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 clots) 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 blood clots, your doctor will prescribe Hizentra at the minimum dose and infusion rate practicable and will monitor for signs of clotting events and hyperviscosity. Always drink sufficient fluids before infusing Hizentra.

See your doctor for a full explanation, and the full prescribing information for complete boxed warning.

Hizentra®, Immune Globulin Subcutaneous (Human), 20% Liquid, is a prescription medicine used to treat:

  • Primary immune deficiency (PI) in patients 2 years and older
  • Chronic inflammatory demyelinating polyneuropathy (CIDP) in adults

Treatment with Hizentra might not be possible if your doctor determines you have hyperprolinemia (too much proline in the blood), or are IgA-deficient with antibodies to IgA and a history of hypersensitivity. Tell your doctor if you have previously had a severe allergic reaction (including anaphylaxis) to the administration of human immune globulin. Tell your doctor right away or go to the emergency room if you have hives, trouble breathing, wheezing, dizziness, or fainting. These could be signs of a bad allergic reaction.

Inform your doctor of any medications you are taking, as well as any medical conditions you may have had, especially if you have a history of diseases related to the heart or blood vessels, or have been immobile for some time. Inform your physician if you are pregnant or nursing, or plan to become pregnant.

Infuse Hizentra under your skin only; do not inject into a blood vessel. Self-administer Hizentra only after having been taught to do so by your doctor or other healthcare professional, and having received dosing instructions for treating your condition.

Immediately report to your physician any of the following symptoms, which could be signs of serious adverse reactions to Hizentra:

  • Reduced urination, sudden weight gain, or swelling in your legs (possible signs of a kidney problem).
  • Pain and/or swelling or discoloration of an arm or leg, unexplained shortness of breath, chest pain or discomfort that worsens on deep breathing, unexplained rapid pulse, or numbness/weakness on one side of the body (possible signs of a blood clot).
  • Bad headache with nausea; vomiting; stiff neck; fever; and sensitivity to light (possible signs of meningitis).
  • Brown or red urine; rapid heart rate; yellowing of the skin or eyes; chest pains or breathing trouble; fever over 100°F (possible symptoms of other conditions that require prompt treatment).

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

The most common side effects in the clinical trials for Hizentra include redness, swelling, itching, and/or bruising at the infusion site; headache; chest, joint or back pain; diarrhea; tiredness; cough; rash; itching; fever, nausea, and vomiting. These are not the only side effects possible. Tell your doctor about any side effect that bothers you or does not go away.

Before receiving any vaccine, tell immunizing physician if you have had recent therapy with Hizentra, as effectiveness of the vaccine could be compromised.

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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