Contents:
- Medical Video: Living with sickle cell disease: Shaniya's story
- How can sickle cell disease occur?
- Can sickle cell anemia be cured?
Medical Video: Living with sickle cell disease: Shaniya's story
The term sickle cell disease or sickle cell disease (SCD) includes several inherited red blood cell abnormalities. SCD sufferers have abnormal hemoglobin called hemoglobin S or sickle hemoglobin in their red blood cells.
Hemoglobin is a protein in red blood cells that carries oxygen throughout the body.
The term "inheritance" indicates that this disease is caused by genes passed down by parents to their children. Unlike the common cold or infection, SCD is not contagious so that healthy people will not be exposed to SDC from sufferers.
SCD sufferers inherit two abnormal hemoglobin genes from father and mother. Whatever type of SCD is suffered, at least one of the two abnormal genes causes the body to produce hemoglobin S. Meanwhile, someone who has two hemoglobin S genes (or SS hemoglobin) will suffer from a disease called sickle cell anemia. Sickle cell anemia is the most severe and most common type of SCD.
SC hemoglobin and hemoglobin Sβ thalassemia are two other common forms of SCD.
Some forms of sickle cell disease:
- Hemoglobin SS
- Hemoglobin SC
- Hemoglobin Sβ0 thalassemia
- Hemoglobin Sβ+ thalassemia
- Hemoglobin SD
- Hemoglobin SE
How can sickle cell disease occur?
Cell tissues of the body need a supply of oxygen to work properly. Usually, hemoglobin in red blood cells takes oxygen from the lungs and carries it to all body tissues.
Red blood cells that contain normal hemoglobin are shaped like pieces (like donuts without holes). This form allows cells to become flexible so that they can move through large and small blood vessels to carry oxygen.
Sickle hemoglobin is not like normal hemoglobin. Sickle hemoglobin forms a rigid stem in red blood cells, turns into a sickle, or shape sickle.
Crescent cells are inflexible and can stick to the vessel wall, causing blockages that slow or stop blood flow. When this condition occurs, oxygen cannot reach the surrounding tissue.
Lack of tissue oxygen can cause severe pain called a pain crisis (pain crises) This pain can strike suddenly without warning, so that patients need effective treatment at the hospital.
Most children with SCD experience no pain or pain criseswhile adolescents and adults can suffer from chronic, ongoing pain.
Abnormal red blood cells and low oxygen intake can also cause damage to organs, such as the spleen, brain, eyes, lungs, liver, heart, kidneys, penis, joints, skin, or even bones.
Because the shape is not flexible, sickle cells can explode or are called hemolyze. The normal life cycle of red blood cells ranges from 90 to 120 days, while sickle cells can only last for 10 to 20 days.
The body always makes new red blood cells to replace old cells. However, the body of SCD sufferers has difficulty balancing new blood cell production with the rapid destruction of blood cells. As a result, the number of red blood cells tends to be lower than the normal number. This condition is called anemia, as a cause of the body's reduced energy.
Can sickle cell anemia be cured?
Sickle cell disease is a lifelong disease. The severity of the disease of each sufferer must vary.
In high-income countries such as the United States, the life expectancy of SCD sufferers is currently around 40-60 years. In 1973, the average age of SCD patients in the United States was only 14 years. This shows progress in the diagnosis and treatment of SCD.
At this time, hematopoietic stem cell transplantation (HSCT) is the only medicine for SCD. Unfortunately, most SCD sufferers are too old to undergo a transplant or do not have relatives who have a genetic match as a donor. Suitable donors are needed to get the best transplant results.
There are many effective treatments that can reduce symptoms and prolong the life of SDC sufferers. Early diagnosis and regular medical care to prevent complications also contribute to improving the quality of life for patients.
