Practical management of pain in sickling disorders.

Sickle cell disease affects approximately 5000 people in the UK predominantly of African and Afro-Caribbean origin.1 The majority of those affected live in inner city areas; for example it is the most common inherited condition in the City and Hackney district of London. There is a high morbidity and mortality in children with up to 10% dying within the first 10 years of life.2 Sickle cell related pain is the most frequent problem experienced by young patients.1 3 4 In a carefully documented home study of children with homozygous sickle cell disease the number of painful crises averaged 3-9 per patient per year, not all requiring hospital admission but severe enough to undermine normal activities.5 The disease varies in severity depending in part on the genetic abnormality inherited; those homozygous for the classical sickle mutation tend to have the greatest frequency of painful episodes followed by sickle ,' thalassaemia, haemoglobin SC disease, and sickle PI thalassaemia.6 However within each genotype the number of painful episodes experienced per year varies widely.6 A recent large study found that approximately 25% of those with homozygous sickle cell anaemia have severe disease with three to 10 admissions per year, 50% have at least one severe episode per year, and the remainder rarely present with sickle cell related pain.6 7 The inpatient stay is usually between 5-10 days in hospital, but may be longer making this complication a considerable source of morbidity and consumer of resources.6-8 Sickle cell related pain occurs as an acute circumscribed event within the context of a chronic illness presenting a clinical challenge whose management is controversial.9 10 There is substantial evidence that pain is undertreated in children, not least in those with sickling disorders.11-3 This may occur due to fear of inducing addiction or a failure to understand the importance of treating pain in children and is hindered by the complexity of assessing its severity.11-13 Pain in children should be judiciously and competently treated in order to minimise immediate complications and allow normal emotional and physical development.'4 The increased life expectancy for those with sickling disorders means that the long term consequences of treating sickle cell related pain in children needs to be carefully considered in terms of development, function, and quality of life. This is best achieved within the context of a multidisciplinary team approach.