Diagnosis and Treatment of PI

The National Institutes of Health estimates that there are approximately 500,000 Americans with undiagnosed PIDD.3

Primary immunodeficiency disease (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

Getting a Diagnosis of PI

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
10 Warning Signs of PI for Adults

The 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 from the Jeffrey Modell Foundation. The next step is to get an expert evaluation.

10 Warning Signs of PI for Children

The 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 from the Jeffrey Modell Foundation. The next step is to get an expert evaluation.

PI Treatment Options

Certain types of PI are associated with low immunoglobulin G levels (Ig are proteins that help fight infection); one treatment option is Ig therapy, which replaces the Ig G that is missing or in low supply. lg therapy is made from the blood plasma of carefully screened donors, and is manufactured and purified under strict conditions for efficacy and safety.

Because Ig therapy is made from blood plasma, it cannot be in pill form. Instead, Ig therapy is infused. Ig infusions are typically given 2 ways:

  • Just below the skin: subcutaneous Ig, or SCIg
    SCIg products (like Hizentra) are typically self-administered using an infusion pump and small needle (SCIg has typically been administered weekly; Hizentra is the first product approved for administration as often as daily or as infrequently as every 2 weeks.)
  • Into the vein: intravenous lg, or IVIg
    IVIg therapy is administered by a medical professional, into the vein, about every 3 to 4 weeks

Hear Dr. Wasserman Discuss IVIg vs. SCIg Therapy Options

“When considering the most appropriate route of administration of immunoglobulin therapy,there are [a number of] factors that play a role”

- Richard L. Wasserman, MD, PhD

Hear Prescribers Discuss Topics Surrounding SCIg Therapy

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

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.

Patient/Caregiver PI Learning Center Diagnosis & Treatment
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