What Is Primary Immunodeficiency Disease (PIDD)?

Your immune system keeps you healthy by fighting off germs. But people living with primary immunodeficiency disease (PIDD, sometimes known as just PI) have an immune system that is not working correctly.

The World Health Organization currently recognizes more than 200 types of PIDD. 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 it3
  • About 80% of people with PI are diagnosed before the age of 20, but PI may not be recognized until adulthood4
  • Some 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
  • See the FAQ about PI page above for more information.

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.

Clinical Perspectives

Hear prescribers discuss the benefits of customizing Ig therapy for each of their patients with PI.

Hizentra Is a 20% SCIg Therapy With Proven Efficacy and Safety in Both Adults and Children Age 2 Years and Older

For Adults
Living With PI

If you or someone you love has PI, you can still live a full and active life. Thanks to new therapies and medical advances, people living with PI go to school and work, socialize and play sports. In addition to medical treatment, the following tips may also help you or your loved one better manage the condition.

For Parents
& Caregivers

If you're the parent or caregiver of a child with PI, you're likely faced with coordinating his or her care as well as communicating with others about your child's condition. The following information provides helpful tips in accomplishing both.

Important Safety Information

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.

CSL Behring
Hizentra is manufactured by CSL Behring AG and distributed by CSL Behring LLC.
Hizentra® is a registered trademark of CSL Behring AG.
© 2018 CSL Behring LLC. The product information presented on this site is intended for US residents only. HIZ/09-12-0016k(1) 9/2015