What is sickle cell anemia?

Sickle cell anemia (SCA), sometimes called sickle cell disease, is a blood disorder that causes your body to make an unusual form of hemoglobin called hemoglobin S. Hemoglobin carries oxygen. It’s found in red blood cells (RBCs).

While RBCs are usually round, hemoglobin S makes them C-shaped, like a sickle. This shape makes them stiffer, preventing them from bending and flexing when moving through your blood vessels. As a result, they can get stuck and block the flow of blood through blood vessels. This can cause a lot of pain and have lasting effects on your organs.

Hemoglobin S also breaks down faster and can’t carry as much oxygen as typical hemoglobin. This means that people with SCA have lower oxygen levels and fewer RBCs. Both of these can lead to a range of complications.

What does all of this mean in terms of survival rate and life expectancy? SCA is linked to a shorter life span. But the prognosis for people with SCA has become more positive over time, especially within the last 20 years.

When talking about someone’s prognosis, or outlook, researchers often use the terms survival rate and mortality rate. While they sound similar, they measure different things:

  • Survival rate refers to the percentage of people who live for a certain amount of time after getting a diagnosis or treatment. Think of a study looking at a new cancer treatment. The five-year survival rate would show how many people were alive five years after starting the treatment being studied.
  • Mortality rate refers to the percentage of people with a condition who died within a certain time frame. For example, a study mentions a condition has a 5 percent mortality rate for people between the ages of 19 and 35. This means 5 percent of people in this age range with this condition die from it.

When talking about the prognosis for people with SCA, experts tend to look at mortality rates.

The mortality rate of SCA for children has dropped dramatically over the last few decades. A 2010 review references a 1975 study indicating a mortality rate of 9.3 percent for people with SCA under the age of 23. But by 1989, the mortality rate for people with SCA under the age of 20 dropped to 2.6 percent.

A 2008 study compared mortality rates of African-American children with SCA from 1983 to 1986 to morality rates in the same group from 1999 to 2002. It found the following decreases in mortality rates:

  • 68 percent for ages 0 to 3
  • 39 percent for ages 4 to 9
  • 24 percent for ages 10 to 14

Researchers believe a new pneumonia vaccine released in 2000 played a large role in the reduced mortality rate for children between the ages of 0 and 3. SCA can leave people more vulnerable to serious infections, including pneumonia.

While advances in medicine and technology have decreased the mortality rate of SCA among children, the condition is still linked to a shorter life span in adults.

A 2013 study looked at more than 16,000 SCA-related deaths between 1979 and 2005. The investigators found that the average life expectancy for women with SCA was 42 years and 38 years for men.

This study also notes the mortality rate of SCA in adults hasn’t decreased the way it has for children. The investigators suggest this is likely due to a lack of access to quality healthcare among adults with SCA.

Many things play a role in determining how long someone with SCA will live. But experts have identified a few concrete factors, particularly in children, that can contribute to a poorer prognosis:

  • having hand-foot syndrome, which is painful swelling in the hands and feet, before the age of 1
  • having a hemoglobin level that’s less than 7 grams per deciliter
  • having a high white blood cell count without any underlying infection

Access to nearby, affordable healthcare can also play a role. Regularly following up with a doctor helps identify any complications that might need treatment or concerning symptoms. But if you live in a rural area or don’t have health insurance, this is easier said than done.

Find low-cost health clinics in your area here. If you can’t find one in your area, call the clinic that’s closest to you, and ask them about any rural healthcare resources in your state.

The Sickle Cell Society and Sickle Cell Disease Coalition also offer helpful resources for learning more about the condition and finding medical care.

Whether you’re the parent of a child with SCA or an adult living with the condition, there are several things you can do to reduce the risk of complications. This can have a big impact on prognosis.

Tips for children

One of the easiest ways to improve the prognosis of a child with SCA is to focus on reducing their risk of infection. Here are some tips:

  • Ask your child’s doctor if they should be taking a daily dose of penicillin to prevent certain infections. When you ask, make sure to tell them about any infections or illnesses your child had in the past. It’s strongly recommended all children with SCA under 5 years old be on daily preventive penicillin.
  • Keep them up to date on vaccinations, especially for pneumonia and meningitis.
  • Follow up with a yearly flu shot for children ages 6 months or older.

SCA can also lead to a stroke if sickle RBCs block a blood vessel in the brain. You can get a better idea of your child’s risk by having them do a transcranial Doppler ultrasound every year between the ages of 2 and 16. This test will help identify whether they have an increased risk of stroke.

If the test finds that they do, their doctor may recommend frequent blood transfusions to help lower the risk.

People with SCA are also prone to vision problems, but these are often easier to treat when caught early. Children with SCA should have a yearly ophthalmic exam to check for any issues.

Tips for adults

If you’re an adult living with SCA, focus on preventing episodes of severe pain, known as a sickle cell crisis. This happens when abnormal RBCs block small blood vessels in the joints, chest, abdomen, and bones. It can be very painful and damage organs.

To reduce your risk of having a sickle cell crisis:

  • Stay hydrated.
  • Avoid overly strenuous exercise.
  • Avoid high altitudes.
  • Wear plenty of layers in cold temperatures.

You can also ask your doctor about taking hydroxyurea. It’s a chemotherapy drug that can help reduce sickle cell crises.

Suggested reads

  • “A Sick Life: TLC ’n Me” is a memoir by Tionne “T-Box” Watkins, the lead singer of Grammy-winning group TLC. She documents her rise to fame while having SCA.
  • “Hope and Destiny” is a guidebook for people living with SCA or caring for a child who has it.
  • “Living with Sickle Cell Disease” is a memoir by Judy Gray Johnson, who’s lived with SCA through childhood, motherhood, a long teaching career, and beyond. She recounts not only how she handled the ups and downs of the condition, but also the socioeconomic barriers she faced in finding treatment.
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People with SCA do tend to have a shorter life expectancy than those without the condition. But the overall prognosis for people, especially children, with SCA has improved over the last few decades.

Regularly following up with a doctor and staying aware of any new or unusual symptoms are key to avoiding potential complications that could affect your prognosis.