World Sickle Cell Day

World Sickle Cell Day 2018

Sickle cell disease (SCD) is the world’s most common genetic blood disorder. SCD is an inheritable, fatal disease causing red blood cell disorders classified as sickle cell anemia and may lead to death. It is present in Sub-Saharan Africa, Asia, North and South America and Southern Europe. According to the United Nations, nearly 500,000 babies with SCD are born each year. Of these babies, 50% will die before the age of 5. Lack of access to treatment and inadequate healthcare facilities are the chief reasons for morbidity. SCD is caused by a mutation in the globin gene which is responsible for hemoglobin production. The mutated sickle cell gene (HbS) leads to the sickling of red blood cells which causes painful clots and tissue damage in the body. Blood clots can also cause heart attacks and strokes. Abnormally shaped cells cause anemia and patients with SCD require regular blood transfusions to stay alive. Currently there is no permanent cure for SCD except bone marrow transplant.

It is important to raise awareness about this common genetic disorder and in 2008, the United Nations officially declared 19 June as World Sickle Cell Awareness Day. SCD is a public health priority globally and efforts have been made towards prevention and proper management of the disease. Since the disease is currently incurable, it is important to put in robust prevention programs.

World Sickle Cell Day is a key platform for raising awareness and making the public aware of this disease. Not only the public but clinicians and healthcare providers must also be aware and updated about the spread of this disease. Genetic counseling, antenatal and prenatal testing are necessary to prevent the spread of SCD. Information is the key to take decisions and young people who get themselves tested for carrier status must be cautious.

Children and adults with SCD often face not just health but other social challenges. Discrimination in education and employment is one of the major problems faced by children and adults. Globally there are legislations to protect the rights of people with disabilities and in many countries like India, SCD is included in disability laws. World Sickle Cell Anemia Day is a platform where such issues are highlighted and patient communities are made aware of their legal rights. World Sickle Cell Day is also a day to call attention towards strengthening the medical and healthcare systems to serve the patient communities better. Often such rare, genetic disorders do not receive proper treatment within the public health systems due to lack of expertise and facilities. This day is an occasion to appeal to governments, public health organizations and medical care providers to ensure adequate diagnostics, treatment and interdisciplinary management of SCD.

In India, the National Guideline on Hemoglobinopathies was released by the National Health Mission in 2017. This guideline aims at improving the treatment and management of patients with SCD by providing free blood transfusions, iron chelation medicines and other interventions in all government hospitals and state-run health centers. Such policies are a positive step for improving the life quality of patients. The Disability Act 2016 also provides legal protection for SCD patients including access to education and employment.

World Sickle Cell Day is a day to empower patients with robust healthcare and societal and community facilities to lead a quality life despite this serious condition.

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References:

1. World Sickle Cell Awareness Day - (https://parentsguidecordblood.org/en/news/june-19-world-sickle-cell-day)
2. June 19 is World Sickle Cell Day - (https://sicklecellcurefoundation.org/news/world-sickle-cell-awareness-day)

Source-Medindia