This second of two articles on sickle cell disease (SCD) aims to provide advice on nursing care for patients. All health professionals should be aware of the various manifestations of the disease, the life-threatening complications and their optimal management. Prophylactic strategy is of paramount importance as it may avert many possible adverse outcomes. Recurrent episodes of acute, severe pain require frequent hospital admissions. The care of individuals with SCD must extend into the community and take account of domestic, employment and educational issues. Affected pregnant women should be looked after by units experienced in the care of women with this condition. The diagnosis of haemoglobin type is simple and inexpensive and a comprehensive national screening programme to detect SCD in pregnant women and newborn babies was started in April 2002 as part of the Government's broader aims to tackle inequalities in health and community.
Haemoglobinopathies refer to a range of genetically inherited disorders of red blood cell haemoglobin and include sickle cell disorders and thalassaemias. They occur most commonly in populations whose ancestors come from Africa, Asia, Mediterranean Islands, and the Middle and Far East. Haemoglobin (Hb) abnormalities (or haemoglobinopathies) are caused by (i) abnormalities of the protein structure; (ii) imbalanced globin chain production owing to reduced rate of synthesis of normal a or b globin chains; or (iii) a combination of the two. This article will focus on the biological basis of sickle cell disorders and will discuss the history and pathology of the conditions.
The level of training and competence in dealing with haemoglobinopathies (which mainly affect ethnic minorities in the UK) may not be totally adequate among nurses. Nurses indicated that they received little or no information in their teaching for working from a multiracial perspective and what they had learned was through experience and personal research since qualifying as nurses. Knowledge of the biological basis of inheritance, methods of acquisition of thalassaemia and sicklecell anaemia and the ethnic profile of people affected by these conditions may not be totally adequate among nurses. Many nurses wanted more training, including those who had already received instruction, since this was described as 'far too vague', 'not constructive', 'minimal', or 'embarrassingly insufficient', recommending that instruction be given by a sickle-cell anaemia/thalassaemia counsellor with a contribution from patients. A combination of poor quality, or lack, of instruction, together with time and resource pressures, is responsible for this limited understanding, resulting in insufficient awareness of the health needs of ethnic minorities leading to inequalities in healthcare provision.
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