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 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.

Patient/Caregiver PI Learning Center Diagnosis & Treatment
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