Sickle Cell Disease

Sickle cell disease is an inherited disease. It means that your parents must at least carry the disease for you to suffer it. It affects the red blood cells which are the cells in the blood that carry oxygen round the body. In serious cases it results in sickle cell anaemia (low blood count).

Sickle cell disease is common in the Africans.

It a lifelong condition but certain precautions and treatment can help people to live with it as long as possible.

The red cells in the sickle cell disease patients has abnormal shape and because of this

• they block the blood vessels
•  they do not live as long as the normally shaped blood red blood cell.

The things people with sickle cell disease complain about.

The problems of sickle cell disease could start in early childhood as early as six months of age.  

• Painful episodes of sickle cell crises- pains in different parts of the body (the bones, the joints, and the tummy)
• Severe anaemia (low blood count)- where the red cells are destroyed quickly, or they collect in the liver or spleen. The person would have tiredness, weakness, the white of their eyes turn yellow(jaundice) and they could even struggle to breath.
• The person would have increased chance of infections.
• The growth in some people could be delayed, delayed puberty, could have strokes, kidney, or lung problems. Some people have painful legs ulcers.

How do you get sickle cell disease?

Sickle cell disease is caused by inherited gene that make the abnormal red cells.

When both parents have the gene, there is 25% chance that each child they born could have sickle cell disease. It is important to know that not all their children are going to have the sickle disease.

 The parents of a person with sickle cell disease may not have the disease themselves but they would only be carriers- they carry the sickle cell trait.

How do you test for sickle cell disease?

 People could have test during pregnancy or test the baby soon after birth.

The tests during pregnancy could be carried out after discussion with your doctor. It is carried out as from 10 weeks of pregnancy.

 It is important to remember that the baby have 25% chance of having sickle cell disease only if both parents are carriers of the gene. If only one parent is a carrier, testing the baby would not be necessary.

  Pregnant women of mainly African descent are encouraged to have the test called haemoglobinopathy to find out if they are carriers. If they are carrier of sickle cell trait, then partner should be tested. If the partner is also a sickle cell carrier, then the test could be done during pregnancy or after birth if the parents decide to wait.

Life with sickle cell disease.

The sickle cell crisis is not only common but also very distressing. Its prevention could significantly improve the persons quality of life. It is not always clear what triggers the crisis. But certain measures could help prevent or at least reduce the frequency of sickle cell crises in people that suffer this condition.

• Drink plenty of fluids especially if you live in hot weather. Dehydration which is when the body loses too much of fluids increases the chance of crisis.
• Avoid extreme temperatures
• Avoid stress- learn to relax. Too much stress especially during exam times for young people could trigger sickle cell crisis.
• Avoid very strenuous exercise. You should be active and understand your limits. Also, for young people you can go to disco but should know to leave earlier to give your body a lot of rest.
• Avoid alcohol and smoking. Smoking can narrow your arteries making it easier for the abnormal shaped cell to block them. It could also trigger severe chest pains. Alcohol could lead to dehydration (loss of fluids) which should be avoided.
• Be careful at high altitudes- reduced oxygen higher up on hills and mountains could trigger sickle cell crisis. Travelling in a plane is not a problem because planes have steady oxygen supply.
• Avoid infections- clean water, good food and personal hygiene like washing your hands regularly after toilet. Where available regular vaccinations against common diseases like pneumonia, meningitis or even flu, regular antimalarial prevention, and some are put on daily antibiotics.

Treatment

People with sickle cell disease need treatment throughout their lives because it is lifelong condition.

 They need pain killers at times strong ones, especially during the sickle cell crises

 Hydroxycarbamide is a tablet that helps reduce severity and frequency of the problems sickle cell disease people have.

They may need regular blood transfusion which help improve the condition.

 Newer medical developments like stem cell or bone marrow transplant can cure sickle cell disease, but it has a lot of risks.

Most people can lead a normal and happy life if they learn and know how to live with the sickle cell disease.