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.
Assessment of clinical competence in nurse practitioner programmes has been greatly advanced over the last 10 years by widespread use of the objective structured clinical examination (OSCE). This article describes a modified OSCE developed in a nurse education department at a specific university within the UK to standardize evaluation of the physical examination skills of nurse practitioner students. Stations comprise procedure stations, which require students to perform a 'whole' examination of the subject, and question stations, which are composed of two forms of viva, involving various cognitive activities. A number of other universities are currently using this modified OSCE as a tool for formative and summative assessment, as a learning resource, and to identify gaps and weaknesses in clinical skills.
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