PI stands for primary humoral immunodeficiency disease. There are more than 400 different kinds of PI.1 With PI, part of your immune system, which is what protects you from infections and fights off diseases, is missing or doesn't function properly. As a result, your body can't protect you from germs as well as it should, so you are more likely to develop infections and be sick more often.
PI is not contagious; you can't catch it from someone else. PI is usually inherited, which means it is carried through the genes of your parents. If you have the disease, you might pass it along to your children, but you won't give it to anyone else.1
If you or someone you know has PI, you know how difficult it can be. But there is good news, too. There are FDA-approved treatment options* available, as well as lots of support. If you have PI, you are certainly not alone.
Understanding PI symptoms and diagnosis
The signs and symptoms of PI vary from person to person. If someone you know is experiencing any of the symptoms below, encourage them to talk to their doctor. Early detection of PI can help prevent long-term problems.
If a person is experiencing recurrent and/or chronic infections, lab testing may be necessary to detect the presence of PI. The patient's medical history and physical exam will help determine the appropriate choice of lab tests. Blood samples and DNA analysis can be used to confirm a particular diagnosis.1
The most common symptoms1,2
- Chronic, recurring infections that are more severe, longer lasting, and more difficult to cure, such as sinus infections, when compared to people with healthy immune systems
- Frequently getting sick with infections, such as a common cold, that a healthy immune system would normally be able to fight off
- Autoimmune problems, such as autoimmune lung or skin disease, which happen when the immune system attacks the body's own organs. Other issues include anemia, celiac disease, arthritis, lupus, and thyroid disorders
What causes PI?
Overview of the immune system
The immune system is an incredible collection of cells, proteins, tissues, and organs that defends against a wide variety of germs every day.2
How does the immune system work?
The immune system in people with PI1
Usually, the immune system does a great job protecting the body from threats. But in people who have PI, the immune system doesn't work properly. In some cases, the immune system may not work at all. With a lower amount of antibodies, germs are more likely to get through your body's defenses.
Treatment options for PI
In addition to treating PI, it's important to deal with the disease itself, so you will be less likely to get an infection in the first place. Fortunately, there are several treatments available2:
XEMBIFY is an immune globulin (IG)
IG stands for immune globulin. Immune globulin, or IG, is a medicine made from healthy proteins taken from human plasma and contains purified antibodies. Plasma is the liquid part of the blood that contains antibodies needed to keep the body healthy.
Immune globulin is given to people with PI to replace missing proteins to help the immune system protect against germs and other threats. It is sometimes referred to as IG replacement therapy.
Two ways that IG therapy is given
If infused by IV, it is given every 3 to 4 weeks; if given subcutaneously, it can be given daily to weekly, as desired. Also, while intravenous infusion is administered by a healthcare professional in a clinic or home, subcutaneous infusion can be self-administered conveniently at home.
XEMBIFY is a subcutaneous IG replacement therapy used to treat patients with PI who are 2 years of age and older. Learn more about infusing XEMBIFY.