Before the 1960s, it was rare for people with hemophilia to live past the age of 60. Today, the life expectancy for those with this inherited bleeding disorder is close to that of the general population. While the prognosis (outlook) for people with hemophilia B has improved, life expectancy is still not as long as the general population.

Continue reading to learn more about how hemophilia B can affect your life span.

When Is Hemophilia B Life-Threatening?

People with hemophilia B — also known as Christmas disease — lack a blood clotting factor called factor IX (9). Factor IX is a protein found in the blood that helps your blood clot. Without factor IX, people with hemophilia B are at risk of bleeding more or longer than usual after an injury. For those with severe hemophilia, excessive bleeding can occur spontaneously without an injury or accident.

Bleeding

It’s important to be aware that bleeding plays a significant role in the health of people with hemophilia B. Internal bleeding can be serious, especially depending on where it occurs in the body. For instance, even a minor head injury could result in intracranial bleeding (bleeding inside the brain). People with hemophilia B, when compared to the general population, have a slightly higher risk of dying associated with intracranial bleeding.

Infectious Disease

People living with hemophilia B have a higher risk of death from infectious diseases such as human immunodeficiency virus (HIV) and hepatitis C virus (HCV). This is due to potential past exposure to contaminated blood products, such as coagulation factor IX, that occurred in the 1980s. At one point, almost half of people living with hemophilia in the U.S. were infected with HIV and/or HCV.

The number of people with hemophilia who died from HIV was highest between 1992 and 2001 and has been decreasing since then. HCV infection can result in death due to chronic (ongoing) liver diseases such as cirrhosis, liver cancer, and end-stage liver disease.

Factor products are treatments made from blood donations to help people with bleeding disorders. In the 1990s, stricter screening and viral inactivation methods (processes used to eliminate or make viruses inactive) were used to make factor products derived from donated blood safer and reduce the risk of infection. Since then, synthetic recombinant factor products have been developed that aren’t derived from donated blood. Synthetic recombinant factor products for hemophilia are created in a lab to replace the missing clotting factors in blood and help people with hemophilia stop bleeding.

Injury

Compared to the general population, people with hemophilia B may have an increased risk of death from an injury. For example, injury caused by trauma may cause internal bleeding that can result in death.

Read about what to keep in a hemophilia emergency kit.

What Factors Influence Life Expectancy With Hemophilia B?

Several factors influence your life expectancy when you’re living with hemophilia B, including:

• When you were diagnosed with hemophilia B
• Where you live
• The severity of your hemophilia
• Your hemophilia treatment
• Development of infections due to the use of plasma-derived factor concentrates (clotting factor treatments made from human blood)

Long-term observational studies of people with hemophilia B can show how long you can expect to live with the condition. In this type of research, scientists watch and learn from people over many years to understand how their health changes over time. The data from most of these studies includes people with hemophilia A and hemophilia B because the condition is rare. Compared to people living with hemophilia A — in which people lack factor VIII (8) — those with hemophilia B appear to have a lower death rate.

How Is Life Expectancy Calculated?

The life expectancy — the average number of years a person is expected to live — of people with hemophilia B can be measured as a median life expectancy. The median life expectancy is the age where half of people will survive longer and half of people will die before that age.

Researchers can compare the median life expectancy of people with hemophilia B to the general population. Cleveland Clinic notes that because hemophilia B is more common in males, the general population may only include males.

These days, people with hemophilia B are living longer. Advances in hemophilia B treatment and better screening for blood-borne infections in blood products have increased life expectancy. Additionally, life expectancy varies from country to country.

For example, people living with hemophilia in the Netherlands saw the life expectancy for hemophilia (types A and B) increase by 11 years in a few decades. Between 1973 and 1986, the median life expectancy for people with severe types of hemophilia was 66 years. Between 2001 and 2018, it increased to 77 years. This was still six years lower than the median life expectancy of the general male population in the Netherlands, which was 83 years.

How Does Hemophilia B Severity Affect Life Expectancy?

Hemophilia B can be classified as mild, moderate, or severe based on the factor IX activity level. Read more about how the severity of hemophilia is assessed.

In general, median life expectancy is shorter for people with more severe forms of hemophilia B. In the Netherlands, median life expectancy from 2001 to 2018 varied based on hemophilia severity as follows:

• Severe hemophilia — 73 years
• Moderate hemophilia — 80 years
• Mild hemophilia — 79 years

Researchers found that the mortality rate was 80 percent higher in people with severe hemophilia compared to those with mild hemophilia during this time in the Netherlands. The mortality rate is the measure of how many people have died from a particular cause or within a specific population over a certain period of time

Similar observations were made in the United Kingdom (U.K.) about people with hemophilia A and hemophilia B who weren’t infected with HIV between 1977 and 1999. Researchers found that the median life expectancy for people with severe hemophilia was 63 years. In people with mild or moderate hemophilia, the median life expectancy was longer at 75 years.

For comparison, the median life expectancy for males in the general population was 78 years in 1999. Additionally, researchers found that people with severe hemophilia died at twice the rate as those with mild or moderate hemophilia.

How Does Hemophilia B Treatment Affect Life Expectancy?

Advances in the treatment of hemophilia B have improved life expectancy. When factor IX concentrates became available in the 1970s, it was possible to increase factor IX levels in people with hemophilia B and reduce bleeding episodes. Today, prophylactic (preventive) factor replacement therapy is the typical care for hemophilia B treatment. Before factor concentrates were available, the life expectancy of a person living with hemophilia was only 13 years.

If you’ll be doing an activity you know is likely to raise the risk of bleeding, taking factor in advance can also help prevent joint bleeds — episodes of bleeding that occur within the joints of the body.

Gene Therapy

Gene therapy is a new treatment for hemophilia B that may help people live longer by reducing the risk of bleeds, although long-term studies are still needed.

In 2022, the U.S. Food and Drug Administration (FDA) approved the first gene therapy for hemophilia B — etranacogene dezaparvovec (Hemgenix). This treatment introduces a working version of the gene for factor IX into the body. A working version of the gene means a healthy and functional copy of the gene that provides the body with the instructions it needs to make a specific protein, in this case, factor IX. The working version of the factor IX gene can allow people with hemophilia B to produce their own factor IX. Etranacogene dezaparvovec is given as a one-time infusion.

This is a new treatment, so we don’t yet know how it may affect the life expectancy of people taking it. A clinical trial is a scientific research study that tests new treatments, medications, or medical procedures to see how safe and effective they are for people. Current clinical trials have only followed people for two years after receiving the etranacogene dezaparvovec infusion. In one such study, Hemgenix resulted in a 54 percent decrease in annual bleeding rates in people with hemophilia B.

A long-term follow-up study is planned to measure the outcomes of those who received etranacogene dezaparvovec for up to 15 years. This means that scientists will keep watching and studying this group over many years to see if their health changes or stays the same.

Learn more details about gene therapy for hemophilia.

Talk With Others Who Understand

MyHemophiliaTeam is the social network for people with hemophilia and their loved ones. On MyHemophiliaTeam, more than 6,100 members come together to ask questions, give advice, and share their stories with others who understand life with hemophilia.

Do you have concerns about life expectancy with hemophilia B? Have you had a conversation with your doctor about ways to lower your risk for life-threatening bleeds or other diseases? Share your experience in the comments below, or start a conversation by posting on your Activities page.