The Cayman Islands joins the United Nations in observing World Sickle Cell Day on Wednesday, 19 June 2019. Raising public awareness of the disease is the main objective of the day. Sickle cell disease is a chronic disorder with the condition present at birth. It is inherited when a child receives two sickle cell genes. At present, there are 41 persons with sickle cell disease known to the Public Health Department.
While screening for sickle cell disease in the Cayman Islands 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 the 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, with each pregnancy, there is a 25% risk of having a child with sickle cell disease,” said the Health Services Authority’s Genetics Coordinator Joy Merren.
A Sickle Cell Support Group meets three to four times a year. Patients and families receive information on management of the disorder as well as support. The next meeting is scheduled for Monday, 17 June, 2019 at 7:30pm at the Women’s Health Waiting Room. Dr Judith Nicholls will be speaking on “Approaches to Pain Management in Patients with Sickle Cell Disorder” and Dr Anna Matthews will be speaking on “Almost Sickle: The Sickle Cell Trait”.
The public is invited and light refreshments will be served.
For further information, please contact Mrs Merren 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.
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 down 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.
Persons with the trait can, under extreme conditions, have some symptoms, so it is recommended that persons with sickle cell trait:
- Avoid high altitudes
- Avoid scuba diving and free diving
- Drink adequate fluids to avoid dehydration
- Pace themselves when exercising/playing sports
- Rest as needed when exercising/playing sports
- Seek medical care right away if feeling ill when playing sports
- Seek medical attention if blood is noted in the urine (haematuria)
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
- Hydroxyurea for persons who meet the criteria
These are some of the management strategies. For further details, please consult your doctor.