Sickle cell day of recognition

(CNS): With 33 people in the Cayman Islands suffering from sickle cell disease the jurisdiction is joining the United Nations in observing the first annual Sickle Cell Disease World Day on Thursday, 19 June. The worldwide event is intended to 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. Health Services Authority Genetics Coordinator Joy Merren explained that Cayman is lucky to be able to both diagnose and manage the disease for patients here.

“We are fortunate to have facilities to diagnose sickle cell disease and to manage it,” said Merren. “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.

She added that there is also 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. It 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,” Merren explained. “ 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.”

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

Sickle cell disease is an inherited chronic disorder that affects red blood cells 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 and are not able to squeeze through the small blood vessels, so the 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. 

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 don’t develop the disease but a blood test can be done to determine if a person has the trait. 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 being a carrier. If oly 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 but no chance of the child having the disease.

Symptoms include 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 and infections may develop, such as pneumonia. Leg ulcers may also develop due to less oxygen to the lower legs.