Diseases and Disorders

Difference Between Sickle Cell Anemia and Sickle Cell Disease

What is sickle cell disease?

Sickle cell disease is a group of inherited red blood cell disorders. Red blood cells contain hemoglobin, a protein that carries oxygen. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has sickle cell disease, the hemoglobin is abnormal, which causes the red blood cells to become hard and sticky and look like a C-shaped farm tool called a “sickle.”

The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious complications, such as infection, acute chest syndrome and stroke.

What is sickle cell anemia?

Sickle cell anemia is a form of the inherited blood disorder, sickle cell disease. Sickle cell anemia affects your red blood cells, turning them from round flexible discs into stiff and sticky sickled cells. Sickled cells keep red blood cells from doing their job, which is carrying oxygen throughout your body. Sickled cells also don’t live as long as normal red blood cells. As a result, you don’t have enough healthy red blood cells and you develop anemia, the condition that gives sickle cell anemia its name.

Difference between sickle cell anemia and sickle cell disease

Sickle cell anemia is one of a group of inherited disorders known as sickle cell disease. It affects the shape of red blood cells, which carry oxygen to all parts of the body.

Red blood cells are usually round and flexible, so they move easily through blood vessels. In sickle cell anemia, some red blood cells are shaped like sickles or crescent moons. These sickle cells also become rigid and sticky, which can slow or block blood flow.

There’s no cure for most people with sickle cell anemia. Treatments can relieve pain and help prevent complications associated with the disease.

Sickle cell anemia is caused by?

Causes of sickle cell anemia;

Sickle cell anemia is caused by a change in the gene that tells the body to make the iron-rich compound in red blood cells called hemoglobin. Hemoglobin enables red blood cells to carry oxygen from the lungs throughout the body. The hemoglobin associated with sickle cell anemia causes red blood cells to become rigid, sticky, and misshapen.

For a child to be affected, both mother and father must carry one copy of the sickle cell gene, also known as the sickle cell trait, and pass both copies of the altered form to the child.

If only one parent passes the sickle cell gene to the child, that child will have the sickle cell trait. With one typical hemoglobin gene and one altered form of the gene, people with the sickle cell trait make both typical hemoglobin and sickle cell hemoglobin.

Their blood might contain some sickle cells, but they do not show symptoms. They’re carriers of the disease, however, which means they can pass the gene to their children.

Sickle cell anemia symptoms

Signs and symptoms of sickle cell anemia usually appear around 6 months of age. They vary from person to person and may change over the time.

Signs and symptoms of sickle cell anemia can include:
  1. Anemia. Sickle cells break apart easily and die. Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells typically die in 10 to 20 days, leaving a shortage of red blood cells (anemia). Without enough red blood cells, the body can’t get enough oxygen and this causes fatigue.
  2. Episodes of pain. Periodic episodes of extreme pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints.
    • The pain varies in intensity and can last for a few hours to a few days. Some people have only a few pain crises a year. Others have a dozen or more a year. A severe pain crisis requires a hospital stay.
    • Some adolescents and adults with sickle cell anemia also have chronic pain, which can result from bone and joint damage, ulcers, and other causes.
  3. Swelling of hands and feet. The swelling is caused by sickle-shaped red blood cells blocking blood circulation in the hands and feet.
  4. Frequent infections. Sickle cells can damage the spleen, increasing vulnerability to infections. Infants and children with sickle cell anemia commonly receive vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia.
  5. Delayed growth or puberty. Red blood cells provide the body with the oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.
  6. Vision problems. Tiny blood vessels that supply the eyes can become plugged with sickle cells. This can damage the retina, the portion of the eye that processes visual images, and lead to vision problems.
What are the complications of sickle cell anemia?
Sickle cell anemia can lead to a host of complications, including these 11 major complications:
  1. Stroke. Sickle cells can block blood flow to an area of the brain. Signs of stroke include seizures, weakness or numbness of the arms and legs, sudden speech difficulties, and loss of consciousness. If your child has any of these signs and symptoms, seek medical treatment immediately.
  2. Acute chest syndrome. A lung infection or sickle cells blocking blood vessels in the lungs can cause this life-threatening complication, resulting in chest pain, fever and difficulty breathing. It might require emergency medical treatment.
  3. Pulmonary hypertension. People with sickle cell anemia can develop high blood pressure in their lungs. This complication usually affects adults. Shortness of breath and fatigue are common symptoms of this condition, which can be fatal.
  4. Organ damage. Sickle cells that block blood flow to organs deprive the affected organs of blood and oxygen. In sickle cell anemia, blood is also chronically low in oxygen. This lack of oxygen-rich blood can damage nerves and organs, including kidneys, liver and spleen, and can be fatal.
  5. Splenic sequestration. Many sickle cells can get trapped in the spleen, causing it to enlarge and possibly causing belly pain on the left side of the body. This can be life-threatening. Parents of children with sickle cell anemia should learn to feel their child’s spleen for enlargement.
  6. Blindness. Sickle cells can block tiny blood vessels that supply the eyes. Over time, this can lead to blindness.
  7. Leg ulcers. Sickle cell anemia can cause painful open sores on the legs.
  8. Gallstones. The breakdown of red blood cells produces a substance called bilirubin. A high level of bilirubin in the body can lead to gallstones.
  9. Priapism. In this condition, men with sickle cell anemia can have painful, long-lasting erections. Sickle cells can block the blood vessels in the penis, which can lead to impotence.
  10. Deep vein thrombosis. Sickling of red cells can cause blood clots, increasing the risk of a clot lodging in a deep vein (deep vein thrombosis) or a lung (pulmonary embolism). Either can cause serious illness or even death.
  11. Pregnancy complications. Sickle cell anemia can increase the risk of high blood pressure and blood clots during pregnancy. It can also increase the risk of miscarriage, premature birth and having low birth weight babies.
Sickle cell anemia diagnosis

Sickle cell anemia is usually diagnosed through genetic screening done when a baby is born. Those test results will probably be given to your family doctor or pediatrician. He or she will probably refer you to a doctor who specializes in blood disorders (hematologist) or a pediatric hematologist. Know about hb electrophoresis test.

Read about what is SAD?

Is there a cure for sickle cell anemia?

There’s no cure for most people with sickle cell anemia. Treatments can relieve pain and help prevent complications associated with the disease.

What type of mutation causes sickle cell anemia?

Mutations in the HBB gene cause sickle cell disease. The HBB gene provides instructions for making one part of hemoglobin. Hemoglobin comprises four protein subunits, typically, two subunits called alpha-globin and two subunits called beta-globin.

How is sickle cell anemia inherited?

You inherit 1 set from your mother and 1 set from your father. To be born with sickle cell disease, a child has to inherit a copy of the sickle cell gene from both their parents. This usually happens when both parents are “carriers” of the sickle cell gene, also known as having the sickle cell trait.

Is sickle cell anemia genetic?

Yes. Sickle cell anemia is a genetic disease, so it is caused by one or more genes not working correctly.

What mutation causes sickle cell anemia?

Mutations in the HBB gene cause sickle cell disease. The HBB gene provides instructions for making one part of hemoglobin. Hemoglobin comprises four protein subunits, typically, two subunits called alpha-globin and two subunits called beta-globin. The HBB gene provides instructions for making beta-globin.

Originally published in MayoClinic

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