HEALTH - SICKLE CELL DISORDER

What is Sickle Cell Disorder?

Sickle cell is an inherited blood disorder, which affects the red blood cells. Each red cell in the blood contains a protein called haemoglobin. (HbA). The function of the haemoglobin is to transport oxygen from the lungs to all the issues in the body. The red cells containing the usual haemoglobin HbA is round and will remain round and flexible after the oxygen is taken up by the tissues, this roundness enables them to flow easily through the tissue even after the oxygen has been given up.

If a person has inherited or has mostly sickle haemoglobin (HbSS) after giving up its oxygen, the sickle haemoglobin will change shape to that of a sickle (or half moon shape), which will become rigid and unable to flow easily through the tissue.

Inheritance

Two genes determine the haemoglobin a person inherits, one from each parent. For example a person who inherited HbA from both parents will have the usual HbA haemoglobin. A person who inherits the usual haemoglobin Had from one parent and haemoglobin HbS from the other parent, will have 'Sickle Cell Trait' (HbS) and a person who inherits haemoglobin HbS from both parents will have sickle cell haemoglobin (HbSS) also know as Sickle Cell Anaemia.

Sickle Cell Haemoglobin

Sickle cell haemoglobin does not live as long as the usual haemoglobin, which has a life of about 120 days. As a result it is believed that people with sickle cell trait are protected or has protection from malaria.

What is Sickle Cell Trait?

Sickle Cell Trait occurs when a person inherits the sickle haemoglobin HbS from only one parent and the usual haemoglobin from the other parent. They will have haemoglobin HbAS. People with the trait are known as 'carriers' - they do not suffer with sickle cell disorders or any ill effects, but they may pass the sickle gene HbS on to their children.

Whom does Sickle Cell Disorder affect?

People whose origins are Africa, the Caribbean, Asia, the Middle East and Eastern Mediterranean.

What problems are associated with Sickle Cell Disorders?

Pain, tiredness, swelling of the arms or legs; jaundice.

Where do people with Sickle Cell disorder experience pain?

Pain can occur anywhere in the body- in the hands, arms and legs, chest, abdomen (tummy or stomach), or head. It can last for hours, days or weeks.

When do people with sickle cell feel pain?

When they have an infection, don’t drink enough and become dehydrated, overexertion-too much exercise or games, getting too cold in cold weather, sometimes after swimming, eating or drinking too many cold things.

How can parents tell when children are in pain?

• The whites of the eyes may turn yellow (this is called jaundice).
• Children may not be drinking enough for the day.
• Children may be irritable and more tired than usual.
• If the parents are taught how, they may be able to feel if the child has an enlarged spleen. As this indicates that the child has pain.

Important points to remember in managing Sickle Cell Disorder.

• Take penicillin and folic acid as prescribed by your doctor.
• Keep warm in cold weather.
• Drink plenty of fluids – at least 3 litres a day.
• Do not overexert yourself too much.

Things to remember

• Two genes determine the haemoglobin you inherit-one from each of your parents.
• You cannot inherit Sickle Cell disorder from only one parent- you will only inherit the Trait.
• You cannot catch Sickle Cell Disorder like you can i.e. chickenpox; it is not an infection.

Further information about Sickle Cell Disorder can be obtained from www.sicklecellsociety.org