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In 1960, the life expectancy for a person with severe hemophilia was around 20 years old. Today, the life expectancy for those with this inherited bleeding disorder is closer 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 lifespan.
People with hemophilia B — also known as Christmas disease — don’t have a normal amount of factor IX (9), a blood protein that makes 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.
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 an intracranial hemorrhage.
HIV/AIDS
People living with hemophilia B have a higher risk of death from infectious diseases such as HIV and hepatitis C virus (HCV). This increased risk is largely associated with past treatment practices that increased infectious disease exposure. Before blood safety measures were implemented along with sensitive screening tests, many people with hemophilia were exposed to contaminated factor products in the 1970s and 1980s.
Factor products are treatments made from blood donations to help people with bleeding disorders. At the time, these factor concentrates were made by pooling plasma from donors, so a single infected donor could contaminate an entire supply. Rates of infection were highest in those exposed to pooled plasma-derived factor concentrates.
From the late 1970s to the mid-1980s, about half of all people with hemophilia contracted HIV from infected blood products, and about 90 percent of people with severe hemophilia were infected with HIV. The number of people with hemophilia who died from HIV was highest between 1992 and 2001 and has been decreasing since then.
Hepatitis C
Similarly, the hepatitis C virus was spread through contaminated factor products before testing of donated blood began in 1992. At that point, an estimated 44 percent of people living with hemophilia had contracted hepatitis C. HCV infection can result in death due to chronic (ongoing) liver diseases such as cirrhosis, liver cancer, and end-stage liver disease.
Safer Blood Products
In the 1990s, stricter screening and viral inactivation methods (processes used to eliminate or make viruses inactive) were implemented to make factor products derived from donated blood safer and reduce the risk of infection.
Since then, recombinant (lab-made) factor products have been developed that aren’t derived from donated blood. 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. They have been shown to reduce blood-borne infection risk compared with older plasma-derived products.
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. It’s important to keep a hemophilia emergency kit in case of injuries.
Several factors influence your prognosis and life expectancy when you’re living with hemophilia B, including:
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.
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. Because hemophilia B is more common in people born male (since hemophilia genes are on X chromosomes), 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.
Hemophilia B can be classified as mild, moderate, or severe based on the factor IX activity level. Your doctor can assess the severity of your hemophilia.
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:
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 between 1977 and 1998 about people with hemophilia A and hemophilia B who weren’t infected with HIV. Researchers found that the median severe hemophilia life expectancy 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.
Advances in treatment options for hemophilia B have improved life expectancy. When factor IX concentrates were introduced in the 1970s, it became 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.
In the early 20th century, before advances in modern medicine like blood transfusions from stored blood and 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 is a new treatment for hemophilia B that may help people live longer by reducing the risk of bleeds. In 2022, the U.S. Food and Drug Administration (FDA) approved the first gene therapy for hemophilia B.
Gene therapy introduces a working version of the gene for factor IX into the body. This healthy and functional copy of the gene 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.
Gene therapy for hemophilia B is given as a one-time infusion.
This is a newer treatment, so researchers 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 for safety and efficacy.
Research from recent clinical trials that followed people for two years after receiving gene therapy for hemophilia B demonstrated a 54 percent reduction in annual bleeding rates. Five-year follow-up data shows even further reductions in annual bleeding rates, and researchers are continuing to monitor results.
A long-term follow-up study is also being conducted measuring the outcomes of those who received gene therapy for hemophilia B for up to 20 years.
On MyHemophiliaTeam, people share their experiences with hemophilia, get advice, and find support from others who understand.
Do you have concerns about life expectancy with hemophilia B? Let others know in the comments below.
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As a 72 year old person with severe Hemophilia B, I can say that survival and longevity is due to a combination of successful treatment and a healthy lifestyle, which includes diet, exercise and a… read more
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