Understanding Sickle Cell Anemia, Anemia Caused by Genetic Disorders

The Shiro Corporation JAKARTA - Anemia This is one condition that many people in Indonesia experience, especially in children. The disease is often ignored, even though it can affect overall health.

Anemia is a condition where the number of red blood cells or hemoglobin in the body is below the normal range. This condition is not only experienced by adults but also frequently occurs in children.

Based on data from the World Health Organization (WHO), as many as 39.8% of children aged 6 months to 5 years suffer from anemia. In Indonesia itself, the prevalence of children under 5 years old suffering from anemia reaches 38.5%.

The Working Group Coordination Unit (UKK) of IDAI Hematology Oncology, Prof. Harapan Parlindungan Ringoringo said that anemia in children can affect overall health, including causing concentration disorders, easy fatigue, and developmental disorders.

Therefore, recognizing the causes and symptoms of anemia becomes an important first step in determining the proper way to address anemia.

What is Sickle Cell Anemia?

Among the types of anemia, there is sickle cell anemia, which is a genetic disorder that causes red blood cells to become abnormally shaped.

The abnormal shape of those blood cells results in a reduced supply of healthy blood and oxygen to the entire body.

Under normal conditions, red blood cells are round and flexible, making it easy for them to move through blood vessels. While in sickle cell anemia, red blood cells are shaped like sickles, stiff, and prone to blocking small blood vessels, thereby hindering the supply of healthy blood and oxygen needed by the body.

Sickle Cell Anemia Causes

Sickle cell anemia is caused by changes in the gene that instructs the body to produce hemoglobin, an iron-rich compound in red blood cells that enables these cells to carry oxygen from the lungs to the rest of the body.

A child suffering from sickle cell anemia is born to parents who each carry one copy of the sickle cell gene and pass both copies to the child.

Sickle Cell Anemia Symptoms

Sickle cell anemia typically appears in children around 6 months of age. Symptoms vary from person to person and can change over time. According to the Mayo Clinic, symptoms can include:

1. Lack of red blood cells

The fixed cells are prone to breakage and death. Normal red blood cells usually live for about 120 days before they need to be replaced. However, fixed cells typically die within 10 to 20 days, leading to a shortage of red blood cells.

Without enough red blood cells, the body cannot get enough oxygen. This leads to fatigue.

2. Often experiences pain

If the child often becomes irritable, they may be experiencing periodic extreme pain episodes, which are the main symptoms of sickle cell anemia. Pain develops when the crescent-shaped red blood cells block blood flow through small blood vessels to the chest, abdomen, and joints.

The intensity of pain can vary and may last for several hours to several days. In some cases, only a few pain crises occur per year. Severe pain episodes require hospitalization.

Some people with sickle cell anemia also frequently experience chronic pain due to bone and joint damage, ulcers, and other causes.

3. Swelling in hands and feet

Red blood cells shaped like sickles block blood circulation in the hands and feet, which can cause them to swell.

4. Frequently experiences infections

Cleanliness is important to protect against infection. However, sickle cells can damage the spleen, increasing the risk of infection.

Patients with sickle cell anemia usually receive vaccinations and antibiotics to prevent life-threatening infections, such as pneumonia.

5. Barriers to growth or puberty

Red blood cells provide oxygen and nutrients that the body needs for growth. A deficiency of healthy red blood cells can slow growth in infants and children and delay puberty in adolescents.

6. Vision problems

Small blood vessels that supply blood to the eye can become blocked by sickle cells. This can damage the part of the eye that processes visual images, called the retina, and cause vision problems.

So, when should you visit a doctor?

Professor Harapan Parlindungan Ringoringo said, if you or your child experience symptoms of anemia such as pallor, fatigue, frequent fever, and shortness of breath, you should see a doctor immediately.

A child with sickle cell anemia often experiences infections, which are frequently preceded by fever and can be life-threatening.

Because children with sickle cell anemia are prone to infections, seek medical help immediately if their fever is above 38.5 degrees Celsius.

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