(CMR) Today, the Cayman Islands joins the United Nations in observing World Sickle Day which is observed annually on June 19. The main objective of World Sickle Cell Day is to raise public awareness of sickle cell disease, a chronic disorder present at birth that is inherited when a child receives two sickle cell genes.
Each year, the Public Health Department recommends persons with sickle cell get the flu shot as the disorder is an underlying disease and could result in complications. For this same reason, considering COVID-19, the Health Services Authority recommends that persons aged 12 years and older with sickle cell disorder receive the COVID-19 vaccine.
“Eating healthy whole foods, getting adequate sleep, exercising within one’s own limits (remembering to pace oneself), finding time to relax and laugh and finding a safe person to speak with when times are difficult all help in protecting the body from infectious and environmental insults. The old adage, “Prevention better than cure”, holds true in this situation”, said Joy Merren, Genetics Coordinator at the Health Services Authority.
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 the early identification of sickle cell trait and disease with appropriate counselling and management. Currently, there are 45 persons in the Cayman Islands with sickle cell disease known to the Public Health Department.
“It is important to test for sickle cell trait as this information can help parents make informed reproductive choices. If someone has the 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,” Mrs Merren stated.
A Sickle Cell Support Group meets three to four times a year. The next meeting is set for Tuesday, June 22, 2021, at 5:30 pm at the Women’s Health Waiting Room at the Health Services Authority, main campus. Dr Anna Matthews will lead the discussion. All are invited.
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, potentially 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 freediving
-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)
-Do a yearly urine dipstick for blood throughout life
-Inform physician/laboratory technician that you have sickle cell trait when doing HbA1C test for diabetes as some methods for checking this can give inaccurate results if a person has sickle cell trait
-Inform physician/anaesthetist that you have sickle cell trait/disease when having general anaesthesia
How to find out if you have sickle cell trait
A blood test can 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 the 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 a 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