Sickle Cell Simply Explained

FREE PREVIEW The term Sickle Cell Disease is used for a group of inherited conditions in which sickle haemoglobin occurs either alone or in combination with other unusual haemoglobins. It includes Sickle Cell Anaemia (Hb SS), Haemoglobin SC Disease (Hb SC) and Sickle Beta Thalassaemia (Hb S Beta-Thal) and others. Of these the most severe is Sickle Cell Anaemia (Hb SS) Sickle Cell Disease is the most common genetic disease world-wide. For a person to suffer the disease, he or she must inherit the faulty gene from each parent. The disease affects millions of people in the world. It is a chronic illness of serious proportions. Medical, psychological and socioeconomic consequences place a great burden on sufferers and their Carers. In the past sufferers of sickle cell anaemia survived for only a few years. When only one sickle cell gene is inherited with a normal haemoglobin, the condition is referred to as the carrier or the trait. Individuals with the trait have no medical problems under normal conditions of life. The carrier state is also protective to health in some ways to those with it. This is how the gene has been able to survive and passed on from generation to generation. The trait can have serious implications for marriage and childbearing. It is therefore imperative for people intending to have children to know about it in order to prevent the disease. It is worth emphasising that the trait is not a disease. Sickle cell disease which was once thought to be restricted to only a few areas, especially among Black people, is now known to be world-wide. The gene is most common in individuals originating from the following countries: Africa, South America, Central America, The Caribbean Islands, The Mediterranean (Turkey, Italy and Greece), India and Saudi Arabia. It has been described as a world-health problem. It is not always easy to tell where people’s ancestors originated from by looking at their skin colour or their physical features. Also intermarriages with widespread migration which are on the increase have spread the sickle cell gene far and wide. Sickle cell disease can affect virtually every system in the human body. It can ultimately lead to multiple organ failure and premature death, mostly in children under five years, adolescents and pregnant women. The aim of the book is to present information about sickle cell and its associated conditions in a very simple way for everybody who wants to know, to understand them. For workers in sickle cell, it offers some simple non-technical language in which to answer questions about and discuss the subject with others. The book begins with basic facts about blood, blood cells, haemoglobin and other features which should help with the understanding of sickle cell disease, sickle cell trait and other haemoglobins. Owing to the disease being inherited, the basic principles of genetics (inheritance) are explained. There are also some explanations of various normal functions of the human body which can be affected in the disease. At present there is no readily available cure for sickle cell disease. However, with proper management of the many ill-effects of the disease, sufferers can be assisted to live comfortable lives and for many years. Lifestyle measures are paramount and are referred to in some detail in the book. All these require awareness of the disease. There are many myths and misinformation about Sickle Cell disease and other haemoglobin abnormalities like Thalassaemia. These result in a hindrance to the appropriate management of the conditions especially by sufferers and their families. This book gives general awareness, proper knowledge and understanding of all aspects of sickle cell disease to enable sufferers, their families and other carers to accept and help manage it properly. It is unfortunate that at present there are many communities where care for Sickle Cell sufferers and other medical conditions has not reached. Those living in well-developed countries, big towns and cities are the ones who benefit from good diagnostic and medical care. There is the need for publicising sickle cell in communities, schools, religious institutions, health establishments and the media in order to reach as many people as possible. A simple book like this could serve the purpose for people everywhere including those in the remotest areas. It is worth reading it!