Children's Health

Sickle Cell Anemia

Sickle cell anemia is one group of inherited disorders known as sickle cell disease. It affects the shape of red blood cells, which are responsible for carrying oxygen to all parts of the body.

Red blood cells are typically round and flexible, so that they move easily through the blood vessels. In sickle cell anemia, some red blood cells become sickle-shaped, like crescent moons. They also become rigid and sticky, which can slow or stop blood flow. There are currently no cures, but there are treatments available to relieve pain and reduce the risk of complications.

What causes Sickle Cell Anemia?

Sickle cell anemia is an inherited disorder. It is caused by a change in the gene that tells the body to make the iron-rich compound, hemoglobin, in red blood cells. Hemoglobin enables red blood cells to carry oxygen from the lungs throughout the body. The hemoglobin in sickle cells causes red blood cells to become rigid, sticky, and misshapen.

For a child to be affected, the mother and the father have to be carriers of the sickle cell gene, known as the sickle cell trait, and pass both copies of this gene to their child. If only one copy of the gene passes to the child, then the child becomes a carrier of the sickle cell trait. These people with one altered gene make both typical hemoglobin and sickle cell hemoglobin. They generally don’t experience symptoms, but they can pass the gene to their children.

In the United States, sickle cell typically affects people of African, Mediterranean, and Middle Eastern descent.

What are the symptoms of Sickle Cell Anemia?

Signs and symptoms of sickle cell anemia include:

• Anemia – Sickle cells break apart easily and die. Red blood cells usually live for approximately 120 days before they need to be replaced. Sickle cells usually die in 10-20 days leaving a shortage of red blood cells. Without enough red blood cells, the body can’t get enough oxygen causing fatigue.

• Pain episodes – Periodic episodes of extreme pain, known as pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood

cells block blood flow through tiny blood vessels to your chest, abdomen and joints. The pain varies in intensity and can last for a few hours to a few days. Some people have only a few pain crises a year. Others have a dozen or more a year. A severe pain crisis requires a hospital stay. Some adolescents and adults with sickle

cell anemia also have chronic pain, which can result from bone and joint damage, ulcers, and other causes.

• Swelling of hands and feet – The swelling is caused by sickle-shaped red blood cells blocking blood circulation in the hands and feet.

• Frequent infections – Sickle cells can damage the spleen, increasing vulnerability to infections. Infants and children with sickle cell anemia commonly receive vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia.

• Delayed growth or puberty – Red blood cells provide the body with the oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.

• Vision problems – Tiny blood vessels that supply the eyes can become plugged with sickle

cells. This can damage the retina — the portion of the eye that processes visual images — and can lead to vision problems.

What are the treatment options at Compounding Solutions Pharmacy & Wellness?

Hydroxyurea suspension – Hydroxyurea is only available as capsules commercially. At Compounding Solutions Pharmacy & Wellness, we can compound it into a great tasting suspension made with your child’s favorite flavor. By combining flavor and an easy to take liquid, you can rest assured that your child will not mind

taking their medicine. Daily hydroxyurea reduces the frequency of painful crises and might reduce the need for blood transfusions and hospitalizations. However, it can increase the risk of infections.