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Sierra Leone lacks the basic facilities to effectively manage Sickle Cell Disease.  

Blood tests could be used for parental, maternal screening or newborn screening.  However, these tests are not easily accessible in Sierra Leone.  The diagnosis is therefore normally made when a severe complication occurs. In many cases early complications are misdiagnosed and many times, mistaken for malaria.  Sierra Leone has a very high infant mortality rate and according to the WHO, “the majority of children with the most severe form of the disease die before the age of five, usually from an infection or severe anemia.”

 

Something has to change!

 

In developed countries, there is cost-effective treatment available for pain episodes, prevention of infection etc… by early intervention with analgesics, antibiotics, rest, good nutrition, folic acid supplementation and high fluid intake.  In Sierra Leone however, the health care framework is unable to provide sustainable support for Sickle Cell treatment. 

 

The doctors in Sierra Leone, do their part to care for patients but this is mostly done on a volunteer basis and with very limited resources.  Our charge therefore is to raise funds and partner with providers who are committed to educating the community about Sickle Cell Disease, making early diagnosis a priority and pro-actively managing the disease for our warriors.

What is Sickle Cell?

Red blood cells have a protein called hemoglobin.  Hemoglobin carries oxygen throughout the body. 

Normal hemoglobin molecules are smooth, round and flexible.  They allow red blood cells to glide easily through the bloodstream.  

Abnormal hemoglobin molecules have an irregular shape.  This allows them to clump together.  Clumped hemoglobin makes red blood cells curved and rigid.  The red blood cells are shaped like a sickle.  Sickled red blood cells block the flow of blood.  This is dangerous and can cause sudden extreme pain.

 

Normal red blood cells live between 90 – 120 days. 

Sickle Cells last only 10 to 20 days. Because sickle cells don’t last for long, 

persons with Sickle Cell normally have a lower number of red blood cells. 

This condition is called Anemia and it makes people have less energy.

What are the Types of Sickle Cell?

According to the NIH, some forms of Sickle Cell are:

  • Hemoglobin SS

  • Hemoglobin SC

  • Hemoglobin Sβ0 thalassemia

  • Hemoglobin Sβ+ thalassemia

  • Hemoglobin SD

  • Hemoglobin SE

What are some symptoms of Sickle Cell?

A person with sickle cell is born with it however most infants

do not have any problems from the disease until they are about

5 or 6 months old.  The Symptoms of Sickle Cell vary from person

to person and this can change over time. 

The earliest symptoms may include:

  • Dactylitis: painful swelling of the hands and feet.

  • Anemia: fatigue or fussiness.

  • Jaundice: yellowish color of the skin.

  • Icterus: yellowish color on the whites of the eyes.

Over time, other signs and symptoms appear due to complications from Sickle Cell. 

Who can get Sickle Cell?

When the hemoglobin S gene is inherited from only one parent and a normal hemoglobin gene is inherited from the other, a person will have sickle cell trait.   

 

People with sickle cell trait are carriers of a defective hemoglobin S gene. They can pass it on to their kids.

 

If the child’s other parent also has sickle cell trait or another abnormal hemoglobin gene (like thalassemia, hemoglobin C, hemoglobin D, hemoglobin E), that child has a chance of having SCD.

Can sickle cell be cured?

Currently, hematopoietic stem cell transplantation (HSCT) is the only cure for Sickle Cell. 

Otherwise, Sickle cell is a life-long disease. 

An early diagnosis and regular care to prevent complications contribute to overall well-being. 

Can sickle cell be prevented?

There are tests available to help determine whether or not a person carries an abnormal hemoglobin.  Genetic counselors can answer questions about choices available if having a child with Sickle Cell disease is a concern.

Source:  https://www.nhlbi.nih.gov/health/health-topics/topics/sca

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