What is sickle cell disease?

Sickle cell disease (SCD) is a group of hereditary blood disorders. Individuals with SCD don’t have enough healthy red blood cells to carry oxygen throughout the body.

Healthy red blood cells are spherical and disc-shaped. When a person has sickle cell anaemia, their red blood cells change shape and become like a sickle. Over time, these sickle-shaped cells will block blood vessels and restrict blood flow to vital organs like the heart.

Causes

Sickle cell disease results from a mutation in the HBB gene. The HBB gene contains instructions for cells to make a part of a protein called haemoglobin. Because of a defective gene, the haemoglobin forms into stiff rods, causing the red blood cell to become rigid and sickle-shaped.

Such sickle-shaped cells can stick to the walls of the blood vessels, block blood flow, and constrict the supply of blood to surrounding tissues.

Symptoms

SCD has a wide range of symptoms

• Anaemia: In sickle cell anaemia, a person’s healthy red blood cells die. Even sickle cells readily disintegrate and die, leaving the individual with an insufficient number of red blood cells. Red blood cells last approximately 120 days before needing to be replaced. Sickle cells die in 10-20 days, leaving a shortage of red blood cells.
• Painful episodes: Pain crises, or periodic episodes of pain, are a common symptom of sickle cell anaemia. Pain occurs when sickle-shaped red blood cells impede blood flow to your chest, abdomen, and joints through tiny blood channels. Bone pain is also a possibility.
• Swelling of hands and feet: Sickle-shaped red blood cells limit blood flow to the hands and feet.
• Frequent Infections: Your spleen and other organs may be damaged by sickle cells, making you more susceptible to infections.
• Puberty or delayed growth: Red blood cells transport oxygen and nutrients throughout your body, allowing you to thrive.
• Vision problems: Sickle cells can clog the tiny blood arteries that supply blood to your eyes. This can cause vision issues by damaging the retina, which is the part of the eye that interprets visual images.

Complications

Sickle cell disease can cause a variety of other problems:

• Bone and joint pain
• Strokes
• Acute chest syndrome (accompanied by fever, cough, chest pain, and breathing difficulties)
• Kidney or urinary problems
• Pulmonary hypertension
• Gallstones (which may lead to jaundice)

Treatment

SCD requires lifelong treatment. A bone marrow or stem cell transplant can treat the condition. It’s a risky procedure and is only performed on patients with severe SCD.

In this procedure, doctors transfer stem cells from a healthy donor into the patient’s vein. These new cells will begin to produce new red blood cells to replace the sickle cells.

There also are treatments that can control symptoms and reduce complications:

• Pain relievers
• Antibiotics
• Childhood vaccinations
• Blood transfusions

Keeping track of your blood health is essential. HOCC provides comprehensive treatment for patients with blood disorders and blood cancers. If you’re showing any symptoms, consult the team of experts at HOCC without any delay.

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