Sickle cell disease is a disease that affects a special protein inside our red blood cells called haemoglobin. Red blood cells have an important job. They pick up oxygen from the lungs and take it to every part of the body. It is the haemoglobin in these cells that carry the oxygen to different parts of the body.
A person with sickle cell disease makes a different kind of haemoglobin. This causes the red blood cells to change their shape. Instead of being smooth and round, the cells change and become hard and sticky. Their shape often looks like a banana or like a sickle. It is this sickle shape of the red blood cells that gives sickle cell disease its name. The hard, sticky, sickled red blood cells have trouble moving through small blood vessels.
What are the Symptoms of Sickle Cell Disease?
All of the major symptoms of sickle cell disease are the direct result of the abnormally shaped, sickled, red blood cells blocking the flow of blood that circulates through the body. The organs and tissues with impaired circulation are damaged by lack of oxygen.
Damage to tissues and organs of the body can cause severe disability in individuals with sickle cell disease. Individuals endure intermittent pain episodes of variable frequency and severity, depending on the degree of organ involvement. The major symptoms of sickle cell disease are variable, but can include:
Fatigue and Anemia
Hand-Foot Syndrome (swelling and inflammation of the hands and/or feet)
Jaundice (yellowing of the skin and eyes)
Splenic Sequestration (sudden pooling of blood in the spleen) and Liver Congestion
Acute Chest Syndrome
Hip Problems and Bone Pain
Some symptoms of sickle cell disease, such as fatigue, anemia, pain episodes, and bone pain can occur at any age. Many symptoms typically occur in certain age groups. Sickle cell disease usually first presents symptoms in the first year of life. Infants and younger children can suffer with fever, abdominal pain, pneumococcal bacterial infections, painful swelling of the hands and feet, and splenic sequestration.
Adolescents and young adults more commonly develop leg ulcers, hip disease, and eye damage. Additional symptoms in adults typically are intermittent pain episodes, acute chest syndrome, and kidney problems.
What Causes Sickle Cell Disease?
Sickle cell disease is an inherited disease. An inherited disease is one which is passed on from parents to their children through their genes.
To inherit sickle cell disease, a child must get the sickle (S) gene from one parent and a sickle (S), C, D, E or beta thalassemia gene from the other parent.
For additional information, contact your Primary Care Physician.
How to Find Out About Your Hemoglobin Genes
The only way to know for certain what type of hemoglobin you have is to have a special blood test called hemoglobin electrophoresis with a complete blood count (CBC). Contact your doctor or health provider to request this test for you.