Sickle Cell Disease can be managed

Sickle cell is an inherited disease that affects the red blood cells. Red blood cells are cells that are responsible for carrying oxygen in the body.

Normal red blood cells are round, but people with sickle cell have sickle-like cells, which are not capable of passing through the normal blood vessels.

With reduced oxygen levels in the body, or infections, they tend to stick together, making them block blood vessels, and once that happens, blood is not able to pass adequately, in normal circulation, leading to pain.

In some cases, the red blood cells break down and change the colour of the eyes because of the release of haemoglobin, which is broken down to produce a substance called bilirubin, which is responsible for the yellowness of the eyes.

Sickle cell is acquired through inheritance. People who inherit one sickle cell gene and one normal gene have sickle cell trait.

If a carrier marries someone who is also a sickle cell carrier, there is a 50 per cent chance that any child of theirs also will have sickle cell trait, if the child inherits the sickle cell gene from one of the parents. Such children will not have symptoms of sickle cell disease, but they can pass the trait on to their children.

If both parents have the trait, there is a 25 per cent chance that any child of theirs will have sickle cell disease. There is the same 25 per cent chance that the child will not have sickle cell disease or the trait.

Last week, the Ministry of Health launched the neonatal screening guidelines in Vihiga County which is the bedrock of the disease. The various speakers, including the Director General for Public Health at the Ministry of Health Dr Patrick Amoth, encouraged the need for screening among partners before marriage.

In my opinion, this is very sensible and should be supported by all means necessary. In this day of modern technology with available rapid diagnostic kits, one should not take chances. These tests are now as simple as performing a pregnancy test. All one needs is a drop of blood for a test that can be done anywhere even at home.

Health is devolved and county governments must take responsibility to improve care for people with sickle cell disease. We encourage counties to buy a confirmatory haemoglobin electrophoresis machine that costs about Sh3 million.

The current mainstay in the management of sickle cell disease is the use of hydroxyurea which reduces the percentage of abnormal haemoglobin to a friendly form called foetal haemoglobin.

The drug is available in 500mg as a capsule which is very difficult for children to swallow. The drug is the common mode of treatment in Africa because the majority of patients are not able to undergo bone marrow treatment, which is the preferred mode of treatment globally.

In more advanced centres, there is exchange transfusion. Patients are put on a machine that removes a high percentage of abnormal sickle cells, a procedure that reduces symptoms and recurrence of more severe disease.

Finally, county governments and NHIF must finance the management of sickle cell disease and make services accessible in public facilities."