• Life expectancy with hemophilia A has increased from 20 to 30 years in the 1960s to over age 70 in recent years.
• The severity of someone’s hemophilia is an important factor in their prognosis (outlook).
• Advances in hemophilia treatment have contributed to the improved prognosis, and new treatments may continue this trend.

In this article, we’ll cover what the life expectancy is for someone living with hemophilia A and how it’s changed over the years. We’ll also cover how severity and complications may affect prognosis and how new treatments may help.

Life Expectancy Over Time

Hemophilia A is a blood clotting or bleeding disorder caused by low levels of clotting factors — proteins that help your blood create clots. People with hemophilia A have mutations in the gene that controls factor VIII (8) production. People without enough of the clotting factor VIII protein are at a high risk of bleeding episodes.

To better understand how hemophilia A treatment has improved over the years, we’ll walk you through the past nearly 100 years of development.

The Development of Replacement Clotting Factor

The first successful documented hemophilia treatment was performed in 1940. Doctors treated an 11-year-old boy with a whole-blood transfusion, and he survived.

In 1964, researchers found a way to separate clotting factors from donor blood. The resulting treatment improved the average life expectancy for people with hemophilia to 60 years.

However, in the 1980s, doctors found that many donors were infected with the hepatitis C virus (HCV) or human immunodeficiency virus (HIV). Health care facilities had to take extra steps to properly clean and filter the clotting factors to prevent recipients from developing life-threatening complications. Even so, many people with hemophilia who received these contaminated proteins were infected with HCV or HIV.

By the 1990s, these plasma-derived factor concentrates were made much safer as health care facilities started to screen blood donors for HIV and hepatitis viruses. Viral-inactivation techniques — which involves either removing viruses from samples or making them noninfectious — made these concentrates safer.

Recombinant Clotting Factors Reduce Infections

In the mid-1980s, scientists sequenced the gene-providing instructions for factor VIII. This meant that researchers could create recombinant clotting factors without needing human donors. This technique gives the healthy factor VIII gene to animal cells in dishes, which then create the proteins like tiny factories.

Today, the U.S. Food and Drug Administration (FDA) has approved both human and recombinant factor VIII treatments. There hasn’t been a documented case of HCV or HIV infection from clotting factor treatment since 1985.

Better Treatments Have Improved Survival

With the discovery of factor concentrates, life expectancy with hemophilia A has increased over the past five decades. One study from the Netherlands followed 1,031 men with hemophilia — 87 percent of whom had hemophilia A. The authors found that the median life expectancy for all the participants from 1973 to 1986 was 66 years. This means half of these individuals lived to age 66 or more. From 2001 to 2018, median life expectancy for participants was 77 years — an increase of 11 years.

By comparison, median life expectancy among the men in the general population during those same time periods went from 79 to 83 years, a four-year increase.

Prophylactic Treatment, Survival, and Quality of Life

Researchers have found that prophylactic treatment with clotting factor concentrates are effective in helping manage hemophilia A and B. The term “prophylaxis” means “an action to prevent an illness or disease.”

Children with severe hemophilia A should start treatment when they’re young to prevent bleeding events. If your child is diagnosed with severe hemophilia A, their doctor may prescribe regular clotting factor replacement therapy. About 30 percent of people with severe hemophilia A develop inhibitors, which are immune proteins that make clotting factor less effective and hemophilia harder to treat. Developing inhibitors can also affect a person’s prognosis. Doctors sometimes prescribe emicizumab-kxwh (Hemlibra) to people who’ve developed inhibitors to help manage bleeding episodes.

Intracranial hemorrhages — bleeding within the brain tissue or between the brain tissue and skull — are a leading cause of death among people living with hemophilia. Studies show that prophylaxis helps reduce the risk of dangerous and even life-threatening bleeds, including intracranial hemorrhages. As such, prophylactic treatment may help people with severe hemophilia A live longer lives. Many people with severe hemophilia A also develop joint damage due to internal bleeding. By following your treatment plan closely, you can prevent these complications and improve your quality of life as well.

Severity of Hemophilia and Prognosis

The severity of your hemophilia plays a major role in your outlook. This is determined by the factor VIII levels in your blood, as follows:

• Mild hemophilia A — Five percent to 40 percent of healthy factor VIII levels
• Moderate hemophilia A — One percent to 5 percent of healthy factor VIII levels
• Severe hemophilia A — Less than 1 percent of healthy factor VIII levels

Read more about how your doctor determines your severity.

According to the FDA, around 60 percent of hemophilia A cases are classified as severe. The mortality (death) rate for men with hemophilia is twice that of healthy men in the general population — and for those with severe forms of hemophilia, it’s four to six times higher.

The study conducted in the Netherlands found that men with severe hemophilia had a lower median life expectancy than those with mild or moderate hemophilia. From 1992 to 2001, the median life expectancy was 59 years. However, it increased to 73 years between 2001 and 2018. This means that half of the men with severe hemophilia studied between 2001 and 2018 lived at least 73 years. In 2021, the average life span for an American man was just over 76 years, according to the U.S. Centers for Disease Control and Prevention (CDC). This puts the life expectancy of men with severe hemophilia A within three years of normal life expectancy.

Gene Therapy and Prognosis

In June 2023, the FDA approved Roctavian, a formulation of valoctocogene roxaparvovec-rvox, for treating people with severe hemophilia A. This gene therapy, administered as a one-time infusion, delivers a healthy copy of the factor VIII gene using a harmless virus known as adeno-associated virus 5 (AAV5). Your liver cells use the instructions provided in the treatment to start making their own factor VIII.

Before you can receive valoctocogene roxaparvovec-rvox, you’ll first be tested for immune system proteins (antibodies) that may attack the AAV5 virus. If you have these antibodies, the treatment may not be as effective.

A phase 3 clinical trial treated 134 men with severe hemophilia A who were previously treated with factor VIII replacement therapy for at least one year. They all received valoctocogene roxaparvovec-rvox and were followed for up to three years after treatment. Doctors and researchers performing the study found that gene therapy treatment reduced the average yearly use of factor replacement therapy by 98.6 percent. Overall, they reported that 90.3 percent of study participants had no treated bleeds or fewer following treatment.

Unfortunately, no current data shows how valoctocogene roxaparvovec-rvox may lengthen life expectancy in people with severe hemophilia A. More long-term studies are needed to measure whether gene therapy treatment can improve prognosis.

Talk With Your Doctor About Your Prognosis With Hemophilia A

Every person’s hemophilia case is unique. While general information can give you an idea of your outlook and life expectancy living with this disorder, there are many factors that can affect it. To learn more about your specific prognosis and what factors influence it, talk with your health care provider.

It’s also important to stick closely to your hemophilia treatment plan to prevent serious bleeding episodes and complications. You can work with your care team to create and follow a comprehensive care plan to prevent and manage bleeding with hemophilia A. As new treatments become available, your options may expand.

Talk With Others Who Understand

MyHemophiliaTeam is the social network for people with hemophilia and their loved ones. On MyHemophiliaTeam, more than 6,000 members come together to ask questions, give advice, and share their stories with others who understand life with hemophilia.

Have you discussed your prognosis with your doctor? Have you asked about newer treatment options and whether they might improve your outlook? Share your questions in the comments below, or start a conversation by posting on your Activities page.