What is Sickle Cell Disease?

Sickle cell disease is a genetic, life threatening disorder that affects the red blood cells. Normal red blood cells are round, soft and flexible, but sickle cells are crescent-shaped, hard and rigid. These cells cause blockage in the blood vessels leading to organs throughout the body which causes terrible pain and various complications.

The most severe and common form of the disease is called Sickle Cell Anemia in which two inherited genes produce an abnormal hemoglobin (SS). Normal red blood cells live about120 days in the blood stream, but sickle cells die after 10-12 days. Since the body is unable to reproduce cells fast enough, it causes a shortage in red blood cells, a condition called anemia. There are other less severe types of the disease as well. Approximately 1 out of every 400 African-American infants in the United States are born with sickle cell disease.

Symptoms of Sickle Cell Disease

The most common complaint is severe pain, which may be chronic or occasional. An acute pain attack, usually accompanied by other symptoms, is called a Sickle Cell Crisis.

Shortness of breath
Dizziness
Headaches
Coldness in the hands and feet
Paler than normal skin or mucous membranes (the tissue that
lines your nose, mouth, and other organs and body cavities)
Jaundice (a yellowish color of the skin or whites of the eyes)
Severe infections

Complications with Sickle Cell Anemia

The most common complaint is severe pain, which may be chronic or occasional. An acute pain attack, usually accompanied by other symptoms, is called a Sickle Cell Crisis.

Hand-Foot Syndrome

Sickle cells can block the small blood vessels in the hands and feet in children (usually those younger than 4 years of age). This condition is called hand-foot syndrome. It can lead to pain, swelling, and fever.

Swelling often occurs on the back of the hands and feet and moves into the fingers and toes. One or both hands and/or feet may be affected at the same time.

Splenic Crisis

The spleen is an organ in the abdomen. Normally, it filters out abnormal red blood cells and helps fight infections. In some cases, the spleen may trap red blood cells that should be in the bloodstream. This causes the spleen to grow large and leads to anemia.
If the spleen traps too many red blood cells, you may need blood transfusions until your body can make more cells and recover.

Infections

How Do You Get Sickle Cell Disease

A person is born with sickle cell disease based on genes that produce hemoglobin. There are two genes that determine the hemoglobin; one comes from each parent. A person without the disease may still have the Sickle Cell Trait which is a carrier of one gene that produces normal (A) hemoglobin and one that produces the abnormal (S) hemoglobin, thus (AS). In people with sickle cell anemia (SS disease), both genes produce S hemoglobin. The other forms of sickle cell disease would be a combination of S and C hemoglobin (SC disease) or S and beta thalassemia (S-beta thalassemia disease).

In order to have the disease, both parents must have at least one gene that produces S hemoglobin. Below is a diagram of possible outcomes if both parents have the Sickle Cell Trait (Unaffected "Carriers" of the gene producing S hemoglobin [r]).

scd-autorecessive inheritance diagram

Emergency Preparedness: Home and Workplace Preparedness

Preparedness is More than Being Prepared for a Disaster

Preparedness Equals “NOW” (No opportunity Wasted or No other Way)

Developing a Disaster Plan

A disaster plan can mean the difference between life and death. For example:

How will you escape your home?
Where will you meet family members?
What route will you take out of your neighborhood if evacuation cecomes necessary?

The CAAHAz Wants You to Know...

In 2008, African Americans accounted for approximately 13.5% of the population.
48% of African Americans suffer from chronic diseases, compared to 39% of the general population.
70% of African Americans between the ages 18 to 64 are obese or overweight
African Americans are more likely to develop and die from cancer than any other racial or ethnic group.
African American men are 50% more likely than Whites to have prostate cancer.
African American babies are 2.1% more likely to die from Sudden Infant Death Syndrome than Whites.
African Americans experience new HIV infections at 7 times the rate of Whites.

These are just a few of the health disparities in the African American community. Health disparities are preventable differences in health issues by socially disadvantaged populations. Don't become a statistic, let's Close the Gap!

Get Regular Checkups • Eat a Healthy Diet • Excercise • Stop Smoking

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