Sickle Cell Disease: Prognosis and Cures
Sickle cell disease (SCD) can cause long-term complications. Most people experience acute and chronic injuries to different organs. This can increase healthcare costs, reduce quality of life, and a shorten life expectancy.
In the United States, the mortality rate of infants and children with SCD has greatly decreased over the past few decades. Now, more than 90 percent of children survive to adulthood. These improvements have been harder to achieve in adults with SCD.1
The most common causes of death for adults with SCD are acute chest syndrome, stroke, and infection. However, the direct cause of death is often unknown because people with SCD can die suddenly. The only cure is a bone marrow transplant, but very few people get this procedure. For most people with SCD, the focus is on treatments and transfusions that manage complications.
How does sickle cell disease affect life expectancy?
A 2019 study explored the life expectancy for people with SCD in the United States. The study found a life expectancy of 54 years for people with SCD, which is 22 years lower than the general U.S. population. This study did not estimate the life expectancy of people with different types of SCD.2
An earlier study done in 1994 explored the life expectancy of people with SCD between the years of 1978 and 1988. The study found a life expectancy of 42 years for men with SCD and 48 years for women with SCD. For people with HbSC, the life expectancy was 60 years for men and 68 years for women.3
It is likely that early diagnosis and better treatments have increased life expectancy in the United States. It is important to note that life expectancy varies depending on the type of SCD, as well as other genetic and environmental factors. Even with the best care, people with SCD still have a shorter life expectancy, lower quality of life, and increased medical costs.4,5
How is the mortality rate changing?
The mortality rate for infants and children with SCD has greatly decreased. This is related to:5
- Universal newborn screening for SCD
- Prophylactic antibiotics (the use of antibiotics before surgeries and dental procedures)
- Hydroxyurea treatment
In the United States, childhood mortality is now close to that of the general population.5
Compared to 1983 to 1986, the mortality rate from 1999 to 2002 has decreased by:6,7
- 68 percent for children 0 to 3 years old
- 39 percent for children 4 to 9 years old
- 24 percent for children 10 to 14 years old
Mortality rates for adults with SCD have not reduced as much and are still higher than the general population.
It is important to note that these improvements have not yet reached countries with fewer resources. For example, more than half of the children in Africa with SCD will die before they reach 5 years old.1,2
What are common causes of death?
The most common causes of death for adults with SCD include:4,8
- Acute chest syndrome
- Organ failure
However, the direct cause of death is sometimes unknown. This is because people with SCD can often die suddenly due to blockage of the blood vessels in the heart and lungs.4,8
A 2003 study found that about 40 percent of deaths in people with SCD are sudden and expected. About 30 percent of deaths occur within 24 hours after symptoms show, and about 60 percent of deaths are associated with acute events.9
In children with SCD, infection and stroke are leading causes of death. Vaccination, prophylactic antibiotics, and improved screening for stroke have reduced mortality from these causes.
Is there a cure?
The only known cure for SCD is a bone marrow transplant. Bone marrow is where red blood cells are made. This procedure takes healthy stem cells from a donor and puts them into someone with SCD to make normal red blood cells.10
The procedure can have serious side effects or be fatal. A bone marrow transplant only works if the donor is a relative and close genetic match. Transplants are usually only used in children and individuals with severe SCD who have minimal organ damage.Most people with SCD do not qualify for a bone marrow transplant or may have difficulty finding a relative that can be a matched donor. For now, the focus is on treatments and transfusions to manage complications.