BY ROBERT FRASER RN, BS
“You don’t make progress by standing on the sidelines, whimpering and complaining.
You make progress by implementing ideas:” –
Shirley Chisholm (US Congress woman, 1977- 1981)
“It is always better to have information and not need it, than to need it and not have it.” –
Edgar Henry, MBA
What Causes Sickle Cell Disease and Sickle Cell Trait?
Sickle Cell Disease (SCD) is a genetic condition that is present at birth. It is inherited when a child receives two sickle genes – one from each parent. A person with SCD can pass the disease on to his or her children.
Sickle Cell Trait (SCT) is not a disease, but it means that a person has inherited the Sickle Cell gene from one of his or her parents. People with SCT usually do not have any of the symptoms of SCD and live a normal life, but they can pass the Sickle Cell gene on to their children.
- When one parent has SCT, he or she has a 25% chance of having a child with SCD with every pregnancy.
- When both parents have SCT, they have a 50% chance of having a child with SCT with every pregnancy.
Who is affected by Sickle Cell Trait?
Sickle Cell Trait (SCT) affects one in twelve Blacks or African Americans in the United States of America (U.S.A.):
- SCT is most common among Blacks or African Americans, but can be found among people whose ancestors come from Sub-Saharan Africa; the Western Hemisphere (South America, the Caribbean and Central America); Saudi Arabia; India and the Mediterranean countries such as Turkey, Greece and Italy
- Approximately three million (3,000,000) people living in the U.S.A. have SCT and many are unaware of their status.
It is reported by the Pan American Health Organization (PAHO) that 3.3% of the population of Guyana is affected by Sickle Cell.
I invite readers to pay attention to the ‘Likelihood of the offspring’s Genotype.’ Since a child inherits one gene from each parent, you have to multiply the probabilities: the outcomes of all the different possible mating are most conveniently presented as table.
| PARENTAL GENOTYPES | LIKELIHOOD OF AN OFFSPRING’S GENOTYPE | ||
| AA | AS | SS | |
| AA x AA | 100% | 0% | 0% |
| AA x AS | 50% | 50% | 0% |
| AA x SS | 0% | 100% | 0% |
| AS x AS | 25% | 50% | 25% |
| AS x SS | 0% | 50% | 50% |
| SS x SS | 0% | 0% | 100% |
The best way to find out if and how SCD runs in someone’s family is for that person to see a Genetic Counselor. These professionals have experience with genetic blood disorders. They also specialize in prenatal genetic counseling.
The Genetic Counselor will look at the person’s family history and discuss with him or her what is known about SCD in the person’s family. It is best for persons with SCD or SCT to learn all he or she can about SCD before deciding to have children.
A woman and her partner should get tested for SCT if they are planning to have a baby.
- Testing is available at the hospitals or prenatal clinics or at local health centers.
- If a woman or her partner has SCT,
- A Genetic Counselor can provide additional information and further discuss the risks to their children.
- Pregnant women with SCD and SCT should have prenatal test done to find out if the baby would have the disease or carry the trait. These tests usually are conducted after the second month of pregnancy.
- With early prenatal care and careful monitoring throughout the pregnancy, a woman with SCD can have a healthy pregnancy. However, women with SCD are more likely to have problems during pregnancy that can affect their health and that of their unborn baby. Therefore, they should be seen often by their Obstetrician, Hematologist or Primary Care Provider (PCP).
- During pregnancy, SCD can become more severe and pain episodes can occur more frequently.
- A pregnant woman with SCD is at a higher risk of pre-term labor and of having a low birth weight baby.
Women who have SCT can have a healthy pregnancy. They should also be monitored by their Obstetrician or Primary Care Provider for the same health complications as for all pregnant women.
The author of this presentation is a graduate from the New Amsterdam School of Nursing and has practised nursing in New York within both clinical and administrative areas in reputable teaching institutions.
He is also involved in an on-going research in ‘Sickle Cell Anemia in family.’
The aim of this column is to ‘Promote knowledge and dispel ignorance.’ The ultimate goal is to prevent Sickle Cell ailment (SCD) in generations yet unborn.
The author also conducts in-service education for nurses and teachers in Regions Five (5) and Six (6) annually.
The purpose for involving the latter group (high school teachers) in this discussion is for them to impart this important health knowledge/information to the seniors (Graduating students).
In so doing, the teachers will be reminding the students that they are now of age when they could become parents and would be incumbent upon them to understand how Sickle Cell Disease (SCD) is inherited.
