An Inside Look:

My mother, Cynthia L. Graham worked in a doctor's office for several years and had to test children for Sickle Cell Disease. When asked in detail about what she did, she said,

"It was horrible. We inserted a capillary tube into their finger, and the blood shot up. Then, we mixed the blood with a solution. If it separated from the solution, the child had the trait or had the disease."

What IS Sickle Cell Disease?

Sickle Cell disease (also known as Hemoglobin SS disease, Hemoglobin S disease, HbS disease, or SCD) is a condition in which the normal red blood cells in the body are replaced by blood cells shaped like sickles, or 'C's.

A sickle is a tool used in agriculture to harvest crops. The Sickle Cell gets it's name from it's shape that is similar to the sickle.

Inheritance and Causes:

Sickle Cell Disease is an inherited disease. Since it takes two Sickle Cell genes to make a person have the disease, we know that this trait is recessive.

If two parents, each with one Sickle Cell gene and one normal gene, were crossed, they would have a 25% chance of their offspring having Sickle Cell disorder. This cross is shown in the Punnet square below.

Normal red blood cells turn sickle shaped because the Sickle Cell gene causes them to produce an abnormal hemoglobin (the protein in blood cells that carries oxygen to different parts of the body.

Diagnosis:

It's very important to diagnose children early so they can be treated. A blood sample is taken to determine if the person has the Sickle Cell trait or has the disease.

Another test is done to verify. Scinetists observe how the hemoglobin moves within an electric field. The patient is then diagnosed.

Infants can be diagnosed before birth. A sample of amniotic fluid is taken from the placenta, and scientists look for the Sickle Cell gene.

Early diagnosis is important. If an infant or child is diagnosed early enough, doctors can work towards treating the disease.

Symptoms:

Symtoms that affect sufferers of Sickle Cell Disease are:

~Anemia-fatigue, pale skin and nails, jaundice, and shortness of breath.
~Sickle Cell Crisis- over whelming pain caused by the clumping of Sickled Cells in small vessels.
~Pain/Swelling in the feet.
~Damaged spleen
~Easily caught infections
~Chest pain
~Fever
~Delayed growth
~Strokes
~Weak retina (may lead to blindness)
~Piriapism
~Gallstones and symptoms associated with them.
~Leg ulcers
~High blood pressure
~Multiple organ failure (rare)

Treatments:

Although the known symptoms can be treated with medicine, there is no known cure. People with the disorder have regular medical care. Doctors specializing in Sickle Cell are hematologists (doctors specializing in blood and blood disorders).

Doctors aim to relieve pain and prevent strokes, infections, and eye problems.

Pain is handled with over the counter medicines and heat pads.

Crises are prevented with daily doses of pennicillin, vaccinations, and a yearly flu shot.

Regular checkups prevent eye damage.

Ultra sounds of the head to monitor brain activity related to strokes.

To treat serious Sickle Cell symptoms, blood tranfusions can be performed. Some side effects of this procedure include building up of iron in the blood, and an increased chance of infection.

Promising cures may lie in bone marrow transplants, although they are risky and may even lead to death. Because of this, only some victims should consider this option.

Living with Sickle Cell Disease:

Sickle Cell Disease can be handled with medicine, but you must maintain a healthy lifestyle by:

~Eating healthy
~Staying hydrated
~Exercise
~Adequate sleep
~Avoiding drinking and smoking

Make sure to get regular checkups, and inform your doctor if any sudden changes in health occur. Talk with your doctor about pain management, as it is different for each person.