SICKLE CELL DISEASE

Cheryl Bitting

What is Sickle Cell Disease? Sickle Cell Disease (SCD) is an inherited

blood disorder that affects red blood cells

People with SCD have blood cells that contain mostly hemoglobin*S, an abnormal type of hemoglobin

There are several types of SCD: the most common are Sickle Cell Anemia (SS) and Sickle Hemoglobin C disease (SC)

What’s the problem with Sickle Cells?

Sickle-shaped cells block small blood vessels, allowing less blood to reach different parts of the body

Abnormal blood flow damages tissue causing the complications of SCD

What happens in the body?

Organ or tissue involved Problem

Kidney bed-wetting, blood in the urine, kidney failure

Hands & Feet Swelling and pain

Spleen increased risk for serious infectionsSplenic sequestration (large amount of body's blood pooled in the spleen)

Lungs Pneumonia, Acute chest syndrome

Skin Slow - healing sores on legs and ankles

Bones Infection, Bone damage

Brain Stroke, Headache

Penis Painful unexpected erection

Eyes Vision problems

Liver Increased size, Gallstones, Gives yellow color to eyes and skin

What is a sickle cell crisis?

A sickle cell crisis happens when sickled red blood cells block small blood vessels that carry blood to the bones.

This causes pain that can begin suddenly and last several hours to several days. The person might have pain in their back, knees, legs, arms, chest or stomach.

The pain can be throbbing, sharp, dull or stabbing. How often and how bad the pain gets varies a lot

from person to person and from crisis to crisis.

What is the treatment or cure for SCD?

The only cure for Sickle Cell Disease is bone marrow transplants, but it is risky and has lots of complications

Some promising treatments: hydroxyurea, blood transfusions, pain relievers, etc.

Young children under 5 years take penicillin twice daily to prevent pneumonia.

Who is affected by Sickle Cell Disease?

SCD affects approximately 72,000 people in the US Predominantly African-American (1 in 500 births) Hispanic Americans (1 in 1000-1400 births) SCD is also present in Portuguese, Spanish, French

Corsicans, Sardinians, Sicilians, mainland Italians, Greeks, Turks, and Cypriots. Sickle Cell Disease also appears in African countries, Middle Eastern countries and Asia.

What should a teacher do?

Be informed about Sickle Cell Disease Anticipate more missed school days Allow extra water and bathroom breaks Pay attention to temperature control Be aware of the need to rest Don’t assume lesser intelligence Student may need special services Know the symptoms of strokes

What are the symptoms of strokes?

Difficulty with memory

Difficulty using a hand, a leg, or one side of the face due to muscle weakness

Numbness or tingling

Difficulty with balance

Difficulty with vision or hearing

Difficulty speaking or understanding what other people are saying

Unusual headache

Sickle cell disease is one of the few conditions that can cause stroke(brain injury) in children. Notify the child's family at once if any of the following symptoms appear without another explanation:

For more information: Duke Comprehensive Sickle Cell Center

http://www.sicklecell.mc.duke.edu/services/resourcesaboutsicklecell.html

Sickle Cell Disease: A Resource for the Educator http://www.dshs.state.tx.us/newborn/educate.shtm

NC Sickle Syndrome Program http://www.ncsicklecellprogram.org/

Dr. Spock - Understanding Sickle Cell Disease: Instructions for Teachershttp://www.drspock.com/article/0,1510,5176,00.html

Sickle Cell Disease Association of America, Inc http://www.sicklecelldisease.org/index.phtml