Sickle Cell Disease

This chapter presents a detailed case scenario of a 15-year-old girl with sickle cell disease (SCD) who was brought to Accident & Emergency with pain in her chest, radiating to her abdomen in question and answer type format. SCD results from the presence of a mutated form of haemoglobin, haemoglobin S. The main symptoms of sickle cell disease are anaemia and episodes of severe pain, but there are a number of other complications such as stroke, acute chest syndrome, priapism, and jaundice. SCD is a chronic long term life-limiting condition, knowledge of SCD is therefore important for the children’s nurse as the disease has serious implications for the quality of life and the life chances of children. Taking a child, young person and family-centred approach, the chapter offers a comprehensive and accessible discussion of care planning with continuous reference to core principles and nursing values.