medigraphic.com
ISSN 1561-2996 (Electronic)
ISSN 0864-0289 (Print)

2017, Number 2

<< Back Next >>

Rev Cubana Hematol Inmunol Hemoter 2017; 33 (2)

Emergency management of adult patients with sickle cell disease in Institute of Hematology and Immunology

Quintero-Sierra Y, Dávila-Ramos Y, Svarch E, Hernández-Padrón C, Granda BM

Language: Spanish
References: 40
Page: 1-12
PDF size: 150.12 Kb.


ABSTRACT

Introduction: Sickle cell disease is the most important structural hemoglobinopathy in the world; and is characterized by chronic hemolytic anemia and recurrent episodes of vascular occlusion.
Objective: To determine the leading causes of adult patient care service with sickle cell disease in the Emergency Department of the Institute of Hematology and Immunology.
Methods: 276 clinical events presented in 143 patients with sickle cell disease that came to Emergency Department b etween February 2012 and November 2013 were included.
Results: Most patients were categorized in the group of 18 to 27 years representing 79 clinical events (28.6%) with female predominance (55.1%). According to the type of sickle cell disease, 76.1% of patients corresponded to sickle cell anemia. Most clinical events occurred during the months of May and August. The main reason for medical assistancewas pain in 200 clinical events representing 72.2% of cases. White blood cell counts and reticulocytes were higher in patients admitted. The most common clinical event was the painful vaso-occlusive crises, represented by 165 events (59.6%). The most frequent clinical events for admission were acute chest syndrome and hepatobiliary diseases with 100% and 91.7%, respectively.Intravenous hydrationand painkillers were the most commonly used treatments with 78.3% and 76.2%, respectively. The main way of hospital reception was on a voluntary basis and the mean observation time was approximately 6.4 hours.
Conclusions: Oportune medical management and measures implemented since the arrival of patients to the Emergency Department will allow reducing complications and the number of hospital admissions as well as improving the quality of life of these patients and increasing their survival.


REFERENCES

  1. Espinosa E, Svarch E, Martínez G, Hernández P. La anemia drepanocítica en Cuba. Experiencia de 30 años. RevCubana HematolInmunolHemoter 1995;12:97-105.

  2. Colombo B, Svarch E, Martínez G. Genética y clínica de las hemoglobinas humanas. La Habana: Pueblo y Educación 1993, p 146-83.

  3. Svarch E. Fisiopatología de la drepanocitosis. Rev Cubana Hematol Inmunol Hemoter [revista en la Internet]. 2009 Abr [citado 2013 Dic 14];25(1):Disponible en: http://scielo.sld.cu/scielo.php?script=sci_arttext &pid=S0864- 02892009000100003&lng=es.

  4. Svarch E. Programa cubano de atención integral al paciente con drepanocitosis. Rev Cubana HematolInmunolHemoter. 2011;27(2):165-7.

  5. Prabhakar H, Haywood C, Malokie R. Sickle cell disease in the United States: Looking back and forward at 100 years of progress in management and survival. Am J Hematol.2010;(85):346-53.

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

  7. Ballas SK, Lieff S, Benjamin LJ, Dampier CD, Heeney MM, Hoppe C, et al. Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol. 2010;85(1):6-13.

  8. Bain BJ. Haemoglobinopathy diagnosis: Algorithms, lessons and pitfalls. Blood Rev. 2011;25:205-13.

  9. Shankar SM, Arbogast PG, Mitchel E, Cooper WO, Wang WC, Griffin MR. Medical care utilization and mortality in sickle cell disease: A population-based study. Am J Hematol. 2005;80(4):262-70.

  10. Daleke DL Devaux PF, Zachowski A. Regulation of phospholipid asymmetry in the erythrocyte membrane. CurrOpinHematol. 2008 May;15(3):191-5.

  11. Ballas SK, Gupta K, Adams-Graves P. Sickle cell pain: a criticalreappraisal. Blood 2012;120:3647-56.

  12. Field JJ, Lin G, Okam MM. Sickle cell vasoocclusion causes activation of iNKT cells that isdecreased by the adenosine A2A receptor agonist regadenoson. Blood. 2013;121(17):3329-34.

  13. Sheth S, Licursi M, Bhatia M. Sickle cell disease: time for a closer look at treatment options? Br J Haematol. 2013 Aug;162(4):455-64.

  14. Manwani D,Frenette PS. Vaso-occlusion in sickle cell disease: pathophysiology and novel targeted therapies. Blood. 2013;122:3892-98.

  15. Panepinto JA, Brousseau DC, Hillery CA, Scott JP. Variation in hospitalizations and hospital length of stay in children with vasoocclusive crises in sickle cell disease. Pediatr Blood Cancer. 2005; 44:182-6.

  16. Ellison AM, Ota KV, McGowan KL, Smith-Whitley K. Epidemiology of bloodstream infections in children with sickle cell disease. Pediatr Infect Dis J. 2013 May;32(5):560-3.

  17. Ellison AM, Ota KV, McGowan KL, Smith-Whitley K. Epidemiology of bloodstream infections in children with sickle cell disease. Pediatr Infect Dis J. 2013 May; 32(5):560-3.

  18. Shankar SM, Arbogast PG, Mitchel E, Ding H, Wang WC, Griffin MR. Impact of proximity to comprehensive sickle cell center on utilization of healthcare services among children with sickle cell disease. Pediatr Blood Cancer. 2008;50:66-71.

  19. Ballas SK. More definitions in sickle cell disease: steady state vs base line data. Am J Hematol.2012;87(3):338.

  20. Svarch E, Machín García S, Arencibia Núñez A, Hernández Padrón C. Normas para el tratamiento de la drepanocitosis. Grupo Nacional de Hematología. Instituto de Hematología e Inmunología. La Habana, Cuba 2013 disponible en: www.sld.cu/sitios/hematologia . (Citado 2015 Dic 14 )

  21. Brown M. Managing the acutely ill adult with sickle cell disease. Br J Nurs. 2012;21(90-2):5-6.

  22. Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steiberg MH, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994 Jun; 330(23):1639-44.

  23. Hussain R Yusuf, Hani K, Scott D, Christopher S, Althea M. Grant. Emergency Department Visits Made by Patients with Sickle Cell Disease. Am J Prev Med. 2010; 38(4S):S536-41.

  24. Hari P, Haywood C Jr, Robert M. Sickle cell disease in the United States: Looking back and forward at 100 years of progress in management and survival. Am J Hematol. 2010;85:346-53.

  25. Wolfson J, Schrager S, Khanna R, Coates T, Kipke M. Sickle Cell Disease in California: Sociodemographic Predictors of Emergency Department Utilization. Pediatr Blood Cancer. 2012 January; 58(1):66-73.

  26. Sheth S, Licursi M, Bhatia M. Sickle cell disease: time for a closer look at treatment options? Br J Haematol. 2013 Aug; 162(4):455-64.

  27. Adeyemo TA, Ojewunmi OO, Diaku-Akinwumi IN, Ayinde OC, Akanmu AS. Health related quality of life and perception of stigmatisation in adolescents living with sickle cell disease in Nigeria: A cross sectional study. PediatrBloodCancer. 2015 Jul; 62(7):1245-51.

  28. Morales E. La Hemoglobinopatía S en el cuerpo de guardia. Trabajo de terminación de residencia de Hematología. La Habana: Instituto de Hematología e Inmunología; 1987.

  29. González JJ. La crisis vasoclusivas de la hemoglobinopatía S. Su relación con el clima. 1984. Trabajo de terminación de residencia en Hematología. La Habana: Instituto de Hematología e Inmunología; 1987.

  30. Wang Y, Barker K, Shi S. Blockade of PDGFR- b activationinhibits morphine analgesic tolerance. Nat Med. 2012;18:385-7.

  31. Kutlar A, Ataga KI, McMahon L. A potentoral P-selectin blocking agent improvesmicrocirculatory blood flow and a marker ofendothelial cell injury in patients with sickle celldisease. Am J Hematol. 2012;87(5):536-9.

  32. Sebastiani P, Nolan VG, Baldwin CT, Abad-Grau MM, Wang L, Adeboye H,et al. A network model to predict the risk of death in sickle cell disease. Blood Rev. 2011 Jan; 110:2727-35.

  33. Olujohunghe A. The clinical care of adult patients with sickle cell disease. Br J Hosp Med. 2008;69:616-9.

  34. Miller AC, Gladwin MT. Pulmonary complications of sickle cell disease. Am J RespirCrit Care Med. 2012; 185(11):1154-65.

  35. Sparkenbaugh E, Pawlinski R. Interplay betweencoagulation and vascular inflammation in sicklecell disease. Br J Haematol. 2013;162(1):3-14.

  36. Rabindra N. Paul, Oswaldo L. Castro, Anita Aggarwal, Patricia A. Oneal. Acute chest syndrome: sickle cell disease. Eur J Haematol. 2011; 87:191-207.

  37. Vincent L, Vang D, Nguyen J. Mast cell activation contributes tosickle cell pathobiology and pain. Blood 2013;122:1853-62.

  38. Haywood C Jr , Tanabe P , Naik R , Beach MC , Lanzkron S . The impact of race and disease on sicklecell patient wait times in the emergency department. Am J Emerg Med. 2013 Apr; 31(4):651-6.

  39. Galarneau G, Coady S, Garrett ME, Jeffries N, Puggal M, Paltoo D, et al. Genecentric association study of acute chest syndrome and painful crisis in sickle cell disease patients. Blood Rev. 2013 July 122(3):434-42.

  40. Lewing K, Britton K, DeBaun M, Woods G. The impact of parenteral narcotic choice in the development of acute chest syndrome in sickle cell disease. J PediatrHematol Oncol.2011; 33(4):255-60.




2020     |     www.medigraphic.com