Download Sickle Cell Anaemia - Women`s and Children`s Hospital

Sickle Cell Anaemia
What is haemoglobin?
Haemoglobin is found in red blood cells. It contains iron and
carries oxygen around the body.
What is anaemia?
Anaemia occurs when there are very few red blood cells in the
body. This means there is not enough blood cells to carry the
amount of oxygen needed by the body. When a child has
anaemia they become pale and more tired than normal.
What is sickle cell anaemia?
Sickle cell anaemia occurs when haemoglobin in red blood cells
is not normal.
Normal haemoglobin (HbA) makes the blood cells round, soft
and easy to move about even in very narrow blood vessels.
Haemoglobin in sickle cell anaemia (HbS) is different. It makes
the blood cells a crescent moon shape, hard, rigid and difficult
to move around the blood vessels. The blood cells break easily
because of their shape causing haemoglobin to spill. They get
stuck in narrow blood vessels causing blockage. They can also
pool in some organs of the body e.g. spleen, causing
enlargement and damage.
(Current Health, 1997)
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What causes sickle cell anaemia?
Sickle cell anaemia is caused when a person receives two
sickle genes from their parents.
One gene is from the mother the other from the father. The
gene tells the body to make abnormal haemoglobin.
What is a gene?
Genes give people their characteristics e.g. hair colour height
etc. Half of your genes come from your mother and half from
your father.
What happens if the person receives only one sickle gene?
If a person receives only one sickle gene they will not have
sickle cell anaemia. The person will be a carrier meaning that
they could pass the gene onto their children.
Sickle gene = S
Normal gene = A
(Sickle Cell Society 2004)
What are the signs and symptoms of sickle cell anaemia?
Signs and symptoms can be mild to severe.
Children may experience the following:
• Infections - due to damage to the spleen by the sickle cells.
The spleen is important in fighting infections. When spleen cells
are damaged they are unable to destroy bacteria in the blood
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making the child more susceptible to infection. Infections are
the most common symptom in infants and young children
• Pain - can vary in length and severity and is due to blockage
of oxygen flow to the tissues. It is often one of the first
symptoms to present. It can occur frequently, with bone pain the
most common site.
• Swelling and pain in the hands and feet - due to blockage of
oxygen flow to the tissues. More common in children under 5
years of age and known as hand-foot syndrome.
• Tiredness, paleness and shortness of breath - due to anaemia
• Yellow skin and eyes - due to rapid breaking down of red
blood cells which causes the jaundice
• Eye problems - due to lack of nourishment as a result of
decreased red blood cells Regular eye examinations usually
start around 5 years of age.
• Strokes - due to blockage and damage of vessels, occur
spontaneously
• Chest problems - due to trapped sickle cells and or infection.
More common in older children.
What is the treatment?
There is no cure for sickle cell anaemia but the signs and
symptoms can be treated.
Treatment depends on the severity of the disease and may
include:
• Pain relief -paracetamol or intravenous e.g. morphine,
• Intravenous fluids - to aid in unblocking blood vessels
• Blood transfusions - to increase the number of normal blood
cells in the body. If regular blood transfusions are needed then
another drug called Desferal is also given to prevent iron
overload.
• Antibiotics - to prevent and treat infections
• Good nutrition - to keep your child healthy
• Good hygiene - to minimise infection
• Resting - to help with fatigue
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• Chest physiotherapy - to aid in preventing infection and lung
complications
• Regular GP check ups - to monitor your child’s progress
• Immunisation - to decrease infections
References
1997, 'It's in the blood sickle cell anaemia and other blood
disorders', Current Health (online) vol.21 (accessed 19/5/04)
Sickle Cell Disease Association of America, Inc.:
www.sicklecelldisease.org/
KidsFirst for Health: illnesses – Sickle cell Anaemia: (Great
Ormond St Hospital)
http://www.goshkids.nhs.uk/kids/health/illnesses/index.html
http://www.sicklecellsociety.org/sicklescene/pshomf.htm
http://www.sicklecellkids.org/
http://www.nih.gov – NIH – Health Information click Health then
find topic under alphabetical listing
Please refer to section 7 “Sources of More Information” or
check the parent’s library in McGuiness & McDermott children’s
clinic for further information.
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