Complications and life expectancy in sickle cell disease: the greatest challenge

Araújo, Adérson

doi:10.1590/S1516-84842010000500004

SCIENTIFIC COMMENTARIES

Fundação Hemope, Hemocentro de Recife (PE), Brazil

Correspondence

Frequently since the third issue of volume 29 (July-September) of this journal in 2007, we have seen new articles on sickle cell disease.⁽¹⁾ Undoubtedly, the epidemiological relevance of this group of diseases and their intricate pathophysiological mechanisms raise scientific curiosity to try to understand the reasons for the inexorable progression of chronic lesions to molecules and cells, tissues and organs,⁽²⁾ an evolution that contributes to such low survival rates worldwide.

The article entitled "Study of morbidity and mortality in sickle cell disease," of Paulo Roberto Juliano Martins, Helio Moraes de Souza Braga and Talita Silva from the Regional Blood Bank of Uberaba, Hemominas Foundation, Federal University of Triangulo Mineiro, in this issue shows epidemiological data on morbidity and death that are probably very common to other Brazilian institutions.⁽³⁾

It is possible that, in the future, with early care and guidance, a significant reduction in the morbidity and mortality resulting from sickle cell disease will occur. An understanding of the pathophysiological mechanisms⁽²⁾ and the detection of modulation factors of severity of these diseases⁽⁴⁾ may indicate conducts that can reduce the so early loss of life and reduce the suffering of those affected. For now however, what the results of the study⁽³⁾ reveal is that life expectancy is still less than 45 years old and that most patients suffer complications.

The study also shows that 82% of patients have homozygous sickle cell disease (SS) and that two thirds of all cases have painful crises that lead to hospital treatment despite the availability of hydroxyurea and of its reported benefits. Is it perhaps ineffective or is the medication being underused? What should be done to ameliorate the plight of people with sickle cell disease? Education of caregivers including health teams from primary care to highly complex institutions, selfcare?⁽⁵⁾ It is clear that education is fundamental; the structuring of a very specific program has been defended in Jamaica and the USA, with some centers dedicated to the care of pain and of acute and chronic symptoms,^(6,7) and to some extent also in Brazil with the care provided by blood centers, some even with an infrastructure to hospitalize patients.

Finally, the study concludes that the epidemiological profile shows a predominance of children and young adults, female and the SS genotype. The hospital admission rates in University Hospital de Clínicas of the Universidade Federal do Triângulo Mineiro and consultation rates in the Regional Blood Center of Uberaba and the low mean age at death confirm the high morbidity and mortality in sickle cell disease. However, the large number of children without complications or admissions reflects the effectiveness of the preventive measures offered by early diagnosis established over the last 10 years.⁽³⁾

It would be interesting for other services that attend people with sickle cell disease to follow up this study so that a national registry could be established and coordinated by the Ministry of Health in order to have a national profile with a multicenter model.⁽⁸⁾Also the impact of recently established public policies such as neonatal screening should be assessed in the adult population of the future and all the publications on the disease be developed and distributed by the Ministry of Health.

References

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2. Zago MA, Pinto ACS. Fisiopatologia da doença falciforme: da mutação genética à insuficiência de múltiplos órgãos. Rev Bras Hematol Hemoter.[Internet]. 2007 [citado 2009 Mar 19];29 (3): 207-14. Disponivel em: http://www.scielo.br/pdf/rbhh/v29n3/v29n3a03.pdf
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