The 19th June is World Sickle Cell Day, the day designated to raise awareness of sickle cell at a national and international level. On 22nd December 2008, the United Nations General Assembly adopted a resolution that recognises sickle cell disease as a public health problem and “one of the world’s foremost genetic diseases.” The resolution calls for members to raise awareness of sickle cell on June 19th of each year at national and international level. So let’s venture on this journey of awareness.
What is Sickle Cell or Sickle Cell Disease (SCD)?
Sickle cell disorders are a group of illnesses which affect your red blood cells that carry oxygen from your lungs to your tissues. Sickle cell is a genetic condition, which means it is passed on from your parents and you are born with it; you cannot catch it from other people.
Sickle cell disorders cause your normally round and flexible blood cells to become stiff and sickle shaped, stopping the blood cells, and the oxygen they carry, from being able to move freely around the body and causing pain as the body’s tissues become starved for oxygen. This can cause episodes of severe pain. These painful episodes are referred to as sickle cell crisis. They are treated with strong painkillers such as morphine to control the pain.
People with sickle cell are also at risk of complications of stroke, acute chest syndrome, blindness, bone damage and priapism (a persistent, painful erection of the penis). These organs are acutely affected by sickling as they are likely to be starved of oxygen resulting in damage.
Over time people with sickle cell can experience damage to organs such as the liver, kidney, lungs, heart and spleen. Death can also result from complications of the disorder. Treatment of sickle cell mostly focuses on preventing and treating complications.
Symptoms of Sickle Cell
Chronic (long term) anaemia – when there are not enough red blood cells or Hb in your body.
Unpredictable Pain (crisis) – When the blood vessels get blocked, it can cause pain and swelling in the area, also known as a crisis. Crisis may be precipitated but things such as stress, dehydration and infection.
You may get tired easily or experience fatigue – constant tiredness, feeling weak or lacking in energy because of the anaemia.
Jaundice – yellowing of the whites of the eyes and skin.
Facts About Sickle Cell Disease
1. SCD is inherited from both parents; sickle cell trait is inherited from one parent.
2. SCD can affect anyone, although it predominantly affects people from African and Caribbean backgrounds.
3. A simple blood test will tell whether you have sickle cell trait or the disorder
4. Children with SCD are at increased risk for stroke, the risk is highest between the ages of 2
5 .Episodes of pain may occur in sickle cell disorder and are generally referred to as a crisis
The only possible cure for the disorder is bone marrow transplant but this is only possible for a limited number of affected individuals who have a suitable donor. A medicine called Hydroxyurea, can significantly reduce the number of painful crises.
The Sickle Cell Trait (SCT)
Sickle cell trait is inherited when only one of your parents has passed on the sickle gene, and will never develop into sickle cell disorder. You do not have symptoms from sickle cell trait, so it is a good idea to have a blood test to see if you have sickle cell trait. If you have the trait, the majority of red cells in the blood are normal round shaped cells. Some sickle shaped cells may be present under certain conditions.
The trait is not an illness, but if you are planning to have children, then certain factors have to be considered.
If your partner does not have sickle cell trait, then any children you have will not have sickle cell disorder, but they could have the trait (50% chance).
If you and your partner both have the trait, there is a 25% chance that any child conceived may have sickle cell disorder and 50% chance they will have the trait.
Often, individuals who have persistent anaemia discover that SCD or SCT may be hereditary in their families and a simple blood test can diagnose the presence of disease.
For more information please visit the Sickle Cell Society of T&T at https://www.onescdvoice.com/place/sickle-cell-society-trinidad-tobago/
Dr. Visham Bhimull
Diploma in Family Medicine (UWI)