Research updates
7 - The sickling disease page 6
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4. Managing sickle cell anaemia Link to the Medical Research Council web site
A lifelong treatment
Many of the serious complications of sickle cell anaemia occur in the first two years of life. Once babies with sickle cell anaemia have been identified by neonatal screening they are prescribed daily doses of penicillin which prevent death from infection during early childhood. This disease-preventing (prophylactic) treatment may be maintained throughout life. As they get older, the spleens of children with sickle cell anaemia get progressively more damaged by the sickling until they no longer function adequately. This also makes them prone to infection. Therefore, these children are now vaccinated against several disease-causing important organisms as well as being given regular doses of penicillin. Sickle cell crises require specialist care in hospital. Intravenous fluids, oxygen, antibiotics and painkillers (analgesics) are necessary. As the pain may be excruciating, powerful analgesics such as morphine may be needed.
During some crises, the concentration of haemoglobin in a patient's blood falls dangerously low. This may be due to infection causing temporary failure of the bone marrow to produce new red blood cells. A blood transfusion is then required to restore normal haemoglobin concentrations.
Bone marrow
A controversial form of treatment for a patient with sickle cell anaemia is bone marrow transplantation. Ideally, this procedure needs to be carried out in children under the age of twelve, using a sibling as the bone marrow donor. However, sickle cell anaemia is extremely variable in severity and it is impossible to predict the course the disease will take. Also, there is a high risk (10-20%) of the child dying or suffering complications following the transplant operation.
Question 7

a) Why is a sibling used as the bone marrow donor?

b) What ethical issues are raised by this form of treatment?

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