Friday, March 12, 2010
Wait A Minute; What is Sickle Cell Disorder?
Sickle cell disorder (SCD) is a family of inherited disorders,
of which the most severe and common is sickle cell anaemia
(HbSS). SCD affects how red blood cells carry oxygen around
the body, is a lifelong condition that affects both males and
females, and is the most common genetic disorder in England.
Sickle cell disease can cause fluctuating symptoms ranging
from pain and infection to anaemia and fatigue.
What do sickle cells look like?
Normal red blood cells (see above picture) are disc-shaped,
flexible and can squeeze easily through small blood vessels.
These cells contain haemoglobin which delivers oxygen to where
the body needs it and give the blood cells their red colour.
People with SCD have abnormal haemoglobin. When this
haemoglobin gives away oxygen it sticks together to form long
rods in the red blood cell. These rods become rigid and can
change the red cell from round and flexible to a shape like
a sickle. It is because of this shape that sickled red blood cells
cannot flow easily through small blood vessels. This means the small blood vessels
can get blocked and stop oxygen from flowing.
Who does sickle cell disease affect?
SCD affects mainly people of African and Caribbean descent,
but people from Mediterranean and Asian backgrounds can also
be affected by it.
How many people have it?
There are about 12,500 people with SCD in the UK. It is thought
that the number will increase in the next 10 years.
Why do I need to know about stroke?
Children with SCD are more likely to have strokes than those
without the disease. Of all people with SCD, the risk of stroke is
highest in the most commonly detected type of sickle cell
disease – sickle cell anaemia (HbSS).
The risk of stroke in children with SCD is greatest between
the ages of 2 and 16. About 10% of all children with SCD
will have a stroke by the time they are 20 years old. Stroke
recurrence is also a major concern for children and their families.
Stroke recurs in over 60% of children with SCD.
If you have a child with SCD, make sure you speak to their
doctor about the risk of stroke.
Tips for parents
• If your child has SCD, make sure you speak to their
doctor to find out more about reducing the risks
of stroke and other complications.
• Make sure that the school is aware of your child’s
condition and has drawn up a care plan.
• Provide the school with booklets to explain
what SCD is.
• Make sure family members and teachers are aware
of the signs of stroke and the possible indications
of ‘silent’ stroke.
Tips for teachers
• Be aware of children with SCD in your school and
draw up a care plan.
• Listen to the needs of children with sickle cell, especially
regarding fatigue, dehydration and toilet needs.
• Make sure you are in regular dialogue with parents
of children with SCD.
• Learn the signs of stroke and ‘silent’ stroke. Look out for
changes in behaviour or attention as these may be signs
that a child has had a ‘silent’ stroke