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The following information about sickle cell is courtesy of www.sicklecellontario.com and www.aboutkidshealth.ca.

What is it:

Sickle cell anemia, which is just one type of sickle cell disorder, is an inherited condition that can be life-threatening.

Sickle cell anemia occurs when an abnormal form of hemoglobin, a protein found in red blood cells that helps transport oxygen, is produced in the body. Normal red blood cells are shaped like doughnuts and are soft and flexible, but sickle cell anemia causes these cells to become sticky, soft and fragile. Instead of moving through the blood stream easily, these cells can clog blood vessels and deprive vital organs of the oxygen they need.

Symptoms:

Sickle cell anemia causes chronic pain and swelling in the joints, fever and respiratory infections. There is no cure for sickle cell anemia.

Most children and adults with sickle cell anemia have periods of no symptoms (called remissions) between crises. The most common symptom of a sickle cell crisis is pain, which is usually felt in the part of the body where the blood is clotting.

Sickle cell anemia can cause the skin to appear pale as the anemia worsens. The paleness may be most obvious on the inside of the eyelids, under fingernails and in the creases of the palms of the hands. The skin may also turn yellow.

One of the life-threatening complications of sickle cell disease can be sudden, severe anemia.

This severe shortage of normal red blood cells may cause weakness, shortness of breath or even heart failure. The symptoms of shock caused by heart failure are low blood pressure, rapid pulse and decreasing consciousness.

Other symptoms can include:

Fatigue

Weakness

Joint pain

Bone pain

Abdominal pain

Fever

Susceptibility to infections

Ulcers on lower legs

Delayed growth and puberty

Rapid heart rate

Hematuria (bloody urine)

Excessive urination

Excessive thirst

Penis pain

Priapism

Chest pain

Decreased fertility

How is it treated?

People with sickle cell anemia are more likely to have serious infections. Prevention of serious infections is an important part of the treatment.

To help prevent infections in your child you should:

* Update immunizations.

* Get the flu shot yearly.

* Get the pneumococcal vaccinations to protect against a common type of pneumonia.

* Check about the possibility of other vaccines including hepatitis A and B, chickenpox, and meningococcus.

* Take a daily multiple vitamin to help the body make new red blood cells.

* Contact the doctor whenever the person with sickle cell anemia becomes sick, even with minor illness.

What happens:

A child with sickle cell anemia will receive IV fluids and pain medication. Rest is also required. As many red blood cells may be destroyed during a crisis, he may need a blood transfusion.

Complications:

Complications can occur in any part of the body. Frequently occurring problems include:

infections such as pneumonia or meningitis

kidney infections

bone infections

gallstones

poorer vision from damage to blood vessels in the eyes

hip and shoulder joint damage

stroke

damage to tissues in the penis, called priapism

Genetics:

When both parents are carriers, each child has a 25 per cent risk of having sickle cell anemia and a 50 per cent risk of being a carrier.

Screening programs are available to identify sickle trait carriers.