Key things people should know about sickle cell disease

Sickle cell disease is a group of inherited red blood cell disorders that affect the haemoglobin – the protein that carries oxygen around the body.

Normally, red blood cells are disc-shaped, and are flexible enough to move easily through the blood vessels. But in sickle cell disease, the red blood cells become misshapen due to a genetic mutation, and break down.

These sickled red blood cells do not bend or move easily, hence causing the blocking of blood flow and resulting to pain in the body.

Many times, people use the term “sickle cell anemia” and “sickle cell disease” interchangeably, because they think both are the same.

However, sickle cell disease is a major term for the different types of sickle cell disorders, which includes sickle cell anemia.

Other disorders that fall under the category of sickle cell disease include:

Haemoglobin SS (HbSS)
HbSS is a severe form of sickle cell disease affecting 65% of people who have sickle cell disease. People with this disorder inherited one gene encoded with haemoglobin S from each parent.

Haemoglobin SC (HbSC)
HbSC is a mild form of SDC that affects about 25% of people with the disease. People with this form inherited a haemoglobin S gene from one parent, and inherited another abnormal type — haemoglobin C – from the other parent.

Haemoglobin (HbS) beta thalassemia
People with this form inherited a haemoglobin S gene from one parent, and inherited an abnormal type called beta thalassemia from the other parent. Haemoglobin (HbS) beta thalassemia has two types, namely:

HbS beta +: This subtype affects about 8% of people with SCD and tends to be more mild. It is also called sickle beta plus.

HbS beta 0: This subtype affects about 2% of people with SCD and is more severe, similar to hemoglobin SS disease. It is also called sickle beta zero.

Sickle cell diseases lead to acute and chronic complications such as pain, anemia, acute chest syndrome, blood clots, stroke, vision problems, priapism, organ damage and failure.

Even as sickle cell is as old as human history, quite a number of people still have misconceptions about it. Below are some key things people should know about sickle cell disease:

Genetic inheritance: SCD is inherited from parents, so family history is important.

Symptoms severity varies: Pain, anemia, infections, and organ damage can occur, but the severity differs from patient to patient.

Management: Sickle cell diseases can be managed, if diagnosed early. Early diagnosis, regular care, and pain management can improve quality of life.

It is Universal: Sickle cell disease is not limited to the blacks or Africans. Yes, it is more common in Africa, but it can affect anyone, regardless of races and ethnicities.

Caring for People with SCD: People with SCD face severe complications. The best support that can be given is compassion and empathy.

To all the strong and resilient SCD soldiers out there, we see your strength and courage to keep overcoming. Our well wishes are with you.

Happy World Sickle Cell Day!