What is Sickle Cell Disease?


What makes the red cell sickle?
How do you get sickle cell anemia or trait?
Is Sickle Cell Disease only found in African Americans?
Sickle cell is in many nationalities including African Americans, Africans, 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

What is Sickle Cell Trait?
Are there different types of sickle cell disease?
There are three common types of sickle cell disease in the United States.
  1. Hemoglobin SS or sickle cell anemia
  2. Hemoglobin SC disease
  3. 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 are the complications?
  1. pain episodes
  2. strokes
  3. increased infections
  4. leg ulcers
  5. bone damage
  6. yellow eyes or jaundice
  7. early gallstones
  8. lung blockage
  9. kidney damage and loss of body water in urine
  10. painful erections in men (priapism)
  11. blood blockage in the spleen or liver (sequestration)
  12. eye damage
  13. low red blood cell counts(anemia)
  14. delayed growth
What can be done to help prevent these complications?
Sickle cell patient should be under the care of a medical team that understands sickle cell disease. All newborn babies detected with sickle cell disease should be placed on daily penicillin to prevent serious infections. All of the childhood immunizations should be given plus the pneumococcal vaccine. Parents should know how to check for a fever because this signals the need for a quick medical checkup for serious infection. The following are general guidelines to keep the sickle cell patient healthy:
  2. Daily penicillin until age six to prevent serious infection
  3. Drinking plenty of water daily (8-10 glasses for adults)
  4. Avoiding too hot or too cold temperatures
  5. Avoiding over exertion and stress
  6. Getting plenty of rest
  7. Getting regular check-ups from knowledgeable health care providers

Patients and families should watch for the following conditions that need an urgent medical evaluation:

  1. Fever
  2. Chest pain
  3. Shortness of Breath
  4. Increasing tiredness
  5. Abdominal swelling
  6. Unusual headache
  7. Any sudden weakness or loss of feeling
  8. Pain that will not go away with home treatment
  9. Priapism (painful erection that will not go down)
  10. Sudden vision change

Download a 1 page fact sheet to print and use [doc]

Used with permission from SCInfo.org