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What is Sickle Cell Disease?

Sickle Cell Disease is a term used to describe a group of inherited disorders which involve an abnormal hemoglobin molecule in the red blood cells, called hemoglobin S (HbS).  Hemoglobin is the substance in red blood cells that carries oxygen.

Sickle Cell Anemia is a disease characterized by chronic severe anemia, episodes of severe painful crises that last hours, or days, or weeks, and may cause organ damage, and eventually death.

Sickle Cell Disease is most prevalent in those of African, Mediterranean and Indian origins.  A large number of Sickle Cell patients are found in Sub-Saharan Africa, India, South America, Central-America,  Cuba, Caribbean Islands, Jamaica, Saudi Arabia, Syria, Turkey, Sicily, Southern Italy and Greece.

In the U.S., SCD affects approximately 100,000 people.  According to the Center for Disease Control, about "one in 365 African Americans, and one in 16,300 Hispanic-Americans are born with the disease." More than 2 million people in the U.S. carry the Sickle gene that allows them to pass it on to their children (Sickle Cell Trait). 


Sickle Cell Disease is a major public health concern according to the Centers for Disease Control (CDC).  Each year in the US an average of 75,000 hospitalizations are due to Sickle Cell Disease.  Total cost for treating Sickle Cell in the US is about  $1.1 billion.

 

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Treatments

Treatment depends on the severity of the case. Treatments include medications, blood transfusions, and rarely a bone-marrow transplant. Always consult a physician for medical advice.

1  Drink Plenty of Water

Drink plenty of water (8-10 glasses per day). Exercise regularly but avoid getting too cold, too hot or too tired.

2  Get Plenty of Bed Rest

Getting the proper rest helps the body do its best work to remain healthy.

3 For Pain

Analgesics (pain medications-including aspirin, ibuprofen and other anti-inflammatory medications, morphine and other narcotics, as well as spinal anesthesia.) Heating pads, meditation, massage and distractions may help some.

4  Medication and Other Treatments

Antibiotics and pneumonia vaccine; Hydroxyurea– A drug that stimulates the production of fetal hemoglobin (a healthier form of hemoglobin); Endari (L-Glutamine oral powder) to reduce acute complications;  Anticoagulant therapies (blood thinners); Red Blood Cell Transfustion. Not everyone responds to these various treatments.

5  Possible Cures

Bone Marrow/Stem Cell Transplantation, a cure for a few. The procedure requires a closely matched donor.

Clinical trials are beginning for gene therapy as a possible cure in the future.

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