Sickle-Cell Anemia 101

By: Ciara Page

Introduction

What even is Sickle-Cell Anemia? Sickle-Cell is a condition where there is not enough red blood cells to carry oxygen throughout the body. In result, the red blood cells become sticky and shaped like crescent moons or sickles unlike normal red blood cells which are round and flexible. The irregular shape of cells can lead to blockage of blood flow and oxygen in many parts of the body. There is no cure for this disease, so we should learn about it to one day find a cure for Sickle-Cell patients.

Causes

You can get Sickle-Cell through two simple words: genetic mutations. Sickle-Cell is caused by a mutation in the genes that tells your body to make hemoglobin. Hemoglobin is a compound that gives your blood its red color. The mutation makes abnormal hemoglobin in which results in the irregular shape of the cells. However the only way a child can be affected by Sickle-Cell is if both the mother and the father have the Sickle-Cell trait. If only one parent has the trait, the trait will simply be passed down with no symptoms.

Morality Rate

According to Lanzkron, Carroll, and Haywood, "We identified 16,654 sickle cell-related deaths. The median age at death in 2005 was 42 years for females and 38 years for males. The overall mortality rate increased 0.7% each year during the time period studied. The adult (>19 years of age) mortality rate increased by 1% each year during the time period studied. The pediatric mortality rate decreased by 3% each year during the time period studied." Although the last study was conducted in 2002, this rate is increasing in adults which isn't good for patients presently and in the future.

Symptoms

Symptoms of Sickle-Cell may not appear in a child until four months old, but there are six main symptoms. The first is symptom is anemia. Anemia is a symptom because sickle cells die and break easily, only living about twenty days. Normal red blood cells live 120 days, which means Sickle-Cell patients have a major shortage of red blood cells in result gives the feeling of fatigue all the time. The next symptom is bursts of pain (crises). This is the major symptom of Sickle-Cell which is the result of blockage in the blood vessels. The pain can last for a few hours or up to weeks, and occur every so often, or more than a dozen times a year. The third symptom is Hand-foot syndrome. This means your hands and feet are super swollen due to the blocking of blood flow to the hands and feet. Then the fourth symptom is frequent infections. Normally your spleen can fight off infections, but because the sickle cells damage your spleen, patients are more prone to infection. Doctors usually give infants and children with Sickle-Cell vaccinations, but that doesn't mean they will not get the infection. The fifth symptom is delayed growth. Delayed growth occurs because the shortage of red blood cells in which affects children and teens. The sixth symptom is vision problems. Sickle cells can block the tiny blood vessels in your eyes which hurts the retina and can permanently damage the patient's vision. Unfortunately these symptoms are not the only ones Sickle-Cell patients go through, and there are many more that give negative effects just like these six.

Treatment

There is no cure for Sickle-Cell Anemia, but there are treatments. One option is a bone marrow transplant, but finding donors are not common, risks are high, and death is a possibility. However since this treatment is not common, treatment centers normally focus on relieving symptoms. These treatments include medications, blood transfusions, and supplemental oxygen. There are three main types of medications Sickle-Cell patients take as a treatment. The first is antibiotics. Antibiotics can be for children aged two months to five years old with the sole purpose of preventing infections, or for adults to just fight infections. The second medication is pain-relieving medications. These are just over-the-counter medications to help with bursts of pain. The third medication is Hydroxyurea. Hydroxyurea is a medication which reduces the frequency of pain, reduces the need of blood transfusions, and gives off the production of fetal hemoglobin (hemoglobin that prevents the formation of sickle cells). However the medication increases the risk of infection, and long term use can result in tumors or leukemia.

Treatment Center for Children

Emory University Department of Pediatrics

- There are many treatment centers for Sickle Cell patients, including seventeen in Georgia. Out of those seventeen, seven are specifically for children.

Treatment Centers for Adults

Medical College of Georgia Adult Sickle Cell Center

-This is one of seventeen treatment centers in Georgia for Sickle Cell patients. Ten out of the seventeen are for adults.

Some Visuals of Sickle-Cell Anemia