journal of Haematology, 71, 319 326. (Pubitemid 37330503)
8. Chulamokha, L., Scholand, S., Riggio, J., Ballas, S., Horn, D. DeSimone, J 2006). Bloodstream infections in hospitalized adults with sickle cell disease: a retrospective analysis. American Journal of Hematology, 81 (10 723 728. (Pubitemid 44536740)
9. Davis, C., Mostofi, F. Sesterhenn, I 1995). Renal medullary carcinoma. The seventh sickle cell nephropathy. American Journal of Surgical Pathology, 19 (1 1 11.
10. Eichner, E 1986). Sickle cell trait, exercise, and altitude. The Physician and Sportsmedicine, 14 (11 144 157. (Pubitemid 17185332)
11. Franklin, Q. Compeggie, M 1999). Splenic syndrome in sickle cell trait: Four case presentations and a review of the literature. Military Medicine, 164, 230 233. (Pubitemid 29133722)
12. Gladwin, M., Sachdev, V., Jison, M., Shizukuda, Y., Plehn, J., Minter, K., et al 2004). Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. New England Journal of Medicine, 350 (9 886 895. (Pubitemid 38252536)
13. Gladwin, M. Kato, G 2005). Cardiopulmonary complications of sickle cell disease: Role of nitric oxide and hemolytic anemia. Hematology (American Society of Hematology Education Program 51 57.
14. Guasch, A., Navarrete, J., Nass, K. Zayas, C 2006). Glomerular involvement in adults with sickle cell hemoglobinopathies: Prevalence and clinical correlates of progressive renal failure. Journal of the American Society of Nephrology, 17, 2228 2235. (Pubitemid 44141913)
15. Hoffman, M. Haynes, J 2005). Acute chest syndrome: Getting down to the basics. Journal of Respiratory Diseases, 26 (12 529 534.
16. Josephson, C., Su, L., Hillyer, K. Hillyer, C 2007). Transfusion in the patient with sickle cell disease: A critical review of the literature and transfusion guidelines. Transfusion Medicine Reviews, 21 (2 118 133. (Pubitemid 46482993)
17. Keenan, R., Boswell, T. Milligan, W 1999). Do post-splenectomy patients take prophylactic penicillin? British Journal of Haematology, 105, 509 510. (Pubitemid 29232179)
18. Koduri, P. Kovarik. P 2006). Acute splenic sequestration crisis in an adult with sickle β-thalassemia. Annals of Hematology, 85 (9 633 635. (Pubitemid 44283089)
19. Labbe, E., Herbert, D. Haynes, J 2005). Physicians' attitudes and practices in sickle cell pain management. Journal of Palliative Care, 4 (21 246 251.
20. Lottenberg, R. Hassell, K 2005). An evidence-based approach to the treatment of adults with sickle cell disease. Hematology (American Society of Hematology Education Program Book).
21. Machado, R., Anthi, A., Steinberg, M., Bonds, D., Sachdev, V., Kato, G., et al 2006). N-terminal pro-brain natriuretic peptide levels and risk of death in sickle cell disease. Journal of the American Medical Association, 296 (3 310 318. (Pubitemid 44079315)
22. Manci, E., Culberson, D., Yang, Y., Gardner, T., Powell, R., Haynes, J., et al 2003). Causes of death in sickle cell disease: An autopsy study. British Journal of Haematology, 123, 359 365. (Pubitemid 37305074)
23. Mantadakis, E., Ewalt, D., Cavender, J., Rogers, Z. Buchanan, G 2000). Outpatient penile aspiration and epinephrine irrigation for young patients with sickle cell anemia and prolonged priapism. Blood, 95 (1 78 82. (Pubitemid 30017228)
24. Martin, J. Moore, G 1997). Pearls, pitfalls, and updates for pain management. Emergency Medicine Clinics of North America, 15 (2 399 415. (Pubitemid 27240605)
25. Mehta, S., Afenyi-Annan, A., Byrns, P. Lottenberg, R 2006). Opportunities to improve outcomes in sickle cell disease. American Family Physician, 74 (2 303 310, 313 314. (Pubitemid 44099188)
26. Melton, C. Haynes, J 2006). Sickle acute lung injury: A role of prevention and early aggressive intervention strategies on outcome. Clinics in Chest Medicine, 27, 487 502. (Pubitemid 44118529)
27. Moll, S. Orringer, E 1996). Splenomegaly and splenic sequestration in an adult with sickle cell anemia. American Journal of the Medical Sciences, 312 (6 299 302. (Pubitemid 26422616)
28. National Institutes of Health 1992). The management of sickle cell disease (NIH Publication No. 02-2117). National Heart, Lung, and Blood Institute, Division of Blood Diseases and Resources. Washington, DC : U.S. Department of Health and Human Services.
29. National Institutes of Health 2002). The management of sickle cell disease (NIH Publication No. 92-2117). National Heart, Lung, and Blood Institute, Division of Blood Diseases and Resources. Washington, DC : U.S. Department of Health and Human Services.
30. National Newborn Screening and Genetics Resource Center 2006, August 15). National newborn screening status report update. Austin, TX : Author.
31. National Institutes of Health 2004, December 5). NIH Clinical Alert to US Physicians: U.S. Department of Health and Human Services NIH News. NHLBI stops study testing how long children with sickle cell anemia should have blood transfusions to prevent stroke. Bethesda, MD : Author.
32. Norris, C., Smith-Whitley, K. McGowan, K 2003). Positive blood cultures in sickle cell disease: time to positivity and clinical outcome. Journal of Pediatric Hematology/Oncology, 25 (5) 390 395. (Pubitemid 36569971)
33. Ohene-Frempong, K., Weiner, S., Sleeper, L., Miller, S., Embury, S., Moohr, J., et al 1998). Cerebrovascular accidents in sickle cell disease: Rates and risk factors. Blood, 91 (1 288 294. (Pubitemid 28018756)
34. Pack-Mabien, A., Labbe, E., Herbert, D. Haynes, J 2001). Nurses' attitudes and practices in sickle cell pain management. Applied Nursing Research, 4 (14 187 192. (Pubitemid 33601944)
35. Platt, O., Thorington, B., Brambilla, D., Milner, P., Rosse, W., Vichinsky, E., et al 1991). Pain in sickle cell disease: Rates and risk factors. New England Journal of Medicine, 325 (1 11 16.
36. Platt, O., Brambilla, D., Rosse, W., Milner, P., Castro, O., Steinberg, M., et al 1994). Mortality in sickle cell disease: Life expectancy and risk factors for early death. New England Journal of Medicine, 330 (23 1639 1644.
37. Price, V., Dutta, S., Blanchette, V., Butchart, S., Kirby, M., Langer, J., et al 2006). The prevention and treatment of bacterial infections in children with asplenia or hyposplenia: Practice considerations at the hospital for sick children, Toronto. Pediatric Blood and Cancer, 46, 597 603.
38. Roshkow, J. Sanders, L 1990). Acute splenic sequestration crisis in two adults with sickle cell disease: US, CT, and MR imaging findings. Radiology, 177 (3 723 725.
39. Rosse, W., Narla, M., Petz, L. Martin, S 2000). New views of sickle cell disease pathophysiology and treatment. Hematology (American Society of Hematology Education Program 2 17.
40. Saad, S., Lajolo, C., Gilli, S., Marques, J., Lima, C., Costa, F., et al 2004). Follow-up of sickle cell disease patients with priapism treated by hydroxyurea. American Journal of Hematology, 77, 45 49. (Pubitemid 39108796)
41. Serjeant, G 1994). The geography of sickle cell disease: Opportunities for understanding its diversity. Annals of Saudi Medicine, 14 (3 237 246. (Pubitemid 24227952)
42. Serjeant, G 1997). Sickle-cell disease. Lancet, 350 (9079 725 730. (Pubitemid 127739268)
43. Servey, J., Reamy, B. Hodge, J 2007). Clinical presentations of parvovirus B19 infection. American Family Physician, 75 (3 373 376, 377. (Pubitemid 46232574)
44. Steinberg, M 1999). Management of sickle cell disease. Drug Therapy, c340 (13 1021 1030. (Pubitemid 29157635)
45. Sumaraju, V., Leon, G. Smith, S 2001). Infectious complication in asplenic hosts. Infectious Disease Clinics of North America, 15 (2 551 565.
46. Sumoza, A., deBisotti, R., Sumoza, D. Fairbanks, V 2002). Hydroxyurea for prevention of recurrent stroke in sickle cell anemia. American Journal of Hematology, 71, 161 165. (Pubitemid 35253329)
47. Taylor, C., Carter, F., Poulose, J., Rolle, S. Babum, S. Crichlow, S 2004). Clinical presentation of acute chest syndrome in sickle cell disease. Postgraduate Medical Journal, 80, 346 349. (Pubitemid 38833417)
48. Walker, T., Hambleton, I. Serjeant, G 2000). Gallstones in sickle cell disease: Observations from the Jamaican cohort study. Journal of Pediatrics, 136 (1 80 85. (Pubitemid 30468562)
49. Warren, K., Gidvani-Diaz, V. Duval-Arnould, B 1999). Renal medullary carcinoma in an adolescent with sickle cell trait. Pediatrics, 103 (2 1 3.
50. Wethers, D 2000). Sickle cell disease in childhood. Part I. Laboratory diagnosis, pathophysiology, and health maintenance. American Family Physician, 62 (5 1013 1020, 1027 1028.
51. Williams, T., Honeywell, M., Branch, E., Glenn, Z. Close, F 2004). Hydroxyurea in the treatment of sickle cell anemia. Drug Forecast, 29 (1 25 29. (Pubitemid 38113935)
52. Wright, J., Thomas, P. Serjeant, G 1997). Septicemia caused by salmonella infection: An overlooked complication of sickle cell disease. Journal of Pediatrics, 130 (3 394 399. (Pubitemid 27492683)
53. Vermylen, C 2003). Hematopoietic stem cell transplantation in sickle cell disease. Blood Review, 17, 163 166. (Pubitemid 36876676)
54. Vichinsky, E., Styles, L., Colangelo, L., Wright, E., Castro, O., Nickerson, B., et al 1997). Acute chest syndrome in sickle cell disease: Clinical presentation and course. Blood, 89 (5 1787 1792. (Pubitemid 27097475)
55. Vichinsky, E., Neumayr, L., Earles, A., Williams, R., Lennette, E., Dean, D., et al 2000). Causes and outcomes of the acute chest syndrome in sickle cell disease. New England Journal of Medicine, 342 (25 1855 1865. (Pubitemid 30419049)
56. Zimmerman, S., Davis, J., Schultz, W. Ware, R 2003). Subclinical parvovirus B19 infection in children with sickle cell anemia. Journal of Pediatric Hematology/Oncology, 25 (5 387 389. (Pubitemid 36569970)
57. Zimmerman, S., Schult, W., Davis, J., Pickens, C., Mortier, N., Howard, T., et al 2004). Sustained long-term hematologic efficacy of hydroxyurea at maximum tolerate dose in children with sickle cell disease. Blood, 103 (6 2039 2045. (Pubitemid 38326218)