Things to know about Sickle Cell Anaemia

Ministry of Health, Wellness and the Environment – Antigua and Barbuda will join the World Health Organization (WHO) and the Pan American Health Organization (PAHO) along with the rest of the region in observance of World Sickle Cell Day tomorrow Saturday 19th June 2021.

Sickle-cell disease (SCD) is a blood disorder which alters the shape of the red blood cells. Instead of being round and flexible, they appear half-moon-shaped, and are brittle and sticky. The disease makes the cells shred rapidly, causing severe anaemia and blockage of small blood vessels.

When blood vessels are blocked, this prevents the flow of blood, oxygen and nutrients to the tissue, causing unbearable pain and organ damage. This also predisposes sickle-cell patients to serious infections.

Some persons have inherited only the sickle-cell trait, so they are referred to as trait carriers. A person is diagnosed with SCD after inheriting two abnormal genes, but those with the trait obtained one normal and one abnormal gene. 

If both parents have the sickle-cell trait, there is a 25% chance that children born to them can have SCD and a 25% chance these children can be normal. If one parent has the trait and the other has SCD, there is a 50% chance the children will have the disease and/or a 50% chance of them being carriers.

A normal person with a partner who is a trait carrier has a 50% chance of producing carriers and a 50% chance of producing normal children. Carriers of the sickle trait may display symptoms of SCD while most are asymptomatic.  

Symptoms of sickle-cell disease include anaemia in which patients can become dizzy, easily exhausted or experience headaches. Patients sometimes become pale with yellowish eyes. The most prominent symptom of SCD is pain crises as a result of blockage of the tiny blood vessels. These crises require emergency medical attention and hospitalisation.

Pain intensity differs for those in a sickle-cell crisis with a duration of a few hours to a few weeks. Some SCD patients have only a few pain crisis annually, while others find themselves having more than a dozen each year.

SCD manifests itself in infancy usually between the ages of 5-6 months. In a normal body, the life span of red blood cells is around 120 days. But in those with sickled cells, the cells die within 10-20 days. As a result, the body is left with a shortage of red blood cells, causing anaemia.

People with sickle cell anaemia have frequent infections due to impairment of their spleens. Children and infants with sickle cell are generally vaccinated against specific pathogens and given antibiotics upon infection.

Toddlers with SCD  may experience painful, swollen fingers or toes with severe anaemia. An exceptionally low blood count will put patients at risk for devastating complications.

Regular eye examinations are a necessity in SCD as sickling can cause damage to the retina and ultimately lead to blindness. Additionally, leg ulcers may occur due to poor blood flow. Severe, long-term complications of  sickling causes damage to the hip joints which eventually requires hip replacement surgery.   

Splenic sequestration or blood pools in the spleen is a life-threatening condition associated with SCD. It is commonly found in children and causes the child’s spleen to expand as it reduces the amount of oxygen-carrying red blood cells in the body. 

Presently, a blood and bone marrow transplant is the only cure for sickle-cell disease. However, there are treatments that are effective in reducing symptoms and extending patients’ lives. 

Some treatment options for SCD are: daily folic acid to produce red blood cells; penicillin prophylaxis for children; non-routine vaccines (particularly Prevenar-13) for children and adults; along with hydroxyurea, an oral medication to reduce the number of pain crises and complications. 

For those with SCD, their lifestyles are contributing factors. Therefore, they need to avoid alcohol use, smoking and exertion. Getting enough rest, plenty of fluids and regular check-ups are compulsory as well.

A sickle-cell diagnosis increases one’s risk of becoming extremely sick with COVID-19. Knowing your sickle-cell status is important – get tested. 

For those seeking additional information or help, contact your community clinics or the Antigua and Barbuda Sickle-cell Association. 

Special thanks to Dr. Edda Hadeed for her wealth of information and willingness to share.

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