What Causes Sickle Cell Anemia?

Anemia refers to a condition in which your body fails to produce healthy red blood cells – the inherited form of anemia is sickle cell anemia. Under normal circumstances, your red blood cells will be round and rather flexible, and face no issues when passing through tiny blood vessels. Those cells become sticky and rigid and look like crescent moons or sickles when you develop this blood disorder. Due to their shape, they can get stuck in blood vessels and cause a blockage.

What Causes Sickle Cell Anemia?

Sickle cell anemia is the outcome of a mutation in the gene that signals your body to make hemoglobin – your blood looks red due to this iron-rich compound. The presence of hemoglobin is essential for red cells to take oxygen from your lungs and supply it to the rest of your body. In sickle cell anemia, the abnormality in hemoglobin affects the shape of red cells and makes them sticky.

It is a genetic condition and passes in a pattern of inheritance known as autosomal recessive inheritance. It means you cannot have this disorder if you have one mutated gene only – you develop this condition only when you receive one mutated gene from your father and one from your mother. Having one mutated gene makes you a carrier of the sickle cell gene, which means that if you marry to someone who is also a carrier your child may develop this disorder.

If both you and your partner are carriers, there are chances that your child will inherit it as well.

  • There is a one in four chance that your baby will have a pair of normal hemoglobin genes.
  • There is a one in two chance that your newborn will have one mutated gene and one normal gene.
  • There is a one in four chance that your newborn will have two mutated genes and have the disorder.

What it implies is that if you have sickle cell trait but your partner does not have any mutated gene, your baby cannot develop this disorder. Your baby may still have the sickle cell trait though.

In order for a baby to have this disorder, both parents must carry a mutated gene. The gene is more commonly found in families that come from India, Africa, Saudi Arabia, Mediterranean countries, and South and Central America. It usually affects only blacks in the United States.

What Are the Symptoms of Sickle Cell Anemia?

Now you know what causes sickle cell anemia, how will the disorder present itself? There are usually no symptoms until your baby is at least 4 months old. Once the symptoms do appear, you may witness the following:

  • Anemia: Since sickle cells are quite fragile, they break apart with ease and leave you with inadequate supply of red blood cells. Healthy red blood cells usually die in 120 days but sickle cells may die in 20 days or even less. This leads to a lasting shortage of red cells that causes anemia.
  • Pain: Anyone with this red blood cell disorder has periodic episodes of pain, called crises. The pain you experience is due to a blockage caused by sickle cells in tiny blood vessels. This restricts blood flow to your abdomen, chest, and joints, causing severe pain. The pain may last from a few hours to several weeks, and may vary in intensity as well.
  • Hand-foot syndrome: Swollen feet and hands may indicate your infant has sickle-shaped cells. Hands and feet swell when sickle cells block flow of blood to these limbs.
  • Frequent infections: Sickle cells can cause serious damage to your spleen, which makes you more vulnerable to infections. Doctors use antibiotics and vaccinations to prevent pneumonia and other life-threatening infections.
  • Growth issues: Due to sickle cell anemia, the red blood cells cannot supply oxygen and nutrients to all parts of your body and this often results in slow growth, especially in children and infants.
  • Vision problems happen because sickle cells causing blockage in tiny vessels that supply blood to your eyes. This may also lead to retinal damage.

How Can Sickle Cell Anemia Be Treated?

Once you know what causes sickle cell anemia you may also want to know about treatment options available for this condition. There are some medical treatments and home remedies available to make you feel better.

Medical Treatments

  • Your doctor may give penicillin to your newborns after they are 2 months old – this continues to 5 years. This helps prevent pneumonia and other infections.
  • Patients may be given supplemental oxygen through a mask to improve oxygen levels in the blood and make breathing easier.
  • Pain medications are also available to help relieve pain experienced during a sickle crisis. OTC drugs may also help relieve pain. Sometimes, your doctor recommends stronger prescription pain medications, such as morphine.
  • The use of hydroxyurea is also common – it helps improve production of hemoglobin in fetus.
  • Surgery is also an option in case other treatment options do not work – it helps treat painful and persistent erections.
  • Bone marrow transplant is another treatment option that works great for children under 16 years of age.
  • Blood transfusions also help treat the condition – the procedure involves removing red cells from donated blood and given to patients.

Home Remedies

No matter what causes sickle cell anemia, there're some home remedies that you can try to make yourself feel better.

  • Take folic acid supplements. Your bone marrow cannot make red blood cells if there isn't enough folic acid in your body. You may take folic acid supplements daily or pay attention to your diet to increase your folic acid intake.
  • Stay hydrated. Dehydration may lead to a sickle cell crisis, so it is important to drink plenty of water throughout your day.
  • Limit your exposure to extreme cold or heat to lower your risk of a sickle cell crisis.
  • Stay active but do not overdo it. Ask your doctor about the exercises you can do safely.
  • Opt for pressurized cabins during air travel. Unpressurized cabins will limit oxygen supply and increase your risk of experiencing a sickle crisis.
  • Ask your doctor for advice before traveling to high-altitude areas where there isn't enough oxygen in the air.
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