How does sickle cell disease kill a person?

Contact [email protected] to license this or any News Direct video
For story suggestions please contact [email protected]

RESTRICTIONS: NONE

Prodigy, one half of hip-hop and rap duo Mobb Deep, died Tuesday in Las Vegas at the age of 42 after being hospitalized for complications related to sickle cell anemia.

Mobb Deep is widely regarded as one of the most successful acts of all time. Prodigy, born Albert Johnson, suffered from sickle cell anemia since birth.

Sickle cell disease is a group of red blood cell disorders passed by genes from parents to their children. For example, if both parents have sickle-cell trait, a child has a 50% chance of having the sickle-cell trait, 25% will not carry sickle-cell alleles, and 25% develop a chance of having sickle-cell disease as two hemoglobin S genes are inherited.

Hemoglobin is a protein that carries oxygen in red blood cells. Red blood cells that contain normal hemoglobin are disc shaped, whereas sickle cell hemoglobin forms long and inflexible chains. Normal red blood cells are flexible and can pass through large and small blood vessels. Sickled red blood cells can stick to vessel walls, which can cause a blockage in the vessels that stops the oxygen delivery.

Normal red blood cells can live up to 120 days. When the number of red blood cells decreases, the kidneys releases erythropoietin hormone that stimulates the bone marrow to produce more red blood cells.

Sickled blood cells can only last up to 20 days due its inflexible nature. The body often cannot keep up with the fast pace of decreased red blood cells, causing the person to suffer anemia. Anemia can cause various organ damage over time, including to the brain, heart, spleen, lungs and kidneys, as well as to the skin and bones.

According to the U.S. National Heart, Lung and Blood Institute, most people with sickle cell disease are of African ancestry. About one in 13 African American babes is born with sickle cell trait and one in 365 black children is born with sickle cell disease.

RUNDOWN SHOWS:
1. Genetics of sickle cell disease
2. Normal red blood cells and hemoglobin compare to sickled blood cell and abnormal hemoglobin
3. Normal red blood cell cycle compare to sickle cell anemia
4. Organ damage caused by sickle cell anemia

SOURCES: National Heart, Lung and Blood Institute, BBC, The Washington Post
https://www.nhlbi.nih.gov/health/health-topics/topics/sca
http://www.bbc.co.uk/newsbeat/article/40351370/prodigy-from-rap-group-mobb-deep-has-died-aged-42-after-performing-in-las-vegas
https://www.washingtonpost.com/news/arts-and-entertainment/wp/2017/06/20/prodigy-of-iconic-new-york-rap-duo-mobb-deep-dead-at-42/?utm_term=.6e33cb3312a6