What's your sickle cell disease status?

Lesley King, Contributor

Public awareness of the sickle cell disease (SCD), as well as knowledge of individual carrier status, is poor. Also, persons and their families living with SCD are often disadvantaged due to misinformation about the disease.

Sickle cell disease is a genetic disease. Affected individuals inherit an abnormal gene from each parent resulting in the production of abnormal red blood cells (or sickle cells).

These abnormal red blood cells have a shortened life span, are destroyed rapidly in the circulation and can block blood vessels. Rapid red-cell destruction can cause anaemia (weak blood), jaundice (yellow eyes) and gallstones. Blocked blood vessels may cause bone pain, pneumonia, leg ulcers (sores) and strokes. These are just a few of the many problems associated with this disease.

Severe and mild forms

There are many forms of the disease. In Jamaica, one in 150 persons are affected by some form of the disease. If one inherits the sickle gene from both parents, the affected person has the most common as well as one of the most severe forms of the disease (that is, Hb SS disease). If one inherits the sickle gene as well as another abnormal gene (for example, C gene, Thalassemia gene), other forms of SCD may occur such as Hb SC disease and sickle thalassemia.

Hb SS disease is often referred to as 'full blown' which can lead to the misconception that other forms of the disease are not important. On the contrary, whereas Hb SS disease is a severe form, it is not the only severe form of the disease, and even so called mild forms of the disease can have many problems.

World Sickle Cell Day

Persons who carry a single sickle gene are called carriers. They have the sickle-cell trait. Carriers usually have no signs or symptoms and so are often unaware of their status. In Jamaica, 1 in 10 persons are carriers of the sickle gene and another 1 in 20 persons carries another gene which puts them at risk of having a child with SCD.

Since 2009, the United Nations has declared June 19 as World Sickle Cell Day to heighten awareness of the disease. Find out how this disease may affect you and your family today. Join us on Sunday, June 19 as we mark World Sickle Cell Day at Hope Botanical Gardens, Band Stand Lawn, in Kingston, 3 - 6 pm.

Dr. Lesley King is a Paediatrician and Clinical Research Fellow at the Sickle Cell Unit, Tropical Medicine Research Institute, University of the West Indies; email: yourhealth@gleanerjm.com