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Sickle Cell Disease in Children

Sickle cell disease (SCD) is a blood disorder that a child is born with. It’s passed down through a parent’s genes. Children with SCD make an abnormal type of hemoglobin. This is the protein in red blood cells that carries oxygen to all parts of the body. With SCD, the body organs and tissues don’t get enough oxygen. When a child has SCD, the red blood cells are hard and sticky. They are shaped like the letter C (and like a farm tool called a sickle). These damaged red blood cells (sickle cells) clump together. They can’t move easily through the blood vessels. They get stuck in small blood vessels and block blood flow. This blockage can cause pain. It can also damage major organs.

There are several complex types of the sickle cell gene. Some don’t cause symptoms or severe problems, but others do. Talk to your child’s healthcare provider about the specific form of sickle cell your child has. Most children with SCD will start showing symptoms during the first year, often around 5 months.

Why do People have SCD?

Sickle cell is present at birth. It is inherited when a child has two sickle cell genes, one from each parent.(i.e AS, AS). A child who has only one sickle cell gene is healthy. But he or she is a carrier of the disease. If two carriers have a child, there is a greater chance their child will have sickle cell disease.

What are the symptoms?

Symptoms start to show at an early age often around 5 months. Each child’s symptoms may vary. They may be mild or severe. Symptoms can include:

Anemia. This is the most common symptom. Having fewer red blood cells causes anemia. Anemia can make a child pale and tired.

Yellowing of the skin, eyes and mouth (jaundice). Sickle cells don’t live as long as normal red blood cells. They die faster than the liver can filter them out. The yellow color is caused by a substance (bilirubin) that is released when the red blood cells die.

Pain crisis, or sickle crisis. When sickle cells move through small blood vessels, they can get stuck. This blocks blood flow and causes pain. This sudden pain can happen anywhere, but most often occurs in the chest, arms and legs. Babies and young children may have painful finger and toe swelling.

Acute chest syndrome. This is when sickle cells stick together and block oxygen flow in the tiny vessels in the lungs. This can be deadly. It often occurs suddenly, when the body is under stress from infection, fever, or fluid loss (dehydration). It looks like pneumonia and can include fever, pain and a violent cough.

How do I know if my Child has SCD?

Diagnostic procedures for sickle cell may include blood tests, a complete medical history, physical examination and complete family history and results of newborn screening- usually done by experienced medical professionals.

Early diagnosis is essential to providing proper preventive treatment for some of the devastating complications of the disease.

What can I do if my Child is diagnosed with SCD?

Treatment will depend on your child’s symptoms, age and general health. It will also depend on how severe the condition is.

Early diagnosis and preventing further problems is important in treating this disease. Your child’s healthcare provider will refer you to a hematologist. This is an expert in blood disorders. Other specialists may also be involved in your child’s care.

Get in touch with our hematologists if you will like to know more about diagnosis and treatment for Sickle Cell Disease.

To book an appointment to see a haematologist, e-mail frontdesk@thelecc.com or call us.