What is Sickle Cell Anaemia?
The disorder affects the red blood cells which contain a
special protein called haemoglobin (Hb for short). The function
of haemoglobin is to carry oxygen from the lungs to all
parts of the body.
People with Sickle Cell Anaemia have Sickle haemoglobin
(HbS) which is different from the normal haemoglobin (HbA).
When sickle haemoglobin gives up its oxygen to the tissues,
it sticks together to form long rods inside the red blood
cells making these cells rigid and sickle-shaped. Normal
red blood cells can bend and flex easily.
Because of their shape, sickled red blood
cells can't squeeze through small blood vessels as easily
as the almost donut-shaped normal cells. This can lead to
these small blood vessels getting blocked which then stops
the oxygen from getting through to where it is needed. This
in turn can lead to severe pain and damage to organs.
Everyone has two copies of the gene for
haemoglobin; one from their mother and one from their father.
If one of these genes carries the instructions to make sickle
haemoglobin (HbS) and the other carries the instructions
to make normal haemoglobin (HbA) then the person has Sickle
Cell Trait and is a carrier of the sickle haemoglobin gene.
This means that this person has enough normal haemoglobin
in their red blood cells to keep the cells flexible and
they don't have the symptoms of the sickle cell disorders.
They do however have to be careful when doing things where
there is less oxygen than normal such as scuba diving, activities
at high altitude and under general anaesthetics.
If both copies of the haemoglobin gene
carry instructions to make sickle haemoglobin then this
will be the only type of haemoglobin they can make and sickled
cells can occur. These people have Sickle Cell Anaemia and
can suffer from anaemia and severe pain. These severe attacks
are known as Crises. Over time Sickle Cell sufferers can
experience damage to organs such as liver, kidney, lungs,
heart and spleen. Death can be a result.
Another problem is that red blood cells
containing sickle haemoglobin do not live as long as the
normal 120 days and this results in a chronic state of anaemia.
In spite of this, a person with sickle cell disorder can
attend school, college and work. People with sickle cell
disorder need regular medical attention particularly before
and after operations, dental extraction and during pregnancy.
Many hospitals arrange follow-up appointments and it is
advisable to discuss with the doctors questions concerning
schooling, strenuous exercise, family planning, suitable
types of employment and air travel. When a person is found
to have a sickle cell disorder it is important that all
members of the family be tested. They will not necessarily
have sickle cell disorder but may be healthy carriers of
a sickle cell trait.
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