at least every 4 hours · People with an acute painful sickle cell episode should be assessed for acute chest syndrome if they have one or more of the following: abnormal respiratory signs or symptoms, chest pain, fever or hypoxia. Annual audit of the first of these quality standards is included in the data which specialist haemoglobinopathy centres must collect for the Quality Dashboard. The British Society of Haematology has produced national guidance on some aspects of sickle cell care and audit templates have been produced for their guidelines. These are available on their website (www.b-s-h.org.uk) and include the following: Chapter 2: Organisation of care Standards for Clinical Care of Adults with Sickle Cell Disease in the UK, 2nd Edition 49 · Red Cell Transfusion in Sickle Cell Disease Part 1: Principles and Laboratory government grants, membership and special events to support Sickle cell anaemia (SCA), the most common inheritable disease in Africa, leading to public health problem in the region and elsewhere where descendants of Africans have settled (Roberts and Montalembert, 2007). Worldwide, it is recognized as a major cause of morbidity and mortality with tremendous social and economic impact mainly due to the recurrent acute episodic clinical events called “crises” and hospitalization (Lena et al., 2012). In Africa, SCA is estimated to contribute to an equivalent of 5% of under-five deaths, and only half of the affected children live beyond their fifth birthday (Lena et al.,2012). The symptoms and complications of sickle cell disease (SCD) arise mainly from the crises (clinical and subclinical). Activation and damage of endothelial cells with activation of adhesion molecules lead to inflammation, release of C-reactive protein (CRP) and other inflammatory mediators and subsequent enhancement of ischemia. (Manwani and Frenette, 2013). A study done by Raphael and Vichinsky, (2005) have shown that inflammatory markers such as C-reactive protein (CRP) and tumor necrosis factor-alpha (TNF-α) are elevated in SCA patients. Also (Akinlade et al. 2013) reported elevated CRP level in SCA patients. The above and other lines of evidence suggest that SCD is associated with a chronic inflammatory state, in which inflammation, oxidative stress and tissue oxidative damage occur, leading to various degrees of disease severity and end-organ dysfunction (Bandeira et al., 2014). 3 1.2. Literature Review 1.2.1 .Sickle cell anaemia: Sickle-cell anaemia (SCA), is a hereditary blood disorder, that results from a single point mutation in the haemoglobin B chain by substitution of a valine in state of glutamic acid at 6 position ( Schnog et al.,2004). 1.2.1.1. History of sickle cell anemia: In 1910, Herrick described an anemia characterized by bizarre, sickle-shaped cells. The role of deoxygenation was discovered in the 1920’s by Hahn and Gillespie. The hereditary nature of the disease was suspected but not demonstrated until 1949 by Dr. James V. Neel. The association with hemoglobin was discovered by Linus Pauling and Harvey Itano in 1951 and the actual amino acid substitution by Vernon Ingram in 1956. Thus the 100th anniversary marks the discovery of this ancient disease from Africa by western medicine and naming of the disease for a simple agricultural implement to which a medical resident in 1910 likened the shape of the abnormal cells he saw under the microscope (Herrick, 2001). 1.2.1.2. Inheritance: Sickle cell disease is inherited as an autosomal recessive condition whereas sickle cell trait is inherited as an autosomal dominant trait. This means that the gene can be passed on from a parent carrying it to male and female children. In order for sickle cell disease to occur, a sickle cell gene must be inherited from both the mother and the father, so that the child has two sickle cell genes (Samir, 2012). The inheritance of just one sickle gene is called sickle cell trait or the "carrier" state. Sickle cell trait does not cause sickle cell anaemia. Patients with sickle cell trait 4 usually do not have many symptoms of disease and have normal hospitalization rates and life expectancies. 1.2.1.3. Distribution of sickle cell anaemia Worldwide, it is recognized as a major cause of morbidity and mortality with tremendous social and economic impact mainly due to the recurrent acute episodic clinical events called “crises” and hospitalizations (Lena et al., 2012). 1.2.1.3.1. In Africa: SCA is estimated to contribute to an equivalent of 5% of under-five deaths, with up to 16% in some countries such as Nigeria and only half of the affected children live beyond their fifth birthday. The highest frequency of sickle cell disease is found in tropical regions, particularly sub-Saharan Africa, India and the Middle-East (Weatherall and Clegg, 2001). Migration of substantial populations from these high prevalence areas to low prevalence countries in Europe has dramatically increased in recent decades and in some European countries sickle-cell disease has now overtaken more familiar genetic conditions such as hemophilia and cystic fibrosis (Roberts and Montalembert, 2007).. In 2010, there were about 29,000 deaths attributed to sicklecell disease globally ( Weatherall and Clegg, 2001). Sickle-cell disease occurs more commonly among people whose ancestors lived in tropical and sub-tropical sub-Saharan regions where malaria is or was common. Where malaria is common, carrying a