Sickle Cell Disease

What is Sickle Cell Disease?

Sickle Cell Disease is an inherited blood disorder that affects a person's red blood cells. It is a chronic illness passed from the parents to the children. Sickle cell anemia is believed by some scientist to have originated in other parts of the world such as: Africa, Mediterranean, Middle East and India. It is believed that the body began an evolutionary process whereby with the cells sickling it became a protective mechanism to fight malaria. (Note: People with the sickled cells often survived the Malaria Outbreaks.) With migration, sickle cell disease spread throughout the world.

There are approximately 70,000 cases of sickle cell disease in the United States. There are approximately 2,000,000 people with the sickle cell trait in the United States. 1 out of 400 African Americans have Sickle Cell Disease. 1 out of 12 African Americans has the Sickle Cell Trait. Many people are affected by sickle cell disease such as: African Americans, Hispanics Mediterranean, Central, South Americans, Middle Easterners, Africans, and East Indians to name a few.

Some Sickle Cell Disease Facts:

Normal red blood cells are donut shaped, soft, flexible and move easily through the blood vessels. Their purpose is to carry nutrition and oxygen and help remove waste products and last up to 120 days in the blood stream. Sickled red blood cells are curved, sickled or crescent shaped irregular, sticky and tend to clump together. They usually last 10-20 days and lose the ability to carry vital oxygen and tend to block the blood vessels and cause damage. This blockage can cause pain, infection and anemia and lead to more serious damage within the body. There are several types of sickle cell disease. The main ones are sickle cell disease (SS), sickle hemoglobin C (SC), sickle beta plus thalassemia, sickle beta zero thalassemia.

Diagnostic Testing:

Diagnostic testing is done with newborn screening, adult screening blood test and pregnancy screening blood test called the hemoglobin electrophoresis.

Signs & Symptoms:

Weakness

Fatigue

Shortness of breath

Severe back, abdominal and leg pain

Swelling of the hands and feet

Jaundice

Frequent infections

Growth Retardation

Complications:

Sickle cell crisis

Anemia

Acute chest syndrome Infections

Organ damage

Vision problems

Leg ulcers

Spleen blockage and damage

Hand foot syndrome

Stroke

Gall stones

Neurological dysfunction

Priaprism or impotence

Death

The life expectancy is 40's to 50's. The medical community treatment objectives are to 1. Avoid Crisis, 2. Relieve Symptoms, 3. Prevent Complications.

Treatments:

The traditional treatments include: Medications, Blood transfusions, supplemental oxygen antibiotics, hydration, hydroxyurea, and bone marrow transplant. Some of the experimental treatments in research are: gene therapy, butyric acid, clotrimazole and nitric oxide.

Prevention & Self Care:

Regular Physician Visits

Avoid Stress Vaccinations

Exercise Regularly

Folic Acid

Promptly treat cuts and Insect bites

Balanced Diet

Avoid Alcohol, Cigarettes and drugs

Avoid Temperature Extremes

Fly in pressurized passenger cabins

Avoid High Altitudes

Genetic Counseling

Coping Skills:

Manage Stress Obtain Counseling Join Support Groups Ask Questions

Resources:

Sickle Cell Awareness Group

3770 Reading Road

Cincinnati, Ohio 45229

(513) 281-4450

dharrison@gcul.org

National Association of Sickle Cell

231 East Baltimore Street Suite 800

Baltimore, Maryland 21202

(800) 421-8453

scdaa@sicklecelldisease.org

The American Sickle Cell Anemia Association

10300 Carnegie Avenue

Cleveland, Ohio 44106

(216) 229-8600

www.ascaa.org

If you would like more information you can contact us at Health Initiatives Coordinator Greater Cincinnati Urban League 3458 Reading Road Cincinnati, Ohio 45229 TEL: (513) 281-9955.