Helping children learn to live with Sickle Cell Disease
17 September 2003
An Action Medical Research project has found that some children with Sickle cell disease (SCD) may be at risk of learning difficulties because of their condition. The study has also used new techniques to monitor the development of babies with SCD.
Children with SCD suffer from poor blood circulation and a reduced blood flow to the brain. This can often lead to stroke and damage to parts of the brain that are important for a vast array of skills such as attention, short term memory, planning, coordinated movements, and speech. Children who have damage to these brain regions often find it difficult to maintain their attention and to concentrate. Over time, these problems can lead to learning difficulties and interfere with the course of intellectual development.
The study involved a cohort of 50 children and a group of babies with SCD. The aim was to use a combination of behavioural, and brain imaging techniques to track the development of the children, and also to detect any signs of abnormality that may be related to the disease.
Simon Moore, Chief Executive of Action Medical Research said: “This is a really devastating disease that affects both these young people and their families. Having Sickle Cell Disease means they have to live with pain and difficulties all their lives. This research is hopefully going to ensure that any potential learning problems are picked up early on, when something can be done about it, so these children can have the best start in life.”
Leading the research Professor Faraneh Vargha-Khadem of the Institute of Child Health said: “At present there is no cure for Sickle Cell Disease. Our aim is to identify cognitive problems early on, and to intervene if the child is considered to be at risk of developing learning difficulties. By intervening early in the educational process we hope to remedy the learning difficulties before they become deeply rooted.”
“We have used a multi-disciplinary, and a longitudinal approach to study the babies and children with Sickle Cell Disease. Currently the learning problems associated with SCD are often diagnosed only when the child has reached adolescence. By this time the difficulties have become instilled and have caused psychological damage as well. This has a bad effect on these young people so it is really important that effective methods of therapy are introduced early on to help the most vulnerable.”
The children underwent a number of tests including brain imaging, measurement of their brain waves, blood flow to the brain, and levels of oxygen in the blood. Each child was examined twice so that any changes in brain or behaviour related to the progression of the disease could be identified.
The babies in the study were assessed at three, nine and twelve months using measurements of brain waves, blood flow, as well as standardized tests of cognitive development.
SCD is a disease affecting people of Afro-Caribbean descent and in London alone there are approximately 10,000 people with the condition. The condition affects blood cells and causes them to stick together. This in turn affects blood circulation and can lead to blocked blood vessels and damaged organs. The irregular shape of the blood cells also interferes with the transport of oxygen around the body, and some parts of the brain are particularly vulnerable to reduced levels of oxygen.
It is the blocked vessels that cause the acute pain particularly around the back and chest. These episodes of acute pain are referred to as a Sickle Cell crises. Other symptoms include anaemia, painful swelling of the hands and feet and ulcers on the lower legs. Some sufferers only have one crisis a year, whereas others have many and require hospitalisation to receive pain relief.
For more information or to interview Professor Faraneh Vargha-Khadem please telephone Louise Brown on 01403 327403 or Rob Orme on 01403 327404.
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