Get the facts about life with PI

If you or someone you love has primary immunodeficiency disease (PI), also known as PIDD, it's still possible to live a full and active life. Thanks to new therapies and medical advances, people living with PI can go to school, work, socialize, and play sports.

What is primary immunodeficiency disease (PI)?

Your immune system keeps you healthy by fighting off germs. But people living with PI have an immune system that is not working correctly.

The World Health Organization currently recognizes more than 300 types of PI. It is estimated that approximately 250,000 individuals (or 1 in 1,200) in the United States have been diagnosed with PI. Although rare, PI is not as uncommon as once believed.

  • According to the National Institutes of Health, about 500,000 people may have PI and not know it
  • About 80% of people with PI are diagnosed before the age of 20, but PI may not be recognized until adulthood
  • Examples of PI are common variable immunodeficiency (CVID), selective immunoglobulin A (IgA) deficiency, X-linked agammaglobulinemia (XLA), autosomal recessive agammaglobulinemia (ARA), and hyper IgM (HIGM) syndrome

For people living with PI, infections may not go away or can come back often, even with the use of antibiotics. Infections may be common, severe, long-lasting, or hard to cure. Appropriate therapy can prevent complications associated with PI, such as frequent infections, fevers, long-term permanent organ damage, and premature death.

Could I or my child have PI?

The National Institutes of Health estimates that there are approximately 500,000 Americans with undiagnosed PI.

PI often goes untreated because there are no unique or specific symptoms. Symptoms can:

  • Range from mild to severe
  • Be mistaken for ordinary infections of the sinuses, ears, or lungs
  • Cause gastrointestinal problems or inflammation in joints
PI Adults warning signs

10 Warning Signs for PI for Adults

If you suspect that you or someone you know has PI, review this list of 10 warning signs of PI from the Jeffrey Modell Foundation. The next step is to get an expert evaluation.

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PI Children warning signs

10 Warning Signs for PI for Children

If you suspect that your child or the child of someone you know has PI, review this list of 10 warning signs of PI from the Jeffrey Modell Foundation. The next step is to get an expert evaluation.

View PDF

How is PI diagnosed?

If you believe you might have PI, the first step is to get an expert evaluation. An immune system specialist, called an immunologist, can help with diagnosis and treatment. When an immunologist evaluates your immune system, the evaluation may include:

  • Detailed medical history
  • Physical exam
  • Blood tests
  • Vaccines to test your immune response

Are there any materials I can review?

A list of resources is provided below. Simply click on the link below to view, download, or print your selected item.

Electronic personal health record image

Electronic Personal Health Record

The Immune Deficiency Foundation (IDF) eHealthRecord is a one-of-a-kind electronic personal health record developed for individuals and families living with primary immune deficiency diseases.

View/Download PDF
PI Adults warning signs

10 Warning Signs of PI for Adults

If you suspect that you or someone you know has PI, review this list of 10 warning signs of PI from the Jeffrey Modell Foundation. The next step is to get an expert evaluation.

View/Download PDF
PI Children warning signs

10 Warning Signs of PI for Children

If you suspect that your child or the child of someone you know has PI, review this list of 10 warning signs of PI from the Jeffrey Modell Foundation. The next step is to get an expert evaluation.

View/Download PDF
Doctor Discussion Guide image

Doctor Discussion Guide

This guide will help you talk to your doctor about your condition, treatment options, and the unique features of Hizentra. Simply print this out and bring to your next doctor’s appointment.

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Video thumbnail

Hizentra Self-administration Video

Watch the Hizentra self-administration video to learn the step‑by‑step instructions on preparation, proper infusion techniques, and administration.

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Step-by-step instructions for Self-administering Hizentra

Step‑by‑Step Instructions for Self‑administering Hizentra

Once you've watched the video, use this step‑by‑step guide for administering.

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How can I talk to my family and friends about PI?

It's important for the entire family to know about PI. They need to understand that a person with PI can live a normal life with proper treatment.

Family needs to be aware that having PI makes a person more susceptible to getting sick. If they have friends with colds or the flu, they should make it a point not to bring them home.

Family and friends should also know that PI is not contagious and cannot be spread to others. Let them know that having PI does not necessarily limit a person’s activities.

How do I talk to my child’s educators about PI?

If your child has PI, it's important that other adults that he or she comes in contact with—teachers, school nurses, and administrators—understand the condition and its treatment.

Share These Important Points to Help Educators Understand

  • Explain that PI stands for "primary immunodeficiency disease," a genetic condition that makes your child susceptible to illness and infections
  • Reassure your child's educators that your child poses no risk to other children. PI is not contagious and cannot spread to other children or adults
  • Let educators know that your child can participate in classroom and playground activities
  • Describe how common germs can be especially harmful—even life-threatening—to your child's health
  • Request a 504 plan, a plan that you and the school develop to ensure your child receives accommodations that will ensure academic success and access to the learning environment

Ask Educators to Help Minimize Your Child's Exposure to Germs

  • Ask educators to encourage children to stay home from school when they are sick
  • Request that the educator separate as much as possible your child and any children who come to school sick
  • Appeal to the educator to reinforce good hygiene and encourage children to wash their hands after sneezing, blowing their noses, or using the restroom

Alert Educators to Recognize Potential Problems

  • Ask educators to contact you if your child appears overly tired, feverish, or chilled, or exhibits symptoms including cough, congestion, runny nose, earache, difficulty breathing, or headache
  • Communicate that your child's treatment experience may result in side effects, such as fatigue, headache, or swelling at infusion site

Tips for living with PI

The following tips will help people with PI:

  • Work collaboratively with your doctor to manage your PI, and ask about treatments that can fit easily into your lifestyle
  • Eat a healthy diet. A well-balanced, nutritious diet may help your body fight against infection. Your doctor may suggest a special diet in times of illness or if you have food sensitivities
  • Adopt good hygiene habits. Bathe or shower regularly. Wash your hands before meals, after outings, and after using the bathroom. You may want to carry disposable hand wipes or antibacterial gel for times when soap and water are not available
  • Take proper care of cuts or scrapes and clean them thoroughly
  • Visit your dentist regularly and brush your teeth and floss daily

Organizations that can help

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