Sickle Cell Disease

Introduction

Sickle Cell Disease (SCD) refers to a category of hereditary blood disorders. The conditions are characterized by the lack of healthy red blood cells to carry oxygen throughout the body.

Healthy red blood cells are spherical and disc-shaped. The red blood cells of an individual suffering from sickle cell anemia will be sickle-shaped. Such cells will subsequently block blood vessels and restrict blood flow to the heart.

Causes

A mutation in the HBB gene contributes to the development of SCD. The HBB gene contains instructions for cells to make a part of a protein called hemoglobin. When this gene is defective, the hemoglobin changes its form into stiff rods. This will cause the red blood cells to become stiff and sickle-shaped. Such abnormal cells will 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

Anemia: In sickle cell anemia, 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 anemia. 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: Swelling, in conjunction with a fever, is a result of sickle cells getting stuck in the blood vessels and blocking the flow of blood in and out of 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 is incurable and 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

Patients are our number one priority. We are committed to their health and wellbeing. It is this dedication that guides us to give our very best, and our multidisciplinary team of trained professionals work together to ensure quality care. If you’re showing any symptoms of Sickle Cell Disease listed above, consult the team of experts at Haemato Oncology Care Centre (HOCC) without any delay.

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