John James OBE – Sickle Cell, The Facts

John James is the Chief Executive of the Sickle Cell Society (SCS), a role which he has held since 2013. Prior to working at SCS, John spent 33 years working in the National Health Service, also known as the NHS, including as Chief Executive of NHS Trusts and in health policy as a senior civil servant for the Department of Health and Social Care. During his time as Chief Executive of SCS, the organisation has published a number of reports highlighting the impact of sickle cell disease on patients, and has been instrumental in the publishing of national clinical standards and guidelines. In June 2018, John was awarded an OBE as part of the Queen’s Birthday Honours.

What is Sickle Cell? Sickle cell anemia is one of a group of inherited disorders known as sickle cell disease. It affects the shape of red blood cells, which carry oxygen to all parts of the body.

Red blood cells are usually round and flexible, so they move easily through blood vessels. In sickle cell anemia, some red blood cells are shaped like sickles or crescent moons. These sickle cells also become rigid and sticky, which can slow or block blood flow.

There’s no cure for most people with sickle cell anemia. Treatments can relieve pain and help prevent complications associated with the disease.

Causes ~ Sickle cell anemia is caused by a change in the gene that tells the body to make the iron-rich compound in red blood cells called hemoglobin. Hemoglobin enables red blood cells to carry oxygen from the lungs throughout the body. The hemoglobin associated with sickle cell anemia causes red blood cells to become rigid, sticky and misshapen.

For a child to be affected, both mother and father must carry one copy of the sickle cell gene — also known as sickle cell trait — and pass both copies of the altered form to the child.

If only one parent passes the sickle cell gene to the child, that child will have the sickle cell trait. With one typical hemoglobin gene and one altered form of the gene, people with the sickle cell trait make both typical hemoglobin and sickle cell hemoglobin.

Their blood might contain some sickle cells, but they generally don’t have symptoms. They’re carriers of the disease, however, which means they can pass the gene to their children.

Risk factors ~ For a baby to be born with sickle cell anemia, both parents must carry a sickle cell gene. In the United States, sickle cell anemia most commonly affects people of African, Mediterranean and Middle Eastern descent.

Website: www.sicklecellsociety.org

Email: info@sicklecellsociety.org

Facebook: Sickle Cell Society UK

Twitter: @SickleCellUK

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