Diagnosis and Treatment of PI

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

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.

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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.

View PDF >

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 weekly, using an infusion pump and small needle (the Ig therapy is infused under the skin)
• Into the vein: intravenous lg, or IVIg
  IVIg therapy is administered by a medical professional, into the vein, about every 3 to 4 weeks