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Sickle Cell Disease

This US National Institutes of Health image shows how sickle cells can impede smooth blood flow.

The Cayman Islands joins the United Nations in observing Sickle Cell Disease World Day on Saturday, 19 June 2010.

In December 2008, the United Nations General Assembly adopted a resolution that recognizes sickle cell disease as a public health problem, and the Sickle Cell Disease World Day was first observed in 2009. Raising public awareness of the disease is the main objective of the day. 2010 is the 100th anniversary of sickle cell disease being recognized as a separate disorder.

At present, there are 36 persons in the Cayman Islands with sickle cell disease, known to Public Health Department. In the Cayman Islands, we have facilities to diagnose sickle cell disease and to manage it. 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. Sickle cell testing is offered to all newborns in the Cayman Islands.

There is a peer support group, the Sickle Cell Support Group, where family and patients come together to share experiences in coping with the disease, and educational sessions are organized for awareness and management of the disease. This group meets 3 to 4 times a year and is supported by the Public Health Department.

"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. Knowing ahead of time can help couples make informed reproductive choices. While sickle cell trait is mild, sickle cell disease is serious and can potentially affect every organ of the body", said Mrs. Joy Merren, Genetics Coordinator at the Health Services Authority.

Patients with sickle cell disease can live lives optimally as they work together with family and health care workers in managing this disorder.

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.