Sickle cell disease is a complex inherited disorder of the red blood cells. This disorder was first described in 1910 and has a variable clinical course and at present has no universal cure.

Dr. Brett Hodge

What is sickle cell disease?
This is a group of inherited red blood cell disorders. Normal red blood cells are round like doughnuts and they move through small blood vessels in the body to deliver oxygen. Sickle red blood cells become hard, sticky and are shaped like sickles used to cut wheat. When these abnormally shaped cells go through the small blood vessels they clog the blood flow and break apart resulting in low blood count or anaemic and other complications.
There are several types of sickle cells disease. The most common are:
• Sickle cell anaemia (SS)
• Sickle-Haemoglobin C disease (SC)
• Sickle Beta-Plus Thalassaemia
• Sickle Beta-Zero Thalassaemia.
Sickle cell disease results from a disorder of the haemoglobin molecule. Haemoglobin is the main substance of the red blood cell and it helps the red blood cells carry oxygen from the air in our lungs to all parts of the body.
What is Sickle Cell Trait?
This is not the same as sickle cell disease. Sickle cell trait is an inherited condition where only one set of abnormal haemoglobin gene is inherited. People with sickle cell trait are generally healthy.

Who gets Sickle Cell Disease?
This disorder occurs predominantly in people of African descent but occurs in other people in parts of Europe, South America and the Middle East.

How do you get Sickle Cell Disease?
Sickle cell disease is inherited from your parents. Like most genes, the haemoglobin genes are inherited in two sets, one from each parent. If both parents have Sickle Cell Trait, they have a 25% chance (1 of 4) of having a child with sickle cell disease with each pregnancy.
You cannot catch sickle cell disease from someone who has it. Sickle cell disease is not an infectious condition.

How do you know if you have the sickle cell trait?
A simple blood test followed by a laboratory technique called electrophoresis will determine the type of haemoglobin you have. Individuals with sickle cell trait and sickle cell disease especially teenagers, should be offered genetic counseling as this may help to decrease the number of cases of sickle cell disease in the future.

Is Sickle Cell Disease present in Anguilla?
Yes! At present we do not know the exact incidence and prevalence of this condition in Anguilla. There are a number of individuals in Anguilla who have sickle cell disease and a much larger number who have sickle cell trait. In several Caribbean countries the incidence of sickle cell trait is 8 - 10% and Anguilla is believed to have a similar incidence.
What are some of the complications of sickle cell disease?
Individuals with sickle cell disease have a wide spectrum of clinical findings. Complications will vary from one person to the other. Complications include the following:
• Pain episodes (arms, legs, chest and abdomen)
• Stroke
• Painful prolonged erections in men (priaprism)
• Increased infections
• Leg ulcers
• Bone damage
• Early gall stones
• Eye damage
• Kidney damage
• Anaemia
• Delayed growth

Treatment
Early diagnosis of sickle cell disease is very important. The diagnosis can be made in the newborn period and in some countries in the prenatal period. Prevention of complications includes penicillin prophylaxis, routine vaccination and in addition pnuemococcal vaccination in children. Folic acid supplication is strongly recommended.

Treatment of complications includes pain management, re-hydration and blood transfusion. Psychosocial support is important as this condition is a chronic lifelong disease for many individuals.

New treatments for sickle cell disease include the use of Hydroxyurea and bone marrow transplant.

What can individuals with sickle cell do to improve their health?
Individuals with sickle cell disease should have regular medical check ups. They should also do the following:
• Drink plenty of water. Adults should use 8 - 10 glasses of water/day
• Avoid too hot or cold temperatures
• Avoid over exertion and extreme stress
• Get plenty of rest
• Take folic acid daily.

Like most chronic disorders increased patient education is vital for improved care. Support groups have also proven beneficial for individuals and families who have to deal with sickle cell disease.

Conclusion
Sickle cell disease is an inherited blood disorder that affects red blood cells. The sickle-shaped cells block small blood vessels causing less blood and oxygen to reach that part of the body. This results in a variety of complications that characterize this disorder. A number of factors have resulted in improved health for individuals with sickle cell disease, but in a few cases complications still occur and can be serious requiring intensive multidisciplinary professional care.

Ask Your Doctor is a health education column and is not a substitute for medical advice from your physician. Dr. Brett Hodge is an obstetrician/gynaecologist and family doctor who has over twenty years in clinical practice. Dr. Hodge has a medical practice in the Johnson Building in The Valley.

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