Life Expectancy With Sickle Cell Anemia

Sickle cell disease is a grouping of blood disorders that occurs in some people that affect the body's red blood cells. Sickle cell anemia is one of these disorders. Although rare, sickle cell anemia can significantly impact the health and life of an individual with the condition.

Prior studies looking into the life expectancy of those diagnosed with specifically sickle cell anemia revealed that the average survival age was 48 years for women and 42 years for men. In most cases, the individuals that lost their lives at an early age with sickle cell anemia did so after a sudden complication from the disease.¹

Although other sickle cell disease disorders are also linked to a shorter lifespan, the average life expectancy is higher for those individuals than for patients with sickle cell anemia.

Can you die from sickle cell disease?

The impact of sickle cell disease on the body of an individual with the condition takes its toll over time. The effects of sickle cell disease on the functions and organs within the body can heighten the risk of sudden and premature death. Many people with sickle cell disease may have no symptoms or may even be unaware they have the condition. 

When a person begins to experience symptoms of the disorder and its impacts on their health, they can become at an increased risk of fatal complications.

Sickle cell disease lifespan

In the past, sickle cell disease found in a child made it unlikely that the child would survive into adulthood due to the various complications and challenges presented by the disorders, particularly sickle cell anemia. However, advances in identifying risks, prophylaxis, and treatment options have prolonged the lifespan of those diagnosed with sickle cell disease early in life. 

Reports estimate that now almost 95% of people with sickle cell disease at birth will survive into adulthood. However, individuals with severe forms of the disease, such as sickle cell anemia, are likely to have a shorter lifespan by 20–30 years compared to an individual without a sickle cell diagnosis.

What are the types of sickle cell disease?

The type of sickle cell disease an individual experiences will depend on the genetic traits inherited by their parents. If a person has a single sickle cell trait, they are carriers of the potential disease but do not have it and are unlikely to develop any symptoms or impacts on their life from the trait. 

However, when a person carries two sickle cell traits or a combination of the sickle cell trait with another problematic genetic trait, they may have one of the following sickle cell disorders:

• Sickle cell anemia

• Sickle hemoglobin disease

• Sickle beta thalassemia

What is sickle cell anemia?

Sickle cell anemia is one of a family of sickle cell diseases. Sickle cell anemia is also most commonly the worst and most severe form of sickle cell disorder.

In sickle cell anemia, a person is born with the sickle cell trait inherited from both parents. Sickle cell is hereditary. It is not something you will develop or contract later in life. 

However, there are instances where a person may not know they have sickle cell trait or the disease unless a specific blood test is done to test for the condition. In the US, when babies are born in the hospital, sickle cell testing is part of the recommended newborn screening for all newborns. Additionally, sickle cell disease may be discovered as part of genetic testing during pregnancy.

The genetic trait causes the red blood cells that develop to be shaped irregularly, referred to as a sickle (crescent moon) shape, due to abnormal hemoglobin production. The abnormal shape of the red blood cells from the inherited disorder, in turn, affects how the body transports oxygen through the blood.

The sickle cells inhibit the blood's ability to carry oxygen effectively through the body. This can harm organs and the body's functions, which can worsen over time.

How common is sickle cell disease?

In the US, sickle cell disease affects approximately 100,000 people. The incidence of sickle disease among the general population is very low. However, there are certain demographics where the disorder is more prevalent, such as in African American and Hispanic populations.²

It is estimated that one in thirteen African Americans in the US is born with sickle cell trait, and one in 365 is born with sickle cell disease. Although the frequency of the disease is lower in Hispanic populations compared to African American demographics, it still occurs at an incidence of one in every 16,300 Hispanic US births.

Carrying the sickle cell trait does not mean you have symptoms or effects of a sickle cell disorder, although it is possible depending on other genetic factors. To have sickle cell anemia, a person must carry the trait from both parents.

However, knowing you are a carrier of sickle cell trait can be beneficial when planning on having children, as it is a genetic trait you may pass down to the next generation.

Symptoms of sickle cell anemia

When a baby is born with sickle cell anemia, it may appear healthy and show no signs of the disorder in the first months of life. However, symptoms relating to sickle cell disease are likely to begin after the six-month mark as the baby starts to develop. 

It is important to remember that if a baby carries the sickle cell trait, that does not mean they have sickle cell disease. It is only when a baby carries both traits that they can develop sickle cell anemia and, in turn, develop symptoms of the condition as they age.

Warning signs and symptoms of sickle cell anemia include:

• Feeling fatigued, which can present itself as irritability or fussiness in babies

• Jaundice or yellowish appearance of the skin or whites of the eyes

• Fever

• Pain episodes

• Symptoms of a stroke

• Swelling in the hands or feet

What causes sickle cell disease?

Sickle cell disease, including sickle cell anemia and other sickle cell disorders, occurs because of an individual's genetic makeup. You cannot catch sickle cell disease or trigger the disease with your lifestyle or environmental factors. 

The only way a person can get sickle cell disease is by inheriting the sickle cell trait. Inheriting one sickle cell trait makes you a carrier of the trait and is unlikely to significantly impact your health. However, when an individual inherits the sickle cell trait in combination with another sickle cell trait from another parent or other hemoglobin abnormality, they can then develop sickle cell disease.

What are the complications of sickle cell disease?

The root impact of sickle cell disease on a person with the condition is how it affects the oxygen passing through your body and blood. Over time the lack of sufficient oxygen to organs and other areas in the body has a negative effect and leads to a wide range of possible complications. Some of the complications can, in some cases, lead to other life-threatening conditions or death.

Possible complications that can arise with sickle cell disease include:

• Stroke

• Anemia

• Susceptible to infection

• Liver damage

• Kidney damage

• Damage or loss of vision

• Pulmonary hypertension

• Bone tissue loss

• Painful hand and foot swelling

• Damage to organs

• Sleep apnea

• Pain episodes

Sickle cell disease mortality rate

Although mortality rates for children with sickle cell disease have continued to improve over time, the overall life expectancy of a person with sickle cell disease remains much lower than that of people without the disease. 

Studies evaluating the mortality rate among sickle cell patients revealed that approximately 65% of the deaths were identified as having sickle cell disease as the underlying cause. The most common comorbidities in patients with sickle cell disease were infections, heart disease, stroke, and liver disease.³

Risk factors for early death from sickle cell disease

Predictors as to the risk of early death from sickle cell disease include whether the individual has sickle cell anemia or another sickle cell disorder. There is a nearly 18–20 year difference in the life expectancy of an individual with sickle cell anemia versus another, less severe form of sickle cell disease. 

Additionally, whether an individual is symptomatic at the moment can increase their risk of death from the disease or its complications. Studies on the cause of death of sickle cell disease patients identified that in many of the deaths, the death did not arise because of chronic organ failure or long-term complications but rather in a sudden manner due to an acute onset of pain, stroke, or acute chest syndrome.

How to improve the survival rate for sickle cell disease?

While you cannot prevent sickle cell disease development, some measures have been identified in the medical community over time as beneficial to improving the survival and life expectancy of sickle cell disease patients.

Better screening measures, prophylactic treatment in pediatric patients, and vaccinations against pneumococcal disease have positively impacted the survival rates of patients with sickle cell disease and sickle cell anemia in childhood. 

However, as children transition into adulthood, renewed challenges and healthcare access issues could have a detrimental impact on patients. Continued access to medical care, involvement in treatment, and access to treatments such as hydroxyurea can improve the quality of life and health of patients with sickle cell disease as they age.

What causes death in sickle cell disease patients?

The continued deprivation of oxygen to organs in the body and the subsequent complications that can arise from the lack of sufficient oxygen ultimately leads to the death of sickle cell disease patients. 

The disease causes the body significant strain to function properly when it does not have the same ability to produce healthy red blood cells as a person without sickle cell disease. Death from sickle cell disease may ultimately occur because of damage to the heart, liver, kidneys, arteries, or a range of vital functions of the body.

The lowdown

Sickle cell disease can affect life expectancy. However, unlike in the past, children are much more likely to survive well into adulthood, and adults can live longer thanks to advances in early detection, treatments, and prophylactic measures. 

Sickle cell disease life expectancy may be shorter than the life expectancy of those without the condition, but the risk of death is mostly associated with complications that can arise. 

If you have sickle cell disease, talk to your doctor about the type of sickle cell disease you have and if there are any options and measures you can take to manage and improve your quality of life and reduce your risk of complications if possible.