What is Sickle Cell

 
Sickle Cell Disease is a group of inherited red blood cell disorders. Normal red blood cells are round like doughnuts Healthy Cell, and they move through small blood tubes in the body to deliver oxygen. Sickle red blood cells Sickle Cell become hard, sticky and shaped like sickles used to cut wheat. When these hard and pointed red cells go through the small blood tube, they clog the flow and break apart. This can cause pain, damage and a low blood count, or anemia.
 
What makes the red cell sickle? There is a substance in the red cell called hemoglobin that carries oxygen inside the cell. One little change in this substance causes the hemoglobin to form long rods in the red cell when it gives away oxygen. These rigid rods change the red cell into a sickle shape Sickle Cell instead of the round shape Healthy Cell.
 
How do you get sickle cell disease or trait? You inherit the altered hemoglobin from your parents, who may be carriers with sickle cell trait or parents with sickle cell disease. You can not catch it. You are born with the sickle cell hemoglobin and it is present for life. If you inherit only one sickle gene, you have sickle cell trait. If you inherit two sickle cell genes you have sickle cell disease.
 
Is Sickle Cell only in African Americans? Sickle cell is in many nationalities including African Americans, Arabs, Greeks, Italians, Latin Americans, and those from India. You can be Caucasian and have sickle cell disease or trait. All races should be screened for this hemoglobin at birth.
 
How can I be tested? A simple blood test called the hemoglobin electrophoresis can be done by your doctor or local sickle cell foundation. This test will tell if you are a carrier of the sickle cell trait or if you have the disease.
 
Newborn Screening Most States now perform the sickle cell test babies are born. The simple blood test will detect sickle cell disease or sickle cell trait . Other types of traits that may be discovered include:
  •  Hemoglobin C trait 
  •  Hemoglobin E trait 
  •  Hemoglobin Barts - which indicates an alpha thalassemia trait
  •  Beta thalassemia trait
Are there different types of sickle cell disease? There are three common types of sickle cell disease in the United States.
  • Hemoglobin SS or sickle cell anemia 
  •  Hemoglobin SC disease
  • Hemoglobin sickle beta-thalassemia Each of these can cause sickle pain episodes and complications, but some are more common than others.
All of these may also have an increase in fetal hemoglobin which can protect the red cell from sickling and help prevent complications. The medication hydroxyurea also increases fetal hemoglobin.
 

What is sickle cell trait? Sickle cell trait is found in a person who carries one sickle hemoglobin producing gene inherited from their parents and one normal hemoglobin gene. Normal hemoglobin is called type A. Sickle hemoglobin called S. Sickle cell trait is the presence of hemoglobin AS on the hemoglobin electrophoresis. This will NOT cause sickle cell disease. Other hemoglobin traits common in the United States are AC and AE traits.

 

How do you get sickle cell trait? You inherit the altered hemoglobin from your parents, who may be carriers with sickle cell trait or parents with sickle cell disease. You can not catch it. You are born with it, like eye color or hair color, and it is present for life. If you inherit only one sickle gene, you have sickle cell trait. If you inherit two sickle cell genes you have sickle cell disease.

 

Is sickle cell trait only in African Americans? Sickle cell trait (HbS) and other hemoglobinopathies (HbC, HbE, etc.) may be found in individuals whose ancestors come from East India, Africa, the Mediterranean, the Middle East, the Caribbean, Central and South America, or South East Asia. You can be Caucasian and have sickle cell trait. All races should be screened for this hemoglobin at birth.

 

How can I be tested? A simple blood test called the hemoglobin electrophoresis can be done by your doctor or local sickle cell organization. This test will tell if you are a carrier of the sickle cell trait or if you have the disease.

 

Newborn Screening Most States now perform the sickle cell screening when babies are born. The simple blood test will detect sickle cell disease or sickle cell trait . Other types of traits that may be discovered include:

 

  • Hemoglobin C trait
  • Hemoglobin E trait
  • Hemoglobin Barts - which indicates an alpha thalassemia trait
  • Beta thalassemia trait

 

Why is it important to know if you have a hemoglobin trait?
 
A person inherits genes that produce hemoglobin from his/her parents. It is important to identify people with a hemoglobin trait so they will be aware of their risk of having children with sickle cell disease.

If one parent has sickle cell trait and the other parent has normal hemoglobin, there is a 50 percent (1 in 2) chance with each pregnancy that the child will be born with sickle cell trait.

If both parents have sickle cell trait or another hemoglobin trait, there is a 25 percent chance (1 in 4) with each pregnancy that the child will have sickle cell disease.

Healthy Guidelines
 
Get regular checkups from knowledgeable health care providers.

If your baby has a sickle cell disorder, be sure he or she receives the appropriate immunizations at the correct intervals.

Visit a genetic counselor; counseling and education regarding the trait are important because the sickle gene can be passed to a carrier’s children.

Be sure children with sickle cell disease begin taking penicillin by 3 months of age and continue until at least 5 years of age.

Take multivitamins and folic acid supplements.

Drink plenty of water daily (8-10 glasses).

Avoid over-exertion and stress.

Avoid too hot or too cold temperatures.

While a special diet is not necessary, eat three well-balanced meals each day.

Keep a positive mental attitude.