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February 23, 2019

Sickle cell is a hereditary disease of the blood

Observe daily life around you and you will notice different behaviour. There are those rule following, law abiding types who patiently queue behind the person ahead with the hope that life is fair and that their turn will come.

Then there are those who appear from the left field, out of your sight of vision, looking to sneak in to that tiny safety gap you have left ahead of you just in case. Because our threshold for disobeying basic rules of society are quite low, it takes just one or two to do this to turn a fairly orderly process into a time delaying mess.

Africans often are proud to announce and they show it, that time is not an issue when it comes to development. But there are a few exceptions; those who have to differentiate themselves from the rest. The most powerful way to do this is to show that you do not wait for time.

Time, Einstein showed has a close relationship with light, so having a car with flashing lights and just in case Einstein did not work out the full formula, whopping sirens; gets such people to cut through traffic leaving the left fielders and those patiently waiting their turn to wish they had learned physics, ‘beyond what goes up must come down’.

Often the aggregate result of all these different types of behaviour is one. Everyone gets delayed because each segment of the population has its own rules of behaviour and it is never clear when the rules apply and when they do not.

On some days the left fielders win because only a few of them attempt and get away with it, on other days the sireners win because they manage to get a clear path ahead.

As time goes by and with more and more people commuting the chances of the law abiders getting through in predictable time without the other groups interfering become less and less. The mini-crises are many and every so often there is a full-scale crisis and gridlock.

The police and transport administration are required to reflect the face of Kenya. In a sense these scenes that feed our conversations are similar to the suffering that inflicts those with sickle cell anaemia. Sickle cell anaemia is a hereditary disease of the blood, which is found almost exclusively in black Africans. A mutation in the gene that instructs the body how to make haemoglobin, the compound that carries oxygen and gives blood its red colour, means that those with sickle cell do not produce enough normal haemoglobin.

The pattern of inheritance is autosomal recessive, which means a child has to get two defective genes, one from each parent in order to have the full-blown disease.

A person with one normal and one abnormal gene is said to have sickle cell trait and does not generally have any symptoms. But if two people with the trait have children, each child has a 25 per cent chance of having sickle cell, 25 per cent chance of being normal and 50 per cent chance of being a carrier like their parents.

In the first four months of like while the baby still has blood cells from the mother the baby will be just like any other child, but thereafter symptoms of the disease will begin to appear.

Symptoms include shortness of breath, frequent infections and pain. This is because the defective red blood cell, which under a microscope looks like sickle or a moon crescent, cannot carry enough oxygen to various tissues. The defective cells, because of their odd shape, behave life left fielders in traffic, blocking off access for the good cells. When this happens in the spleen, infection control is compromised. Eventually these blockages and lack of oxygen cause a sickle cell crisis, when the patient has an extreme pain because the sickle cells have totally blocked off blood flow to some part of the body. When this happens in a blood vessel to the brain, a stroke follows and the patient may even die.

A growing child has ever increasing need for oxygen and so sickle cell disease unmanaged leads to problems in milestones. A child with sickle cell will not grow as tall, as healthy as a normal child. They spend many days in pain and in and out of hospital. The only potential cure for sickle cell anaemia is a bone marrow transplant, a risky and expensive procedure that can even kill the patient. Finding a donor is difficult.

So symptomatic treatment and avoidance of a crisis is the goal of medical management. Children with sickle cell anaemia take penicillin antibiotics continuously and they are often of pain medication as well.

A compound hydroxyurea reduces the frequency of painful crises possibly by stimulating the body to continue producing foetal haemoglobin, the kind found in newborns and which has a very high affinity for oxygen when compared to adult haemoglobin. But in the end life expectancy is reduced.

At least we recognise that the disease is a genetic accident, not so when it comes to learned behaviour.

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