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Sickle cell disease (SCD) is an inherited blood disorder. That means it is passed down from a parent’s genes. It causes the body to make abnormal hemoglobin. Hemoglobin is the protein in red blood cells that carries oxygen to all parts of your body. When you have SCD, your body’s tissues and organs do not get enough oxygen. Sickle cell disease is a lifelong condition.
Healthy red blood cells are round and move easily all over the body. With SCD, the red blood cells are hard and sticky. They are shaped like the letter C (and like a farm tool called a sickle).
These damaged red blood cells (sickle cells) clump together.
Sickle Cell Traits versus Disease
People who have sickle cell trait inherited a hemoglobin S gene from only one parent. They inherited a normal gene from their other parent. People with sickle cell trait typically do not have any symptoms of sickle cell disease. However, ongoing research may show that these people
may have symptoms. Importantly, they can pass on the abnormal gene to their own children.
The Sickle Cell trait is more common than we realize with about 10% of the Jamaican population living with the condition (HbAS). As carriers, this can affect how many children are born with Sickle Cell Disease.
World Sickle Cell Awareness Day is held annually on June 19, in order to help increase public knowledge and raise awareness of blood diseases. This year’s theme is: Closing the Survival Gap: Equity in Sickle Cell Disease. The theme represents a global shift from simple disease awareness to demanding actionable healthcare equity. The meaning centers around four core pillars:
Addressing the Survival Gap: This core pillar is critical in addressing the inequalities in life expectancy and survival outcomes for sickle cell disease patients globally.
Equal Access to Care. This pillar highlights the need [for] high standards of diagnosis, pain management, and specialized medicines are available to all patients globally.
Action over Awareness: This core principle is geared to challenging healthcare systems and governments to dismantle structural barriers rather than only engaging in educational campaigns.
Empowering Communities: The final pillar is a call to action for better support for young voices and patients advocates so they can navigate and confidently manage their health.
Objectives of World Sickle Cell Day
Among the objectives are increasing awareness of community, of the global burden of SCD disorder, encouraging health institutions to pay attention to sickle cell anemia through developing health programmes at the national level, and operating specialized centres to facilitate access to treatment. Additionally, it is hoped that this important day will raise awareness of the importance of pre-marital screening to reduce transmission of sickle cell disease among generations, and finally, promoting and supporting research to improve quality of life for those affected.
Symptoms
Symptoms of sickle cell anemia usually appear around 6 months of age. They vary from person to person and may change over time. Symptoms can include: Episodes of Pain. Periodic episodes of extreme pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen, and joints. The pain varies in intensity and can last for a few hours to a few days. Some people have only a few pain crises a year. Others have a dozen or more a year.
Notably, a severe pain crisis requires a hospital stay.
Some people with sickle cell anemia also have chronic pain from bone and joint damage, ulcers, and other causes.
Anemia. Sickle cells break apart easily and die. Typical red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells. This is known as anemia. Without enough red blood cells, the body can not get enough oxygen. This causes fatigue.
Swelling of Hands and Feet. Sickle-shaped red blood cells block blood circulation in the hands and feet, which can cause them to swell.
Delayed Growth or Puberty. Red blood cells provide the body with the oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in babies and children and delay puberty in teenagers.
Vision Problems. Tiny blood vessels that supply blood to the eyes can become plugged with sickle cells. This can damage the portion of the eye that processes visual images, called the retina, and lead to vision problems.
The Caribbean
Studies indicate that the prevalence of sickle cell trait (carrier status) in the Caribbean is around 7-10%. The region surrounding the Caribbean Sea is predominantly composed of island nations for its eastern part and the American continental coast in its western part. A large proportion of the population, particularly in the Caribbean islands, traces its ancestry to Africa as a consequence of the Atlantic Slave Trade. The Caribbean has the highest incidence of sickle cell disease outside of West Africa. The regional gene pool responsible for sickle cell disease is mostly of West African origin.
Treatment
Sickle cell disease treatment includes medications, transfusions, blood and marrow transplant, and gene therapy. Sickle cell disease treatment may begin with antibiotics.
A Stem Cell Transplant can cure SCD. Sometimes called blood or marrow transplant, SCT requires a donor who’s a good match, like a sibling, and ongoing studies are looking to optimize the transplant from alternative donors, such as birthing parents or siblings who are only half-matched. Your healthcare provider will discuss the risks and benefits of this treatment in your specific case.
People with sickle cell disease have a reduced life expectancy. New treatments for SCD are improving life expectancy and quality of life. People with sickle cell disease can survive beyond their 50s with optimal management of the disease.
Wayne Campbell is an educator and social commentator with an interest in development policies as they affect culture and or gender issues.
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@WayneCamo
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