SICKLE CELL DISEASE
Sickle cell disease is the name given to a group of health conditions – usually inherited - that affect the body’s red blood cells. SP tells you all you need to know about this disease that’s common among Scotland’s increasingly large African and Caribbean communities…
caused by a gene that affects how the red blood cells develop.
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Genes come in pairs. A child will inherit one set of genes from the mother and one from the father, so, to be born with sickle cell disease, a child has to inherit a copy of the sickle cell gene from both of their parents.
In most cases, a patient’s red blood cells will be flexible and disc-shaped but, in sickle cell disease cases, they can become rigid and can alter to be shaped like a crescent or sickle. This is because the haemoglobin inside them blends together. As a result, these unusual cells can lead to the symptoms of the disease, simply because they do not live as long as ‘normal’ red blood cells and can become ‘stuck’ in blood cells.
Hereditary nature of disease If a child’s parents both have the sickle cell gene, then there’s a one in four chance of any child they have being born with the disease.
In addition, each child they have will have: • A one in two chance they will just inherit a copy of the sickle cell gene from one parent and be a carrier
• A one in four chance of inheriting copies of the sickle cell gene from both parents and will be born with the disease
That said, however, the child’s parents will often not have sickle cell disease themselves; they’re really only carriers of the sickle cell trait.
In most cases the disease will vary between mild to serious cases but, fortunately, the majority of people with it are able to lead happy, normal lives.
Symptoms The main symptoms of sickle cell disease are: • increased risk of serious infections • painful episodes • anaemia, which can cause tiredness and shortness of breath
Increased risk of serious infections Most people with sickle cell disease are more vulnerable to infections, especially younger people affected by the condition. These infections
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scottishpharmacist.com
n the United Kingdom, sickle cell disease is most commonly seen in people of African and Caribbean backgrounds. The condition is
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