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Sickle Cell Anaemia What is haemoglobin? Haemoglobin is found in red blood cells. It contains iron and carries oxygen around the body. What is anaemia? Anaemia occurs when there are very few red blood cells in the body. This means there is not enough blood cells to carry the amount of oxygen needed by the body. When a child has anaemia they become pale and more tired than normal. What is sickle cell anaemia? Sickle cell anaemia occurs when haemoglobin in red blood cells is not normal. Normal haemoglobin (HbA) makes the blood cells round, soft and easy to move about even in very narrow blood vessels. Haemoglobin in sickle cell anaemia (HbS) is different. It makes the blood cells a crescent moon shape, hard, rigid and difficult to move around the blood vessels. The blood cells break easily because of their shape causing haemoglobin to spill. They get stuck in narrow blood vessels causing blockage. They can also pool in some organs of the body e.g. spleen, causing enlargement and damage. (Current Health, 1997) Section 2 Your child’s diagnosis and treatment 1 What causes sickle cell anaemia? Sickle cell anaemia is caused when a person receives two sickle genes from their parents. One gene is from the mother the other from the father. The gene tells the body to make abnormal haemoglobin. What is a gene? Genes give people their characteristics e.g. hair colour height etc. Half of your genes come from your mother and half from your father. What happens if the person receives only one sickle gene? If a person receives only one sickle gene they will not have sickle cell anaemia. The person will be a carrier meaning that they could pass the gene onto their children. Sickle gene = S Normal gene = A (Sickle Cell Society 2004) What are the signs and symptoms of sickle cell anaemia? Signs and symptoms can be mild to severe. Children may experience the following: • Infections - due to damage to the spleen by the sickle cells. The spleen is important in fighting infections. When spleen cells are damaged they are unable to destroy bacteria in the blood Section 2 Your child’s diagnosis and treatment 2 making the child more susceptible to infection. Infections are the most common symptom in infants and young children • Pain - can vary in length and severity and is due to blockage of oxygen flow to the tissues. It is often one of the first symptoms to present. It can occur frequently, with bone pain the most common site. • Swelling and pain in the hands and feet - due to blockage of oxygen flow to the tissues. More common in children under 5 years of age and known as hand-foot syndrome. • Tiredness, paleness and shortness of breath - due to anaemia • Yellow skin and eyes - due to rapid breaking down of red blood cells which causes the jaundice • Eye problems - due to lack of nourishment as a result of decreased red blood cells Regular eye examinations usually start around 5 years of age. • Strokes - due to blockage and damage of vessels, occur spontaneously • Chest problems - due to trapped sickle cells and or infection. More common in older children. What is the treatment? There is no cure for sickle cell anaemia but the signs and symptoms can be treated. Treatment depends on the severity of the disease and may include: • Pain relief -paracetamol or intravenous e.g. morphine, • Intravenous fluids - to aid in unblocking blood vessels • Blood transfusions - to increase the number of normal blood cells in the body. If regular blood transfusions are needed then another drug called Desferal is also given to prevent iron overload. • Antibiotics - to prevent and treat infections • Good nutrition - to keep your child healthy • Good hygiene - to minimise infection • Resting - to help with fatigue Section 2 Your child’s diagnosis and treatment 3 • Chest physiotherapy - to aid in preventing infection and lung complications • Regular GP check ups - to monitor your child’s progress • Immunisation - to decrease infections References 1997, 'It's in the blood sickle cell anaemia and other blood disorders', Current Health (online) vol.21 (accessed 19/5/04) Sickle Cell Disease Association of America, Inc.: www.sicklecelldisease.org/ KidsFirst for Health: illnesses – Sickle cell Anaemia: (Great Ormond St Hospital) http://www.goshkids.nhs.uk/kids/health/illnesses/index.html http://www.sicklecellsociety.org/sicklescene/pshomf.htm http://www.sicklecellkids.org/ http://www.nih.gov – NIH – Health Information click Health then find topic under alphabetical listing Please refer to section 7 “Sources of More Information” or check the parent’s library in McGuiness & McDermott children’s clinic for further information. Section 2 Your child’s diagnosis and treatment 4