Frequently Asked Questions About PI

What is PI?

PI, sometimes referred to as PIDD, is short for primary immune deficiency disease. Your immune system keeps you healthy by fighting off germs with antibodies. People living with PI have an immune system that is not working properly. The World Health Organization recognizes more than 200 types of PI. 11

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. Some patients with PI require Ig replacement; others will need other types of therapy.

Is PI contagious?

PI is a genetic condition that people are born with. It is not contagious, nor can it be spread to other people. Some people associate the words "immune deficiency" with the AIDS virus. However, AIDS is a secondary immune deficiency, not a primary immune deficiency (PI). A person with PI poses no risk to other people.2

What are the symptoms of PI?

PI has no unique or specific symptoms. It shares symptoms with many other conditions–symptoms that can range from mild to severe. Thus, it is often mistaken for ordinary infections, such as infections of the ears, gastrointestinal tract, sinuses, and/or lungs.3

If there are no unique or specific symptoms of PI, are there warning signs?

In 2012, the Jeffrey Modell Foundation created the 10 Warning Signs of PIDD to help raise awareness of these disorders. The following lists are the Modell Warning Signs for Children and Adults:

Warning Signs for Children:

  1. Four or more new ear infections within one year
  2. Two or more serious sinus infections within one year
  3. Two or more months on antibiotic with little effect
  4. Two or more pneumonias within one year
  5. Failure of an infant to gain weight or grow normally
  6. Recurrent, deep skin or organ abscesses
  7. Persistent thrush in mouth or fungal infection on skin
  8. Need for intravenous antibiotics to clear infections
  9. Two or more deep-seated infections including septicemia
  10. A family history of PI

Warning Signs for Adults:

  1. Two or more new ear infections within one year
  2. Two or more serious sinus infections within one year, in absence of allergy
  3. One pneumonia per year for more than one year
  4. Chronic diarrhea with weight loss
  5. Recurrent viral infections (cold, herpes, warts, condyloma)
  6. Recurrent need for intravenous antibiotics to clear infections
  7. Recurrent, deep abscesses of the skin or internal organs
  8. Persistent thrush or fungal infection on skin or elsewhere
  9. Infection with normally harmless tuberculosis-like bacteria
  10. A family history of PI

This information is available on the Jeffrey Modell Foundation website www.info4pi.org. If you or someone you know is affected by 2 or more of the warning signs above, speak to a physician about the possible presence of an underlying primary immunodeficiency. Your physician will likely refer you to an immune system specialist, or immunologist.

How is PI diagnosed?

An immunologist can help your physician diagnose and evaluate your immune system. This evaluation might include a detailed medical history, a physical exam, blood tests, and vaccines to test how well your immune system responds.

How many people have PI?

It is estimated that approximately 250,000 individuals (or 1 in 1,200) in the United States are diagnosed with PIDD.2 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.3 More than 200 types of PI are currently recognized by the World Health Organization.11

What is an option for treating PI?

One treatment option for certain patients with PI is immunoglobulin therapy, or Ig therapy, such as Hizentra. This therapy replaces or supplements the antibodies in the immune systems of people living with PI. The immunoglobulin in Ig therapy is made from the blood plasma of carefully screened donors. It is manufactured and purified under strict conditions to help ensure safety. The risk of transmission of infectious agents cannot be completely eliminated.











Gain Freedom in Your Therapy With Hizentra

Hizentra provides a number of benefits for people living with primary immunodeficiency disease, including the ease of a treatment option you can administer yourself. No need to take time out of your schedule to receive treatment in an outpatient center or doctor’s office. Hizentra even provides the freedom to do certain activities while you infuse.

Important Safety Information
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Important Safety Information

WARNING: Thrombosis (blood clotting) can occur with immune globulin products, including Hizentra. Risk factors can include: advanced age, prolonged immobilization, a history of blood clotting or hyperviscosity (blood thickness), use of estrogens, installed vascular catheters, and cardiovascular risk factors.

If you are at high risk of thrombosis, your doctor will prescribe Hizentra at the minimum dose and infusion rate practicable and will monitor you for signs of thrombosis and hyperviscosity. Always drink sufficient fluids before administration.

Immune Globulin Subcutaneous (Human), Hizentra®, treats various forms of primary immunodeficiency (PI) in patients age 2 and over.

Tell your doctor if you have had a serious reaction to other immune globulin medicines or have been told you also have a deficiency of the immunoglobulin called IgA, as you might not be able to take Hizentra. You should not take Hizentra if you know you have hyperprolinemia (too much proline in your blood).

Infuse Hizentra under your skin only; do not inject into a blood vessel.

Allergic reactions can occur with Hizentra. If your doctor suspects you are having a bad allergic reaction or are going into shock, treatment will be discontinued. Immediately tell your doctor or go to the emergency room if you have signs of such a reaction, including hives, trouble breathing, wheezing, dizziness, or fainting.

Tell your doctor about any side effects that concern you. Immediately report symptoms that could indicate a blood clot, including pain and/or swelling of an arm or leg, with warmth over affected area; discoloration in arm or leg; unexplained shortness of breath; chest pain or discomfort that worsens with deep breathing; unexplained rapid pulse; and numbness or weakness on one side of the body. Your doctor will also monitor symptoms that could indicate hemolysis (destruction of red blood cells), and other potentially serious reactions that have been seen with Ig treatment, including aseptic meningitis syndrome (brain swelling); kidney problems; and transfusion-related acute lung injury.

The most common drug-related adverse reactions in the clinical trial for Hizentra were swelling, pain, redness, heat or itching at the site of injection; headache; back pain; diarrhea; tiredness; cough; rash; itching; nausea and vomiting.

Hizentra is made from components of human blood. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, cannot be completely eliminated.

Before being treated with Hizentra, inform your doctor if you are pregnant, nursing or plan to become pregnant. Vaccines (such as measles, mumps and rubella) might not work well if you are using Hizentra. Before receiving any vaccine, tell the healthcare professional you are being treated with Hizentra.

Please see full prescribing information for Hizentra, including boxed warning and the patient product information.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.

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