Perhaps, one of the most familiar and least understood forms of anemia is sickle cell. Sickle cell anemia is a disorder which occurs when abnormal haemoglobin causes red blood cells to sickle. Unfortunately, stroke occurs in some children with this disease, which in effect can lead to brain damage, paralysis, convulsions, coma and even death, reports Sade Oguntola. Looking at Remmy cuddling her teddy bearit is obvious she does not know she is sufferingfrom a life-threatening disease. She is a beautiful girl, whose energy does not depict as suffering from sickle cell anemia. But five years ago, her mother almost broke down when the doctor delivered the news that her daughter was sick. Remmy, who is now aged nine, was diagnosed with sickle cell disease.Remmy used to have attacks twice a month, lasting for days. Having mainly suffered in her legs, feet and arms, five years ago she began to get pains in her chest, considered more serious as they indicated lung damage. Remmy also had a brain scan which revealed she had suffered a stroke in the past.Doctors were worried this could happen again and the next time it might cause more damage. As such, Remmy was referred to a child neurologist who tested her and advised that it was best that she should receive regular blood transfusion.Sickle cell is the commonest single gene disorder of humans and has its highest prevalence among persons whose ancestors originate from malaria endemic areas. Although, the myth that sickle cell anemia is a 'black disease' persists in the public mind because it originated in sub-Saharan Africa and still affects a large number of blacks. It has since traveled across many continents and ethnicities.Sickle-cell anemia, named for the crescent moon-shape of affected red blood cells, results from the inheritance of two recessive genes that determine hemoglobin production. In individuals with sickle-cell anemia, abnormal hemoglobin causes red blood cells to sickle. Although sickle-cell anemia is only one of a group of disorders characterised by abnormal hemoglobin production, it is the most common.One can be a carrier of the sickle cell trait or have the sickle cell disease. Sickle cell trait occurs when someone inherits a normal hemoglobin gene from one parent (HbA) and a sickle hemoglobin gene (HbS) from the other (resulting in HbAS).For people with sickle-cell anemia, the disease manifests itself in a variety of ways. These symptoms may be mild or severe, depending on how many sickle cells the body produces and can include exhaustion and susceptibility to infection. But the most common symptom is known as an acute pain crisis, or painful episode, when the sickled red blood cells clump in small blood vessels and prevent the flow of oxygenated blood from reaching body tissues.The symptoms can appear anywhere in the body, and endanger virtually all organs, including the lungs and the brain. Pain becomes a harbinger of potential catastrophe; tissues die from lack of oxygen. 'Sickle cell anaemia is the leading cause of stroke in black children and, it is one of the devastating and potential fatal complications of sickle cell disease. About 10 per cent of children would have suffered a stroke by the age of 20 years, 'said Dr Ike Lagunju, a Consultant Paediatric Neurologist at the 2011 Ibadan Medical Specialists Group (IMSG) symposium.In Nigeria, about 150,000 children are born every year with sickle cell disease. Unfortunately, Dr Lagunju stated that 'in children with sickle cell disease, the risk of a stroke is highest between ages two and nine and between 40 and 75 per cent would have a recurrence after a first stroke in the absence of intervention.' Ironically, incidence of stroke in children with sickle cell disease is higher in Africa compared to what obtains in the United States of America.For instance, in a study carried out at the University College Hospital (UCH), Ibadan, Oyo State, '75 per cent suffered first stroke between the ages of two and nine years, but there was no stroke incidence in children less than two years. About one out of every four (26.1 per cent) experienced a recurrence within the first 12 months after the first stroke.'Although recovery from the stroke may be complete in some cases, frequently, the stroke can cause brain damage, paralysis, convulsions, coma and even death.Sadly, a repeat stroke causes greater brain damage and increases the risk of death. Repeat strokes occur in at least 60 per cent of the children who have already suffered one stroke unless treatment is given.'Nonetheless, ultrasound scan (Transcranial Doppler ultrasonography or TCD) can help to identify children with sickle cell disease who are at increased risk of stroke and subsequently blood transfusions every four to five weeks to prevent its occurrence, could be done,' stated Dr Lagunju.Ironically, due to challenges that fraught long term blood transfusion such as unavailability of blood, economic costs, transfusion reactions and transfusion-transmitted infections, she stated that hydroxyurea was an alternative to regular blood transfusion in preventing stroke in children with sickle cell disease.According to her, 'stroke prevention in sickle cell disease is feasible in Nigeria and hydroxyurea may be a more acceptable and effective preventive modality.'The only cure until now for sickle cell disease has been a bone marrow transplant. Stem cell transplants have been offered only to children with sickle cell disease whose organs are still healthy.Stem cells are transplanted by blood transfusion ' they travel through the arteries and end up in bone marrow. During this, extremely high doses of chemotherapy are given to kill the unhealthy stem cells within the existing bone marrow, before it is replaced with donor marrow.The procedure carries a 20 per cent risk of fatality and could be offered to sufferers only under the age of 18 as the internal organs of adult patients are typically too damaged by the disease to withstand chemotherapy.
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