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Hemoglobin Diseases

Sickle cell disease and sickle cell trait  
This page has been automatically translated from English. MSDH has not reviewed this translation and is not responsible for any inaccuracies.

Hemoglobin diseases are a group of blood disorders passed down through families in which there is an abnormal production or structure of the hemoglobin protein.

These hemoglobinopathies are inherited single gene disorders. Hemoglobinopathies affect the red blood cells' ability to carry oxygen from the lungs to other parts of the body. An example of hemoglobin disease is sickle cell anemia.

There are over 600 hemoglobin diseases that have been medically defined by the American College of Medical Genetics. The Mississippi Hemoglobinopathy Surveillance Registry reports the following diseases:

  • Hemoglobin Sickle Cell Anemia
  • Hemoglobin Sickle C Disease
  • Hemoglobin S/Beta + Thalassemia
  • Hemoglobin C/Beta + Thalassemia
  • Hemoglobin C Disease
  • Hemoglobin S/Beta 0 Thalassemia
  • Hemoglobin Sickle Cell Anemia + Barts
  • Hemoglobin C/Beta 0 Thalassemia
  • Hemoglobin Beta + Thalassemia
  • Hemoglobin Sickle C Disease + Barts
  • Hemoglobin Beta Thalassemia Intermedia
  • Hemoglobin E Disease
  • Hemoglobin E Disease + Barts

Sickle Cell Anemia – The Most Common Sickle Cell Disease

Sickle cell anemia is one of the most frequently occurring genetic disorders that affect the red blood cells. Sickle cell anemia mostly affects people of African descent but the disease can also affect people of Hispanic, Arabic, Indian or Mediterranean descent. Sickle cell anemia affects about 90,000 to 100,000 people in the United States, including one in every 500 African-American babies and one in every 36,000 Hispanic babies.

Sickle cell anemia can cause pain, infections and damage to body organs. The pain symptoms of sickle cell anemia appear during a period called a crisis. A crisis can last from a few hours to a week in length. Other symptoms of sickle cell anemia include fever; pain in hands, feet and joints; shortness of breath; pneumonia-like symptoms; dizziness; headache; sores on the legs and an enlarged spleen or liver. All babies born in Mississippi are tested for hemoglobin disease and trait. Babies with hemoglobin disease are referred to medical specialty care soon after birth for treatment.

Most Frequently Confirmed Hemoglobin Diseases in Mississippi

  • Sickle Cell Anemia: occurs when an abnormal hemoglobin S gene is inherited from both parents. The red blood cells are normally shaped like a disc but the distorted red blood cells of sickle cell anemia are shaped like crescents.
  • Hemoglobin SC Disease: occurs when an abnormal hemoglobin S gene is inherited from one parent and a hemoglobin C gene is inherited from the other parent.
  • Hemoglobin S in combination with Thalassemia: occurs when an abnormal hemoglobin S gene and a beta thalassemia gene are inherited. These genes interact to produce a form of sickle cell anemia called sickle beta thalassemia. There are two forms of this disorder:
      - Hemoglobin S/Beta + Thalassemia
      - Hemoglobin S/Beta 0 Thalassemia
  • Hemoglobin C/Beta + Thalassemia: occurs when an abnormal hemoglobin C gene is inherited from one parent and a beta-thalassemia gene is inherited from the other parent.

Penicillin in Sickle Cell Disease: Quick Facts for Parents

  • Infection is the leading cause of death in young children with sickle cell anemia.
  • Penicillin should be started by 2 to 3 months of age and stopped by your child's healthcare provider, usually by 5 years of age.
  • Taking penicillin by mouth twice daily can greatly decrease the risk of infection.
  • Liquid penicillin is often easier to give young children but is also available in a tablet form.
  • Liquid penicillin must be stored in the refrigerator and should not be kept any longer than 2 weeks.
  • Liquid penicillin should be measured using a dosing syringe given to you by your nurse or pharmacist; do not use a kitchen teaspoon because it is not accurate.
  • Penicillin tablets should be stored in a cool dry place; tablets may be kept as long as 4 months.

Hemoglobin Trait

Sickle cell trait is not a disease. Having a trait means a person has inherited the sickle cell gene from only one parent. Sickle cell trait affects 1 in 12 African-Americans in the United States. People with sickle cell trait usually do not have any symptoms and live a normal life. Approximately 3 million people living in the United States have sickle cell trait and many are unaware of their status.

Most people with sickle cell trait do not have any health effects because they have some normal A hemoglobin. However, some people with sickle cell trait can have health conditions that are similar to a crisis of sickle cell anemia. These episodes can happen when a person with sickle cell trait engages in strenuous activities or sports that cause the body to work harder than normal. Symptoms of an episode can include shortness of breath, coldness in the hands and feet, pale skin, chest pain and headaches. If you have sickle cell trait you may be at risk of having a child with sickle cell trait or sickle cell disease.

Most Frequent Confirmed Hemoglobin Traits in Mississippi

  • Hemoglobin S Trait: occurs when a normal hemoglobin A gene is inherited from one parent and a hemoglobin S gene is inherited from another parent.
  • Hemoglobin C Trait: occurs when a normal hemoglobin A gene is inherited from one parent and a hemoglobin C gene is inherited from another parent.
  • Hemoglobin D Trait: occurs when a normal hemoglobin A gene is inherited from one parent and a hemoglobin D gene is inherited from another parent.
  • Hemoglobin O Arab Trait: occurs when a normal hemoglobin A gene is inherited from one parent and a hemoglobin O Arab gene is inherited from another parent.

Should I Think About Being Tested?

You will not know if you have sickle cell trait unless you have a blood test. Ideally you and your partner should ask for a blood test before you plan to have a child if you do not know your status. If you have been told by a healthcare provider that you have sickle cell trait there is a chance you could pass the abnormal gene to your baby.

More Resources

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Last reviewed on Aug 4, 2017
Mississippi State Department of Health 570 East Woodrow Wilson Dr Jackson, MS 39216 866-HLTHY4U web@HealthyMS.com
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