World Sickle Cell Day is celebrated on June 19 every year, with the primary objective of raising awareness about sickle cell disease or sickle cell disorders, abbreviated as SCD. This global health campaign was inaugurated on June 19, 2009, by the United Nations (UN), after a General Assembly discussion on December 22, 2008, which implemented a resolution that acknowledged sickle cell disease as a public health problem. It even emphasized that SCD was “one of the world’s foremost genetic diseases”.
Sickle cell disease is an inherited ailment that impairs the red blood cells (RBCs) in children right from birth, that is acquired from parents carrying defective genes coding for haemoglobin. Haemoglobin is a protein carrying a heme or iron component that is present in red blood cells, which performs the key role of transporting oxygen to tissues and organs throughout the body. The red blood cells which are round and flexible in normal healthy individuals become quite rigid with an aberrant shape like a sickle in SCD. This restricts RBCs from travelling freely, obstructs the passage of oxygen to cells and tissues in the system, resulting in excruciating pain, thereby impeding the afflicted person’s ability to carry out normal routine activities and their productivity.
Hence, children with SCD require constant monitoring of their health due to increased risk of complications like heart illnesses, liver damage, kidney failure and stroke. They are also prone to eye defects and blindness, a decline in bone strength, sometimes even facing life-threatening events. Treatment for SCD involves averting severe complications and addressing any health anomalies that arise in the affected person at once. As this entails potent prescription medications, regular blood transfusions and sometimes even advanced procedures of bone marrow stem cell transplant, it often gives rise to many misconceptions about SCD and patients affected by the sickness.
Thus, in order for the general public to stay well-informed about sickle cell disease, on the occasion of World Sickle Cell Day 2021, here are some common myths linked with sickle cell disease discredited, along with the relevant scientific facts.
General Myths And Related Facts About Sickle Cell Disease:
Sickle cell disease is a contagious blood-borne illness.
False. Sickle cell disease is an inherited genetic blood-related disorder that is present in children from the time they are born and is therefore not infectious or contagious. Newborns acquire SCD only if both parents possess a faulty haemoglobin gene that is passed on to the offspring.
When infants are born with SCD, they do not survive to adulthood.
Incorrect. Children with parents carrying haemoglobin gene-related flaws go through genetic screening to assist in the early diagnosis of sickle cell disease. This, in turn, helps provide timely treatment, preventing the occurrence of infections, organ damage, stroke, which ensures the long life and endurance of the affected person well beyond 21 years of age.
Children with SCD possess immunity to malaria.
This is not true. Owing to the epidemiology and evolutionary nature of SCD being more common in regions with a high incidence of malaria, it is often assumed that young kids with SCD will not be affected by the mosquito-borne illness. However, they can get infected by malaria and require proper treatment with antimalarial drugs in that case.
There exists no cure for sickle cell disease.
This statement is erroneous. A bone marrow transplant is an effective cure, wherein the patient’s damaged bone marrow is replaced with healthy bone marrow containing optimal blood stem cells, which resolves sickle cell disease. This advanced medical procedure is however given only to individuals with severe SCD, after confirming a family member as an accurately matched donor.
SCD affects only people belonging to certain races and ethnicities.
This is absolutely wrong. Sickle cell disease can arise in children from any country in the world, irrespective of their ethnicity and race. The sole factor that predisposes an individual to SCD is both parents having a copy of a defective haemoglobin gene, affirming the point that it is an inherited genetic condition.