Sickle Cell Disease

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Sickle Cell Disease

Sickle Cell Disease


      Most people who are diagnosed with Sickle Cell Anemia are African-Americans, and about 1 in every 365 African-  American children are born with it. Sickle cell disease is a blood disorder that’s inherited — meaning it’s passed down from parents to their children. Babies are born with sickle cell disease when they inherit two abnormal genes (one from each parent). These genes cause the body’s red blood cells to change shape.




Sickle cells are stiffer. Instead of being disc shaped, they’re curved like crescent moons, or an old farm tool known as a sickle. That’s where the disease gets its name.

While sickle cell disease primarily affects persons of African descent, it is also present in Portuguese, Spanish, French Corsicans, Sardinians, Sicilians, mainland Italians, Greeks, Turks and Cypriots. Sickle cell disease also appears in Middle Eastern countries and Asia.

1910 is regarded as the date of the discovery of sickle cell disease, so 2010 was the 100th anniversary of that discovery, but just what does it mean to say the disease was “discovered”?


Microscopic view of sicke cells causing anemia disease.
Microscopic view of sicke cells causing anemia disease.

Originally from Africa and brought to the Americas by the forced immigration of slaves, it is more frequent where the proportion of African descendants is greater. Carriers of the sickle cell trait have some resistance to the often-fatal malaria. This is why it is found more frequently in persons of Middle Eastern, Indian, Mediterranean and African heritage because those geographic regions are most prone to malaria.

However, in areas such as the US, where malaria is not a problem, the trait no longer provides a survival advantage. Instead, it poses the threat of SCD, which occurs in children of carriers who inherit the sickle cell gene from both parents.

There are several types of sickle cell disease, and the most common one is sickle cell anemia. It makes you feel tired, because your blood cells don’t last as long as other people’s do.

Normally, red blood cells live about 120 days. New ones that replace them are made in the soft, spongy center of your bones called the marrow. If you have sickle cell anemia, your red blood cells start dying after only 10 to 20 days. Your bone marrow can’t replace them fast enough, which causes anemia. Red blood cells carry oxygen around your body, which gives you energy. If you don’t have enough of them, you’ll get tired and you might also feel short of breath.

SCD patients learn to function in a constant state of pain and when that pain becomes debilitating, they often end up in the emergency room. What brings people to the emergency room is called a crisis; in which the blood can’t deliver oxygen to the extremities. It is a sudden onset of severe, excruciating pain, which people describe as feeling as if all their bones are breaking. The pain is so bad and sudden that people require high doses of opioids.

Contrary to the belief of many in the medical profession, there is no data to support that sickle cell patients have any more likelihood of being addicted than anybody else. photo611475392035923911-660x346

Acute and unpredictable pain, severe enough to require large doses of narcotics, is a well-documented feature of sickle cell disease. When many sickle cell patients arrive at emergency rooms, they often have great difficulty getting the treatment they need.

Racism and the disease stigma itself are two barriers that you just can’t get away from. Clearly we can’t pretend that racism doesn’t play some part in this. If this were a white disease, people still wouldn’t be dying in their forties. That’s the bottom line. Sickle cell was “discovered” 106 years ago and there is only one drug, hydroxyurea, and blood transfusions to treat it.

There has been progress in Sickle cell disease. People didn’t used to live to be adults. Kids would die of stroke or of really bad infections before they were 20, and in some countries they still do.