Sickle cell disease

What is sickle cell disease?

Sickle cell disease (SCD), is the name for a group of inherited health conditions which cause the body to produce abnormally shaped red blood cells.
There are several types of the disease, the most common being:

Haemoglobin SS
Haemoglobin SC
Haemoglobin S beta thalassemia
Haemoglobin SD, Haemoglobin SE, and Haemoglobin SO

The most serious type is Haemoglobin SS, commonly called sickle cell anaemia which is passed on when a child receives one sickle cell gene from each parent.

Who is more likely to get sickle cell disease?

Sickle cell disease is particularly common in those with people of African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian origin.

Approximately 100,000 Americans suffer from SCD, and in Cayman there are 41 persons known to the HSA’s Public Health Department who have the four most severe types of sickle cell disease.

What are red blood cells and how does SCD affect them?

Blood contains white blood cells, red blood cells and platelets. Red blood cells contain a protein called haemoglobin, which carries oxygen from the lungs to tissues throughout the body as well as carrying carbon dioxide waste back to the lungs for excretion.

Healthy red blood cells contain haemoglobin A and are soft, flexible and round, while RBCs in those suffering from SCD contain mostly haemoglobin S and are stiff and C-shaped like a sickle, hence the disease’s name. These RBCs die earlier than healthy RBCs and have difficulty navigating small blood vessels, becoming stuck and obstructing blood flow.


What symptoms can SCD sufferers experience?

SCD sufferers begin to show symptoms during their first year of life, usually around 5 months old, and the disease worsens over time.

The reduction in the number of healthy RBCs carrying oxygen, as well as the sickle-shaped cells blocking small blood vessels and the subsequent reduction in blood flow to tissues causes a variety of complications including:

• Pain
• Anaemia
• Hand-foot syndrome (swelling and fever)
• Frequent infection
• Delayed growth
• Acute chest syndrome
• Gallstones
• Bone and joint pain
• Vision loss
• Leg ulcers
• Strokes or transient ischaemic attacks
• Deep vein thrombosis and pulmonary embolism
• Swelling of the spleen

What tests and treatments are available?

Newborns are tested for sickle cell in Cayman, as has been routine since 1997. Early diagnosis of the condition is important due to the increased risk of infection and other health issues that are associated with it.

Various treatments are available to reduce complications and lengthen lives, such as antibiotics, pain relief, and hydroxyurea. Stem cell/bone marrow transplants are a possible cure for SCD but also bring with them challenges and risks and are not a guaranteed cure.

Are there any local resources for SCD sufferers?

The Public Health Department at the HSA hosts a Sickle Cell Support Group three to four times a year. For more information on this please contact the HSA genetics coordinator Joy Merren at [email protected] or phone 244-2630.

For more information on sickle cell disease, visit the following sites: