First Sickle Cell Disease Day
The Cayman Islands joins the United Nations in observing the first annual Sickle Cell Disease World Day on Thursday, 19 June.
The worldwide event will raise awareness of the disease, and follows a United Nations General Assembly resolution adopted in December 2008 that recognizes sickle cell disease as a public health problem.
According to Public Health figures, there at present 33 persons in the Cayman Islands with sickle cell disease.
"We are fortunate to 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," explains Health Services Authority Genetics Coordinator Joy Merren.
"We also have 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," she continues.
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 Merren at 244-2630.
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. Persons with just the trait CANNOT later develop the disease.
How do I know if I 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
- 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.
- 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
Managing the disease - some strategies:
- 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
(Please note: These are some of the management strategies. For further details, please consult your doctor.)
Source: Public Health Department.