Primary Immunodeficiency

What is Primary Immunodeficiency?

Your immune system keeps you healthy by fighting off germs. But people living with primary immunodeficiency disease (PID, sometimes known as PI or PIDD) have an immune system that is not working properly, beginning at birth. For people living with PID, 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, but once correctly diagnosed and treated, people with PID can live full and active lives.

PI Patient Janet Richards

What are the signs of Primary Immunodeficiency?

The National Institutes of Health estimates that there are approximately 500,000 Americans with undiagnosed PID. PID often goes untreated because there are no unique or specific symptoms, but there are common signs.

Learn About the 10 Warning Signs
PI Patient Ben Davies

How is Primary Immunodeficiency diagnosed?

If you believe you might have PID, 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: a detailed medical history, a physical exam, blood tests and vaccines to test your immune response.

Learn More About Diagnosis

Here is a sampling of our Vita stories on Primary Immunodeficiency:

To read all of our stories, please visit our Vita Stories page.

Schoolchildren in class
School, Sports and Sleepovers for Kids Who Have PI

Who needs to know about your child’s primary immune deficiency?

Primary immunodeficiencies affect more than 6 million people worldwide
The Facts About Primary Immunodeficiency (PI) Diseases

World PI Week promotes the early testing and diagnosis of primary immunodeficiencies.

IDF zebra mascot
Showing Stripes for Immunodeficiency Patients

Why the zebra symbolizes hope and progress in the rare disease community.

Resources for you

RESOURCE DESCRIPTION
Hizentra Facebook Page Provides PID patients and their families with information on Hizentra, patient stories and support programs. View Website  
Caring for a Child with PID As the parent or caregiver of a child living with PID, you are an important member of his or her healthcare team. You have to be aware of your child's symptoms and responses to treatment in order to effectively communicate with the healthcare team.
View Website 
Tips for Adults and Children Living with PID These tips from the Immune Deficiency Foundation can help make it easier to manage your or your loved one's condition. View Website 
Jeffrey Modell Foundation Answers, expert referrals, patient connections and patient organizations for people living with primary and secondary immunodeficiencies. View Website 
International Patient Organization for Primary Immunodeficiencies Improving awareness, diagnosis and treatment of primary immunodeficiencies worldwide.
View Website 
Immune Deficiency Foundation U.S. national patient organization dedicated to improving the diagnosis, treatment and quality of life of people with primary immunodeficiency diseases.
View Website 
Product Websites Hizentra.co.uk (United Kingdom)
Hizentra.com (United States)
Hizentra.de (Germany)
Privigen.com (United States)
Privigen.de (Germany)

Treatments Available

Availability of treatments may vary from country to country. Please be sure to visit your local CSLBehring.com site for further information.

Privigen® | Immune Globulin Intravenous (Human) 10% Liquid

US Prescribing Information

US Important Safety Information (Selected) 

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

In predisposed patients, kidney malfunction and acute kidney failure, potentially fatal, can occur with the administration of human immune globulin intravenous (IGIV) products. Kidney problems occur more commonly in patients receiving IGIV products that contain sucrose. Privigen does not contain sucrose.

If you are at high risk of thrombosis or kidney problems, your doctor will prescribe and administer Privigen at the minimum dose and infusion rate practicable, and will monitor you for signs and symptoms of thrombosis and viscosity, as well as kidney function. Always drink sufficient fluids before administration.

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

In clinical trials of Privigen, headache was the most common side effect. Other common side effects that can be seen with treatment include fatigue, nausea, fever, and high blood pressure. These are not the only side effects possible; see the full prescribing information for a complete list of adverse reactions possible with treatment for each condition. Alert your physician to any side effect that bothers you or does not go away.

Full US Important Safety Information

 
 Hizentra® | Immune Globulin Subcutaneous (Human) 20% Liquid

 US Prescribing Information

 US Important Safety Information (Selected)

WARNING:
Thrombosis (blood clots) can occur with immune globulin products, including Hizentra. Risk factors include: advanced age, prolonged immobilization, a history of blood clotting or 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 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.

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.

Full US Important Safety Information

 
 Carimune® NF | Nanofiltered, Immune Globulin Intravenous (Human)
 Known outside the US as: Sandoglobulin®, Redimune®, and Sanglopor®

 US Prescribing Information

 WARNING: THROMBOSIS, RENAL DYSFUNCTION or ACUTE RENAL FAILURE

  • Thrombosis may occur with immune globulin products, including Carimune NF. Risk factors may include: advanced age, prolonged immobilization, hypercoagulable conditions, history of venous or arterial thrombosis, use of estrogens, indwelling central vascular catheters, hyperviscosity, and cardiovascular risk factors.  Thrombosis can occur in absence of known risk factors.
  • Renal dysfunction, acute renal failure, osmotic nephrosis, and death can occur in predisposed patients with immune globulin intravenous (IGIV) products, including Carimune NF. Patients predisposed to renal dysfunction include those with any degree of preexisting renal insufficiency, diabetes mellitus, age over 65, volume depletion, sepsis, paraproteinemia, and those receiving known nephrotoxic drugs. Renal dysfunction and acute renal failure occur more commonly in patients receiving IGIV products containing sucrose, such as  Carimune NF.
  • For patients at risk of thrombosis, renal dysfunction or acute renal failure, administer Carimune NF at the minimum dose and infusion rate practicable.  Ensure adequate hydration in patients before administration.  Monitor for signs and symptoms of thrombosis and assess blood viscosity in patients at risk for hyperviscosity.
  • See full prescribing information for full boxed warning.

    Slow or temporarily stop infusion if patient experiences facial flushing, tightness in chest, chills, fever, nausea, dizziness or other unusual response; stop infusion immediately if anaphylaxis or severe reaction occurs.  Headache, usually mild, is the most common adverse reaction; mild hemolysis, arthralgia, myalgia, and transient skin reactions have also been reported.

 Full US Important Safety Information

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