Explained | Sickle Cell Disease

Sickle Cell Disease is a blood disorder caused by the abnormal haemoglobin that damages and deforms red blood cells. Representative image: Dr_Microbe / iStock

World Sickle Cell Awareness Day is observed annually on June 19. 

The international awareness day is observed with the goal to increase public knowledge and an understanding of sickle cell disease, and the challenges experienced by patients and their families and caregivers.

Sickle Cell Disease

• The haemoglobinopathies are a group of disorders passed down through families (inherited) in which there is abnormal production or structure of the hemoglobin molecule.

• Approximately 5 per cent of the world’s population carries trait genes for haemoglobin disorders, mainly, sickle-cell disease and thalassaemia. 

• Over three lakh babies with severe haemoglobin disorders are born each year globally.

• Sickle Cell Disease (SCD) is one such blood disorder caused by the abnormal haemoglobin that damages and deforms red blood cells.

• Red blood cells contain haemoglobin, a protein that carries oxygen. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has SCD, the haemoglobin is abnormal, which causes the red blood cells to become hard and sticky and look like a C-shaped farm tool called a “sickle”. 

• The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious complications.

• The disorder requires lifelong management and contributes to infant and childhood morbidity and mortality. SCD is caused by inheritance of two abnormal HbS genes, one from each parent or HbS gene from one parent and HbE or β thalassemia gene from the other. 

• Sickle cell syndromes include Sickle Cell Disease (SCD, HbSS), also called Sickle Cell Anaemia (SCA), as well as disorders due to sickle cell gene combined with another hemoglobinopathy such as Hb C, E, or β thalassemia.

• Persons carrying only one of these genes are called ‘carriers’ as they do not suffer from any disease but carry the abnormal gene and transmit it to the next generation. Carriers cannot be recognised clinically, but only by performing special blood tests. Where both mother and father are ‘carriers’, there is a chance that their children may inherit the abnormal gene from both parents.

National Sickle Cell Anaemia Eradication Mission

• In July 2023, Prime Minister Narendra Modi launched National Sickle Cell Anaemia Eradication Mission (NSCAEM) 2047.

• NSCAEM, which aims to address the pressing health challenges posed by sickle cell disease, particularly among the tribal population, was announced in the Union Budget 2023.

• This mission will be implemented in 278 districts of 17 states. 

• The 17 states are: Gujarat, Maharashtra, Rajasthan, Madhya Pradesh, Jharkhand, Chhattisgarh, West Bengal, Odisha, Tamil Nadu, Telangana, Andhra Pradesh, Karnataka, Assam, Uttar Pradesh, Kerala, Bihar and Uttarakhand.

• The programme is executed in a mission mode as part of the National Health Mission (NHM), aims to eliminate sickle cell genetic transmission by the year 2047, showing a long-term commitment to eradicating the disease.

• Over a period of three years, the programme targets screening approximately 7 crore people.

• This ambitious goal highlights the programme’s objective to reach a large portion of the population, promote early diagnosis and intervention.

• About 1 in 86 births among Scheduled Tribe (ST) population have SCD, the prevalence being higher in central, western and southern India. It is predominantly prevalent in Jharkhand, Maharashtra, Madhya Pradesh, Chhattisgarh, western Odisha, eastern Gujarat and in pockets of the Nilgiri Hills in north Tamil Nadu and Kerala. 

• More than 3 crore people have already been screened for Sickle Cell Disease under NSCAEM.

The comments posted here/below/in the given space are not on behalf of Onmanorama. The person posting the comment will be in sole ownership of its responsibility. According to the central government's IT rules, obscene or offensive statement made against a person, religion, community or nation is a punishable offense, and legal action would be taken against people who indulge in such activities.