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What is sickle cell disease?

What is sickle cell disease?

Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disorder have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.

Hemoglobin is the substance in red blood cells that is responsible for the color of the cell and for carrying oxygen around the body. People with sickle cell disorder are born with the condition, it is not contagious. It can only be inherited from both parents each having passed on the gene for sickle cell. The main symptoms of sickle cell disorder are anemia and episodes of severe pain. The pain occurs when the cells change shape after oxygen has been released. The red blood cells then stick together, causing blockages in the small blood vessels. These painful episodes are referred to as Sickle Cell crisis. They are treated with strong painkillers such as morphine to control the pain.

Symptoms. 

These requires a medical diagnosis from a medical practitioner. But a person with sickle cell or a sickler may experience 
1. Infections, 2. pain and fatigue as the easy to identify symptoms of sickle cell disease.

However they may also experience:

Pain areas: in the joints.
Pain types: can be sudden in the chest.
Whole body: dizziness, fatigue, low oxygen in the body, or malaise.
Urinary: inability to make concentrated or dilute urine or blood in urine.
Also common is the abnormal breakdown of red blood cells, delayed development, inflamed fingers or toes, pallor, shortness of breath, or yellow skin and eyes
People with sickle cell are at risk of complications stroke, acute chest syndrome, blindness, bone damage and priapism (a persistent, painful erection of the penis). Over time people with sickle cell can experience damage to organs such as the liver, kidney, lungs, heart and spleen. Death can also result from complications of the disorder. Treatment of sickle cell mostly focuses on preventing and treating complications.

Treatment and Cure.

The only possible cure for the disorder is bone marrow transplant but this is only possible for a limited number of affected individuals who have a suitable donor. A medicine called Hydroxyurea, can significantly reduce the number of painful crises.