Mya Care Blogger 09 Jul 2019

What Is Sickle Cell Disease?

Sickle Cell Disease (SCD) is the most common genetic blood disorder. With this disorder, normally round and pliant red blood cells assume the shape of a sickle and adhere to the blood vessels. This creates a shortage of oxygen-rich red blood cells as well as causing blood flow to slow or stop completely. This causes severe pain, organ and tissue damage, and even strokes in its victims.

SCD is the result of the Sickle Cell gene being inherited from both parents. When this occurs, the result is known as Sickle Cell Anemia, which is just one type of the disease. Those who carry this gene, whether they become infected or not, have what is known as Sickle Cell Trait (SCT). Other forms of SCD come from the Sickle Cell gene being inherited from one parent and other forms of hemoglobin-deficient genes from another parent. Of the many varieties, Sickle Cell Anemia is by far the most dangerous and most painful.

There is currently no cure for SCD However, bone marrow transplant and blood transfusion can keep it under control. There is also hope in stem cell therapy that have been recently developed. This disease is traditionally considered to be limited to those of African descent; however, it can and will affect any ethnic group as long as the gene is present. Since bacterial infections are frequent in the patient, a dose of Penicillin twice a day for the first five years of life will normally keep it under control.

While SCD is detectable at birth, symptoms do not normally manifest until the patient is about six months in age. In the United States, every baby born is screened for the disease no matter what their ethnic background.

Worldwide Sickle Cell Disease Statistics

In Africa, up to 40% of the population carries the gene. In Sub-Saharan Africa, 2% of the population has the disease. The disease is also found in parts of Sicily, Greece, Turkey, and India.

In the United States, there are 100,000 cases of SCD. 1 in 13 African-Americans carry the trait, 1 in 365 African-Americans are born with the disease, and 1 in every 629 births across all races. 1 in 16,300 Hispanic-Americans is born with SCD.

Signs and Symptoms of SCD

The signs and symptoms of SCD include:

  • Fatigue and anemia.
  • Pain crises that can last anywhere from a few hours to a few days.
  • Swelling in the hands and feet.
  • Frequent bacterial infections.
  • Spleen and liver involvement due to blood pooling inside the organ.
  • Injury to the heart and lungs.
  • Leg ulcers.
  • Frequent dehydration.
  • Eye damage that leads to loss of vision.
  • Aseptic necrosis involving death in portions of the bones.
  • Deep vein thrombosis or pulmonary embolism.
  • Stroke.

Seven Facts About SCD

  1. SCD occurs more frequently in parts of the world where malaria was and is more common. SCD patients are infected with Malaria less commonly than other members in the population
  2. SCD is apparent in the first year of life and symptoms normally appear at about six months of age, although it is tested for at birth.
  3. Because it is a blood disorder, SCD can affect every organ.
  4. SCD provides the highest risk of stroke in children
  5. SCD can affect every ethnic group, including Caucasians. 
  6. Breathing problems are extremely common in children with SCD.
  7. Children with SCD who are given penicillin twice a day for the first five years of their lives as it helps prevent pneumonia and other bacterial infections that they are more susceptible to.

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