Sickle cell disease is an umbrella term used to describe a group of inherited disorders of the haemoglobin known as haemoglobinopathies and includes Sickle Cell Anaemia, which generally is the most severe form. It is found in people of African and Caribbean origin and to a lesser extent in people of Mediterranean, Asian and Middle Eastern origin. People of these origins live all over the world, therefore the Sickle Cell disorder is of international significance, (Pembrey, 1994). Sickle Cell Disorder is the most common and fastest growing genetic disorder in England and now affects 15,000 people in the UK, and one in 2,400 babies, with prevalence growing (NHS, 2006). The most common forms of sickle cell disease (SCD) are sickle Haemoglobin C (Hb SC), Sickle Beta Thalassaemia (Hb S BThal), and Sickle Cell Anaemia (Hb SS).The most common and most severe form is Sickle Cell Anaemia (Hb SS). There is no cure. This assignment will briefly describe a case study with discussion of a patient admitted to hospital with one of the complications of Sickle Cell Anaemia, namely Acute Chest Syndrome. This assignment will further discuss the pathophysiology and management of Acute Chest syndrome (ACS). Case Study
A 19 year old African Caribbean male presented to the Emergency Admissions Unit (EAU) with severe pleuritic chest pain. The pain had begun three hours previously with coughing, fever and chills for the previous 24 hours. This was his first hospital admission this year with a sickle cell crisis, although he stated that he was was unwell two weeks previously. On examination, the patient was found to be dyspnoeic and pyrexial (39º C), tachycardic (Pulse 110), hypertensive (BP 145/90), with oxygen saturation at 90%. Bronchial breath sounds with inspiratory crackles were heard on inspiration. Assessment of the patient suggested chest infection leading to Acute Chest Syndrome (ACS). The...