One in every 10 Jamaicans carries the gene for sickle-cell disease. For every 150 live births, one baby is born with sickle-cell disease. This means about 260 babies with sickle-cell disease are born in Jamaica each year.

Clearly, every man should know about this genetic disease and how it affects him, his family and community. The Sickle Cell Unit of the University of the West Indies recently published clinical-care guidelines for this critical disease. Here are some important facts about sickle-cell disease from the guidelines.

What is sickle cell disease?

Sickle-cell disease is a condition of red blood cells where the red pigment (called haemoglobin) causes the cells to acquire a sickle shape rather than the normal doughnut shape. These sickle-shaped red blood cells are easily destroyed, causing the sufferers to have weak blood (anaemia). The sickle cells clog tiny blood vessels resulting in pain, strokes in childhood and other complications.

How do you test for sickle-cell disease?

There is a simple screening blood test which identifies both the sickle-cell trait and sickle-cell disease. A further test using electrophoresis will define the genotype of the person's haemoglobin. A person with sickle-cell disease may have only the sickle genotype (SS disease) or the sickle genotype with some other abnormality. Every man should do this test in order to determine his 'sickle status'.

Screening for sickle-cell disease

Screening for sickle-cell disease occurs at birth at the Victoria Jubilee Hospital, Spanish Town Hospital and the University Hospital of the West Indies. Unfortunately, this only accounts for about 35 per cent of newborns in Jamaica. Thus, about 170 babies born with sickle-cell disease are missed at birth, which results in late identification of the problem.

How do you get sickle-cell disease?

Sickle-cell disease is a genetic disease inherited from both parents. If a man has the sickle-cell trait, he has a 50 per cent chance of passing the sickle genotype to his children. If he has sickle-cell disease, he will most certainly pass this genotype to his children

When a baby is born with the SS haemoglobin genotype; one S haemoglobin genotype has come from each parent. When the baby has one S haemoglobin and a normal (A) haemoglobin genotype, then the baby will have the sickle-cell trait. A man should know his 'sickle status', as well as the 'sickle status' of his sexual partner, since this will determine the likelihood of having children with sickle-cell disease.

Dr Pauline Williams-Green is a family physician and president of the Caribbean College of Family Physicians; email: yourhealth@ gleanerjm.com.