World Sickle Cell Day – June 19
The Cayman Islands again joins with the United Nations in recognizing June 19 as World Sickle Cell Day. The purpose of this day is to raise awareness of sickle cell disease as a public health concern.
While screening for sickle cell disease has been in place since the early 1970s for high risk families, and since the 1980s for school entry screening, routine newborn screening has been in place since 1997. This has helped in early identification of sickle cell trait and disease with appropriate counseling and management.
“It is important to test for sickle cell trait- as this information can help parents make informed reproductive choices. If someone has sickle cell trait, it is important to know if one’s partner is also a carrier. If both parents are sickle cell carriers, then with each pregnancy, there is a 25% risk of having a child with sickle cell disease. While sickle cell trait is mild, sickle cell disease is serious”, said Mrs. Joy Merren, Genetics Coordinator at the Health Services Authority.
At present, there are 47 persons with sickle cell disease known to Public Health Department. Sickle cell disease is a chronic disorder. In the Cayman Islands, we have facilities to diagnose sickle cell disease and to manage it.
A Sickle Cell Support Group meets four times a year. Patients and families receive information on management of the disorder as well as support. The next meeting is on June 23, 2015 from 7:30pm to 9:30pm. Anyone interested is welcome; the group meets at the Public Health Waiting Room.
For further information, please contact Mrs. Joy Merren, Genetics Coordinator, on 244-2630 at the Public Health Department.
What is Sickle Cell Disease?
Sickle cell disease is an inherited chronic disorder that affects red blood cells. It has a worldwide distribution, and is one of the most common genetic disorders.
All persons have two genes that make haemoglobin. Normal red blood cells contain haemoglobin A, a protein that helps red blood cells carry oxygen around the body. With sickle cell there is a different form of protein, haemoglobin S. With sickle cell disease, both genes are affected, causing severe symptoms.
Normal red blood cells are round, flat and very flexible. However, when the oxygen comes out of the red blood cells of sickle cell disease, the cell becomes stiff and takes on the shape of a sickle – hence, the name. The sickle cells clump together, are not able to squeeze through the small blood vessels, and so the sickle cells get destroyed more quickly. A normal red blood cell lives approximately 120 days but a sickle cell may only live 11 or 12 days.
What is Sickle Cell Trait
When only one gene is affected, it is called sickle cell trait, or persons are called sickle cell carriers. Having sickle cell trait means that the person stays healthy under normal circumstances, and the main significance is that it can be passed on to one’s children. However, under certain extreme circumstances, a person with the trait may experience complications as if having sickle cell disease. Persons with just the trait CANNOT later develop the disease.
How to Find out if you have sickle cell trait?
A blood test can be done to determine if a person has the trait.
How is Sickle Cell Disorder Inherited?
If a man and a woman are both sickle cell carriers, with each pregnancy, there is a:
- 25% chance of the child having the disease;
- 25% chance of the child being completely free from sickle cell;
- and a 50% chance of the child having the sickle cell trait, i.e., being a carrier
If only one parent is a carrier, then there is a
50% chance of the child having the trait;
50% chance of the child being completely free of sickle;
and NO chance of the child having the disease.
What Symptoms Are Associated with Sickle Cell Disease?
- Anaemia, jaundice and gallstones due to rapid breakdown of the red blood cells
- Painful swelling of fingers and toes in babies
- Painful attacks of joints, back and abdomen as there may be damage to the bone marrow
- Infections may develop, such as pneumonia
- Leg ulcers may develop due to less oxygen to the lower legs
Management of Sickle Cell Disease
- Full recommended immunizations plus pneumococcal vaccines
- Penicillin, starting from about 2 months of age to 5 years of age to help prevent serious infection
- Management of symptoms by using medications as needed
- Folic acid daily to help make new red cells
These are some of the management strategies. For further details, please consult your doctor.