Can Primary Immunodeficiency Impact Life Expectancy?

Primary immunodeficiency (PI) refers to a group of over 500 genetic disorders that affect how the immune system works. Also known as “inborn errors of immunity,” PI is present from birth and weakens the body’s natural defenses against infection. Some parts of the immune system may be missing or not working properly.

Thanks! You've successfully submitted your vote.

Sign up to view the results!

Continue with Facebook

Sign up with your email

By joining, you accept our Terms of Use, and acknowledge our collection, sharing, and use of your data in accordance with our Health Data and Privacy policies.

Already a Member? Log in

Because of this, people with PI tend to get infections more often, and these infections can sometimes become severe. PI can also lead to other complications that may affect a person’s lifespan.

Fortunately, new treatments and early diagnosis have greatly improved the prognosis (outlook) and extended life expectancy for many people with PI. With ongoing medical care and healthy everyday habits, people with PI can often live longer, healthier lives.

Factors That Affect Life Expectancy With Primary Immunodeficiency

“Primary immunodeficiency” is an umbrella term for a wide range of inherited immune system disorders. There are six major categories of PI, but within those are hundreds of distinct conditions.

PI doesn’t just raise the risk of infections. It can also increase the likelihood of allergies, autoimmune conditions, and certain cancers. If not recognized and treated, these complications can be life-threatening.

Infants and young children with severe forms of PI face the highest risk to their health, especially if the condition isn’t diagnosed early. In the United States and several other countries, all newborns are now screened for the most serious form of PI — severe combined immunodeficiency (SCID) — using a special blood test. This allows doctors to detect the condition early, often before any symptoms appear.

With early treatment, more than 90 percent of babies with SCID survive at least five years, and many go on to live healthy lives. Still, because PI is rare, some doctors may not recognize it early on. Some people aren’t diagnosed until adulthood.

The sooner PI is identified and treated, the better the chances for long-term health. In some cases, such as SCID, early treatment with a bone marrow or stem cell transplant can cure the condition. Thanks to tools like newborn screening and earlier access to treatment, many children with PI are now surviving well beyond infancy and experiencing improved quality of life.

Common Variable Immune Deficiency

Research on common variable immunodeficiency (CVID), one of the more common types of PI, suggests that earlier symptom onset may be linked to a higher risk of early death.

One small study of 18 people with CVID reported an average age of death of 41 years. In those whose symptoms began before age 19, average age at death was reported as 31.

Infections were the leading cause of death among younger people with CVID. The risk of death was also higher for people with other health conditions or those taking immunosuppressive medications.

Hereditary Angioedema

Some types of primary immunodeficiency respond better to treatment than others. One example is hereditary angioedema (HAE), a rare form of PI.

Unlike many other PI conditions, HAE doesn’t increase the risk of infections. Instead, it causes sudden swelling in different parts of the body, including the airways, digestive system, and skin. These attacks can be painful, disabling, and — in some cases — life-threatening.

In the past, sudden airway swelling from HAE could lead to suffocation. Before effective treatments became available, the estimated mortality rate was 20 percent to 30 percent. The study also reported the average age of death from HAE attacks was just over 40.

Today, with access to preventive therapies and emergency treatments, most people with HAE can live full, long lives. Early and appropriate use of medications has helped reduce the severity and frequency of attacks.

Potentially Life-Extending Treatment Options

Getting an accurate diagnosis of primary immunodeficiency is the first step toward understanding how your immune system works and how to manage it. With the right diagnosis, you and your healthcare team can take steps to protect against severe infections and other complications linked to PI.

While life expectancy data reflects outcomes from the past, treatment options for PI are improving every year.

Today, people with PI have access to a wide range of therapies that can help them live longer and healthier lives. These may include:

• Antibiotics and antifungals to treat or prevent infections
• Biologics and other immunomodulators (medications that help regulate the immune system)
• Gene therapy to correct faulty immune-related genes (available for specific types of PI)
• Immunoglobulin replacement therapy (IRT to replace missing antibodies
• Stem cell transplants, which may cure some forms of PI, such as SCID
• Targeted therapies for HAE

Many types of PI are caused by antibody deficiencies. Antibodies help the body recognize and fight infections. If your body doesn’t make enough of certain antibodies, IRT can provide them through injections or infusions. These treatments offer temporary protection and are usually given every one to four weeks. Your immunologist will adjust your treatment based on your specific condition and needs.

People with HAE may experience swelling attacks that resemble allergic reactions. However, HAE doesn’t respond to standard allergy medications like antihistamines or EpiPens. Instead, HAE requires specific approved treatments. Getting the right diagnosis is crucial, as misdiagnosis can delay care and lead to serious complications.

How To Live Better With Primary Immunodeficiency

There’s no single roadmap for living with primary immunodeficiency, but certain daily habits can make a big difference.

Build Daily Habits That Protect Your Health

Daily habits can help prevent life-threatening complications. People with compromised immune systems should be mindful of:

• Washing hands regularly
• Avoiding contact with people who are sick
• Getting recommended vaccines
• Taking care of their teeth and gums
• Staying up to date with health screenings

With PI, your health needs to be a priority. That means making time for medical appointments and reaching out to your healthcare provider if you have questions or concerns. Routine practices like skin checks, blood pressure monitoring, and regular dental cleanings may not seem urgent, but they can help detect issues early — and potentially extend your life.

Share Questions and New Symptoms With Your Doctor

It’s also important to ask your healthcare provider about ways to stay safe in different settings, such as at work, while traveling, or in crowded environments. Let your provider know if you — or someone you care for with PI — gets sick, is injured, or develops new or unusual symptoms.

Frequent infections, like recurring sinusitis or ear infections, should prompt a follow-up call or visit. Staying on top of symptoms can prevent more serious complications down the line.

Take Care of Your Mental Health

It’s easy to feel overwhelmed by statistics. Hearing that the average age of death is in the early 40s for some forms of PI can be alarming. But it’s important to remember that these numbers reflect the people who died from the disease — not the many people living with it. Also, when babies with PI don’t survive, it lowers the average age of death across the board.

Ultimately, no one can predict exactly how long someone with PI will live. But quality of life is just as important as quantity.

People with PI have higher rates of anxiety and depression. Many struggle with sleep issues, fatigue, and social isolation. That’s why it’s important to care for your mental health, not just your physical health.

Family and friends may not fully understand what it’s like to live with the daily fear of getting sick or dying young. Finding a balance between caution and enjoying life isn’t easy. Consider joining a support group or connecting with a therapist to feel less alone on the journey.

Talk With Others Who Understand

On myPIteam, people come together to learn more about life with primary immunodeficiency disorders.

What’s your advice for enjoying life while still managing the risk of infectious diseases? Share your experience in the comments below.