Health

Sickle cell anemia and mortality: Is it fatal?

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People often consider sickle cell anemia (SCA) to be a fatal condition, but recent advancements in care mean that it is common to survive into adulthood. Although SCA often lowers life expectancy, the outlook for those with this condition is improving.

Sickle cell disease (SCD) is an umbrella term for a group of inherited disorders affecting the red blood cells.

The Centers for Disease Control and Prevention (CDC) state that SCA is the most severe form of SCD. The medical community may also refer to SCA as HbSS.

SCA is associated with a change in red blood cell shape that makes them sickle-shaped instead of resembling flat disks. Estimates suggest that 30,000–200,000 people in the United States have SCA.

This article reviews sickle cell anemia mortality, life expectancy, and more.

SCA often leads to a shorter overall lifespan for people with the condition, and it can be fatal.

However, in recent years, SCA survival rates have improved. According to a 2019 study, new guidelines in care within the U.S. have led to higher survival rates, with 95% of people reaching adulthood.

As a result of these guidelines, doctors are more likely to screen for the condition in infancy, prescribe preventive antibiotics, provide vaccinations, and use hydroxyurea for treatment.

However, the study authors note an association between SCA and lower life expectancy. They write that the average life expectancy for a person with SCA is 54 years compared with 74 years for matched controls.

They also note that people with SCA have an average of 33 years of quality life compared with 67 years for people without the condition.

Is it always fatal?

SCA is associated with reduced life expectancy, along with an increased risk of comorbid, potentially fatal, diseases. Although about 95% of children survive to adulthood, a person with SCA lives, on average, about 22 fewer years than a person without the condition.

Read one person’s account of living with SCA.

What can affect life expectancy for those with sickle cell anemia?

SCA is associated with several potentially life threatening complications. These include issues with the heart, brain, and other vital organs. Current understanding does not link any particular complication to a decreased life expectancy.

However, some evidence suggests that access to affordable care can improve a person’s overall outcome. In other words, people who can afford better care are more likely to live longer than those who cannot.

Learn about SCA in African American people.

Abnormal hemoglobin, a protein found on red blood cells, makes the red blood cells different in people with SCA. Usually, these cells have a round, disk-like shape that allows them to travel through blood vessels easily.

The abnormal hemoglobin causes the red blood cells to harden, become stickier, and change shape to resemble a sickle. It is more difficult for the altered red blood cells to travel through blood vessels to carry oxygen and nutrients around the body.

In addition, sickle-shaped red blood cells have a shorter life span, which means that a person has a continual blood shortage, leading to anemia.

Finally, SCA can cause damage to multiple organ systems throughout the body. This can lead to potentially fatal complications, including:

Learn more about SCA.

SCA is a lifelong condition for most people. The National Heart, Lung, and Blood Institute suggests that early bone marrow replacement or blood transfusions can cure SCA in some people.

Other treatments focus on managing chronic pain or reducing complications. The treatment options include:

  • Medication: Some medications prevent blood cells from sickling, whereas others reduce sickle cell pain crises and the risk of infection. Newer medications, such as hydroxyurea, prevent several SCA complications.
  • Blood transfusion: Blood transfusions can increase the number of healthy red blood cells, reducing the risk of complications. Doctors may also recommend them for people with SCA who have had a stroke to reduce the risk of subsequent strokes.
  • Blood and bone marrow transplant: This procedure can cure SCA in some people and is most successful in children. Most people are either too old for treatment or do not have a family member who is a close genetic match. This procedure carries risks even when the donor is a closely matched relative.

Soon, experts hope to use gene therapy to help treat SCA. This type of therapy replaces parts of a person’s DNA with healthy strands to promote the production of healthy red blood cells.

Learn more about blood transfusions and SCD.

The life expectancy for a person with SCA has improved in recent years. This is due to better screening, earlier treatment, and new medications. With proper care, most people live to adulthood.

However, people with SCA still face reduced life expectancy and several possible complications that can severely affect their quality of life. Newer treatments may help improve a person’s symptoms and overall life expectancy.

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