Sickle Cell Awareness
The Cayman Islands once again joins the United Nations in observing World Sickle Cell Disease Day on Sunday, 19 June in an effort to raise awareness about the world's most common genetic disorder.
According to Public Health statistics, there currently are 41 people affected by sickle cell disease in the Cayman Islands. These patients do sometimes get painful crises because of damage to the bone marrow. It is a chronic disease, and management of sickle cell disease is treatment of symptoms and learning to live or cope with the help of health care professionals.
Screening for sickle cell disease started in the Cayman Islands in the early 1970s for high risk families. Since the 1980s all students are tested as part of their school entry screenings, and in 1997 routine newborn screening was put in place.
"This has greatly helped in early identification of sickle cell trait and disease - which is important so people can access the appropriate counseling and treatment available," noted Genetics Coordinator Joy Merren.
She further explained: "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 and can potentially affect every organ of the body."
"We have a Sickle Cell Support Group which meets 3 to 4 times a year that offers support to patients and families alike as they share experiences in coping with sickle cell disease. Also included are educational presentations on awareness and management of the disease," Mrs. Merren added.
The next meeting is planned for Tuesday, 21 June 2011 at 7:30 p.m. in the Public Health Waiting Room at the Cayman Islands Health Services Authority and is open to the public. For further information, please contact Mrs. Merren on 244-2630 at the Public Health Department.
About Sickle Cell Disease
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.
Diagnosing 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.
Sickle Cell Disease symptoms
- 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.
Source: Public Health Department