Saturday 19 June is World Sickle Cell Day – it is an opportunity to raise awareness of Sickle cell disease and celebrate all those who live with the condition or are working to support those living with it.
What is Sickle Cell Disease?
Sickle cell disease refers to a group of inherited red blood cell disorders. It is the most common genetic disease in the U.S. An estimated 70,000-80,000 Americans have sickle cell disease and yet there is still relatively little known about living with and managing its symptoms. Origins are currently working with a group of patients in the US to understand what it is like to live with and be treated for sickle cell disease.
Sickle cell disease is inherited from your parents and affects people of many nationalities including Italians, Latin Americans, Greeks, Arabs, and Asiatic Indians. However, it disproportionately affects people of African descent.
Normal red blood cells are round like doughnuts, and they move through small blood tubes in the body to deliver oxygen. Sickle red blood cells become hard, sticky and shaped like sickles used to cut wheat. When these hard and pointed red cells go through the small blood tube, they clog the flow and break apart. This can cause pain, damage and a low blood count, or anaemia.
How can those not affected by sickle cell disease support World Sickle Cell Day and people with sickle cell disease?
- Give blood! If you are able to, please donate blood. Sickle cell patients often need multiple blood transfusions to alleviate the painful crisis episodes
- Educate yourself – use this awareness day as an opportunity to find out more about sickle cell disease.
- Share news with others about sickle cell disease – below are some examples of ways in which sickle cell disease has been in the public arena in the last couple of year
In June 2021 there was a Sky News Daily Podcast episode entitled “Sickle cell: The lives lost and the campaign for change” featuring the parents of Evan Smith who had sickle cell disease and died in hospital at the age of 21 from sepsis. Due to a lack of education about his condition and outdated policy Evan even resorted to calling 999 from his hospital bed. His parents are working with the NHS to ensure that lessons have been learned from this tragic situation. The podcast episode also interviews Dame Elizabeth Anionwu, who was Britain’s first specialist sickle cell nurse.
In May 2020, the podcast ‘This podcast will kill you’ focused a two-hour episode on sickle cell disease. The two hosts (both named Erin) clearly and simply talk through the aetiology and symptomology of sickle cell disease as well as summarising the history of sickle cell treatment and spotlighting the technologies being explored for the future – including the CRISPR gene-editing technology that won the Nobel Prize in Chemistry for Emmanuelle Charpentier and Jennifer Doudna. One of the patient stories featured in the podcast episode is a member of the B positive choir in the UK. The B positive choir first came to the public’s attention on the 12th season of Britain’s Got Talent and is made up of 60 singers who suffer from the sickle cell disease or who have close friends or family suffering from it.
Finally, a few quick facts about sickle cell disease:
- There are 100,000 individuals living with sickle cell disease in the US
- There are 75,000 annual hospitalisations annually due to sickle cell disease in the US
- 4 new treatments have been approved by FDA since 2017
- 2 new therapies are currently in phase III clinical trials
Origins looks forward to raising awareness and increasing understanding around sickle cell disease through our current and future work.