People get sickle cell disease by a mutation in genes related to the hemoglobin. The blood cells in their body contain a different type of hemoglobin called hemoglobin S. When this molecule is exposed to
, it crystallizes inside the RBCs. This makes the cells elongate and appear as a ‘sickle.’
These ‘sickle-shaped stiff RBCs fail to move freely around the body. As a result, the affected individuals experience multiple complications like,
- Anemia Episodes of pain
- Stroke
- Swelling of hands and feet
- Frequent infections
- Delayed growth or puberty
- Vision problems
- Bone damage
Treatment options for sickle cell disease include drugs to manage anemia, antibiotics, NSAIDs and disease-modifying agents like hydroxyurea (reduce the frequency of painful episodes and the need for blood transfusions and hospitalizations).
Currently, gene therapy and a blood or marrow transplant are also expected to provide promising outcomes in the management of Sickle Cell Disease.
History of World Sickle Cell Day
The United Nations General Assembly recognized sickle cell disease as “one of the world’s foremost genetic diseases” on December 22, 2008. This announcement made its members spread awareness of the disease on June 19th every year in the name of “World Sickle Cell Disease.”
What’s Unique in 2021?
The theme for World Sickle Cell Day 2021 is “Shine the Light on Sickle Cell.” This 24-hour awareness campaign will be held by SiNERGe, collaborating with the Sickle Cell Disease Association of America, Inc. (SCDAA). Other activities of importance have also been planned by some organizations such as,
-
Sickle Cell Disease Association of Illinois (SCDAI) – Let’s Bowl on World Sickle Cell Day - Piedmont Health Services and Sickle Cell Agency – Roadmap to Sickle Cell Transition
- Sickle Cell Foundation of Minnesota – World Sickle Cell Day Selfie Challenge
- Martin Center Sickle Cell Initiative – 25th Annual Sickle Cell 5K Walk/Run Virtual Edition
- Sickle Cell Disease Association of America Michigan Chapter – Sickle Strong Sickle Cell Empowerment Circle
Sickle Cell Disease (SCD) – Facts and Figures
- Approximately 5% of the world’s population carries trait genes for hemoglobin disorders, mainly, sickle-cell disease
- Over 300 000 babies with severe hemoglobin disorders are born each year
- It is more common in African Americans (occurring in about 1 in 365 African American birth)
- Breathing issues are very common in kids with sickle cell disease
- More than 95% of newborns with SCD in US will live to be adults
References:
- Symptoms of Sickle Cell
– (https://www.sicklecellsociety.org/wscd/#didyouknow) - World Sickle Cell Day – (https://www.sicklecelldisease.org/get-involved/events/awareness-month-and-world-sickle-day/)
- June 19, 2021 – Shine the Light on Sickle Cell! – (https://wepsicklecell.org/events/2021-shine-the-light-on-sickle-cell/)
- Sickle cell anemia – (https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876)
- What are the treatments for sickle cell disease (SCD)?
– (https://my.clevelandclinic.org/health/diseases/12100-sickle-cell-disease#management-and-treatment) - 9 Things You Should Know About Sickle Cell Disease – (https://www.nationwidechildrens.org/family-resources-education/700childrens/2018/09/9-things-you-should-know-about-sickle-cell-disease)
Source: Medindia