Jennifer Knight-Madden, Contributor

One in 250 babies born in Jamaica has Sickle Cell Disease (SCD), a group of inherited blood disorders characterised by the predominance of sickle-shaped red blood cells. One in every 10 persons has the sickle cell trait.Most people in Jamaica have family members, friends or colleagues who are affected.

Common fallacies about SCD and the truths are listed below:

1. Fallacy - Only persons of African descent have SCD.

Truth - SCD became common in areas around the equator including areas of Africa, India and the Mediterranean. Thus this condition is also seen in Asians and Caucasians.

2. Fallacy - If your 'sickle test' is negative you can't have a child with SCD.

Truth- Persons who have another abnormal haemoglobin gene can have a child with SCD if their partner has the sickle cell trait.

3. Fallacy - Sickle Cell Disease cannot be prevented.

Truth - If persons who have an abnormal trait or SCD choose not to have children with other persons similarly affected, the birth of children with SCD can be decreased.

4. Fallacy - People with SCD will all die young.

Truth - The median (average) age at which people in Jamaica with SCD die is approximately 55 years.

Treatment methods

Important treatment methods which have contributed to improved survival include:

1. Education. Knowledge of the disease can prevent or minimise the effects of some complications.

2. Newborn screening. This can identify children with SCD before they have problems. Currently babies born at the University Hospital of the West Indies, Victoria Jubilee and Spanish Town Hospitals are screened for SCD.

3. Immunisation. Individuals with SCD are more prone than unaffected persons to some infections caused by particular bacteria. Immunisations are available against two such bacteria, Streptococcus pneumoniae and Hemophilus influenza B.

4. Preventative antibiotics. Children with severe forms of SCD are kept on penicillin constantly until at least four years of age to prevent severe Streptococcus pneumoniae infections.

Treatment options which can more radically change the course of SCD include:

1. Bone marrow transplantation. This treatment option is unavailable locally. Most patients in whom this is contemplated have no appropriate donor.

A small percentage of patients die. In some patients, the procedure is unsuccessful as the transplant does not 'take' while in others, there are transplant-associated illnesses and the need to take medications to weaken the body's immune system for an extended period of time.

When successful, the patient no longer has SCD. This process is also quite expensive and requires the patient and a family member to relocate for at least six months.

2. Hydroxyurea. Hydroxyurea has been found to decrease the frequency of painful crises and acute chest syndrome (similar to pneumonia), two of the most common and distressing complications of SCD. This drug is being used in selected cases in Jamaica.

All Jamaicans should ensure that they know whether they are at risk of having a child with SCD and seek to learn more about the disease. We should work towards universal newborn screening. All available treatment modalities should be used to decrease complications and further improve survival.