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Both prevention and treatment are important when managing sickle cell disease

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Abstract

The management of pain and prevention of end organ damage in patients with sickle cell disease is challenging. Treatment options include antibacterial prophylaxis, appropriate vaccinations, hydroxycarbamide therapy and regular blood transfusion, with the only available cure being haematopoietic stem cell transplantation. Special consideration should be given to patient well-being and management options in low-income countries.

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References

  1. Amid A, Odame I. Improving outcomes in children with sickle cell disease: treatment considerations and strategies. Pediatr Drugs. 2014;16(4):255–66.

    Article  Google Scholar 

  2. Aygun B, Odame I. A global perspective on sickle cell disease. Pediatr Blood Cancer. 2012;59(2):386–90.

    Article  PubMed  Google Scholar 

  3. Ballas SK, Gupta K, Adams-Graves P. Sickle cell pain: a critical reappraisal. Blood. 2012;120(18):3647–56.

    Article  CAS  PubMed  Google Scholar 

  4. Meier ER, Miller JL. Sickle cell disease in children. Drugs. 2012;72(7):895–906.

    CAS  PubMed Central  PubMed  Google Scholar 

  5. Benjamin LJ, Swinson GI, Nagel RL. Sickle cell anemia day hospital: an approach for the management of uncomplicated painful crises. Blood. 2000;95(4):1130–6.

    CAS  PubMed  Google Scholar 

  6. Rees DC, Olujohungbe AD, Parker NE, et al. Guidelines for the management of the acute painful crisis in sickle cell disease. Br J Haematol. 2003;120(5):744–52.

    Article  PubMed  Google Scholar 

  7. Gaston MH, Verter JI, Woods G, et al. Prophylaxis with oral penicillin in children with sickle cell anemia: a randomized trial. N Engl J Med. 1986;314(25):1593–9.

    Article  CAS  PubMed  Google Scholar 

  8. Halasa NB, Shankar SM, Talbot TR, et al. Incidence of invasive pneumococcal disease among individuals with sickle cell disease before and after the introduction of the pneumococcal conjugate vaccine. Clin Infect Dis. 2007;44(11):1428–33.

    Article  CAS  PubMed  Google Scholar 

  9. Gill FM, Sleeper LA, Weiner SJ, et al. Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease. Blood. 1995;86(2):776–83.

    CAS  PubMed  Google Scholar 

  10. Rogers ZR, Buchanan GR. Bacteremia in children with sickle hemoglobin C disease and sickle beta+ thalassemia: is prophylactic penicillin necessary? J Pediatr. 1995;127(3):348–54.

    Article  CAS  PubMed  Google Scholar 

  11. Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000;342(25):1855–65.

    Article  CAS  PubMed  Google Scholar 

  12. Paul RN, Castro OL, Aggarwal A, et al. Acute chest syndrome: sickle cell disease. Eur J Haematol. 2011;87(3):191–207.

    Article  CAS  PubMed  Google Scholar 

  13. Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91(1):288–94.

    CAS  PubMed  Google Scholar 

  14. Fullerton HJ, Adams RJ, Zhao S, et al. Declining stroke rates in Californian children with sickle cell disease. Blood. 2004;104(2):336–9.

    Article  CAS  PubMed  Google Scholar 

  15. Kinney TR, Ware RE, Schultz WH, et al. Long-term management of splenic sequestration in children with sickle cell disease. J Pediatr. 1990;117(2 Pt 1):194–9.

    Article  CAS  PubMed  Google Scholar 

  16. Manwani D, Frenette PS. Vaso-occulsion in sickle cell disease: pathophysiology and novel targeted therapies. Blood. 2013;122(24):3892–8.

    Article  CAS  PubMed  Google Scholar 

  17. Wang WC, Ware RE, Miller ST, et al. Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet. 2011;377(9778):1663–72.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  18. Ohene-Frempong K. Indications for red cell transfusion in sickle cell disease. Semin Hematol. 2001;38(1 Suppl. 1):5–13.

    Article  CAS  PubMed  Google Scholar 

  19. Hsieh MM, Fitzhugh CD, Tisdale JF. Allogeneic hematopoietic stem cell transplantation for sickle cell disease: the time is now. Blood. 2011;118(5):1197–207.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

Download references

Disclosure

This article was adapted from Pediatric Drugs 2014;16(4):255–66 [1] by salaried/contracted employees of Adis/Springer. The preparation of these articles was not supported by any external funding.

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Adis Medical Writers. Both prevention and treatment are important when managing sickle cell disease. Drugs Ther Perspect 30, 411–416 (2014). https://doi.org/10.1007/s40267-014-0158-9

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  • DOI: https://doi.org/10.1007/s40267-014-0158-9

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