Hydroxyurea therapy for sickle cell disease in Britain

BMJ 1998;316:1689-1690 ( 6 June )

Editorials

Hydroxyurea therapy for sickle cell disease in Britain

Disappointing recruitment despite promising results

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Sickle cell disease is the commonest inherited haemoglobinopathy in Britain and affects over 9000 people.¹ Clinical severityvaries considerably, but patients with the most severe diseasehave a life expectancy of just over 40 years. Conventional managementof the disease is largely supportive, highlighting a pressingneed for approaches that can alter the course of the disease.Trials in America have suggested that hydroxyurea can have a significantimpact on the course of the disease, but in Britain it is provingvirtually impossible to recruit patients into trials to confirmthese results in a British population.

The fact that the clinical severity of the sickle cell disease varies even within groups of patients with the same $beta$ globin genotype² has led to the concept that the disease is a multigenedisorder, with inheritance of $alpha$ thalassemia and genes controllingthe concentration of fetal haemoglobin, among others, modulatingdisease expression. ² ³ In those severely affected debilitating. . . [Full text of this article]

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European register of patients with sickle cell disease treated with hydroxyurea is being set up: Sally C Davies and Marilyn Roberts-Harwood
BMJ 1998 317: 541. [Extract] [Full Text]

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Maxwell, K., Streetly, A., Bevan, D. (1999). Experiences of hospital care and treatment seeking for pain from sickle cell disease: qualitative study. BMJ 318: 1585-1590 [Abstract] [Full text]
Yardumian, A, Olujohungbe, A, Cinkotai, K (1999). Register cannot replace prospective studies in sickle cell disease. BMJ 318: 671a-671 [Full text]
Davies, S. C, Roberts-Harwood, M. (1998). European register of patients with sickle cell disease treated with hydroxyurea is being set up. BMJ 317: 541a-541 [Full text]