The PROOF is in our PI indication

Children with PI

The more you know about PI, the more you can help your child face the challenges of living with a chronic disease. Bringing family members, friends, and teachers into the loop will encourage the kind of understanding and special consideration you and your child need.

Let them know that your child has a primary immunodeficiency and that the condition is inherited.¹ It is not contagious, and has absolutely no connection with AIDS (acquired immunodeficiency syndrome). Your child is not a threat to other children. However, other children carry and pass around germs that can cause illness in a child with PI.

Work with your child's school

Discuss your child's condition with teachers and the school principal. Let them know that your child is susceptible to frequent infections and will possibly be absent more than other children. Discuss special requirements your child may have, such as taking medications during the day or needing to make frequent trips to the bathroom.

Let teachers know that intravenous infusions must happen on a regular basis, and that your child will be absent during these times. Give them the infusion schedule if possible.

Keeping a journal for your child

It's important for you to keep teachers, doctors, and other healthcare professionals aware of what's going on with your child. A journal may make this easier. You can use a journal to record your child's medical history, complete with medicines, treatments, lab reports, a contact list of healthcare professionals, insurance information, infusion information, and anything else you or your doctor thinks may be important regarding proper care for your son or daughter.

Having a journal is often invaluable in an emergency situation, when less familiar healthcare providers may need information quickly.

Telling children about illness

You must decide when and how to tell your child about chronic illness. Anxiety, confusion, and self-esteem issues are sure to come up, and will need to be discussed openly. When you know what's troubling your child, you'll be better able to help overcome his or her concerns.

The Immune Deficiency Foundation (IDF) Patient & Family Handbook for Primary Immunodeficiency Diseases, listed below, may prove helpful in discussing PI with your son or daughter. Click the link at the end of this section to download.

Infusions–prepare your child ahead of time

A little preparation before that first infusion can go a long way. Click on links below for comic books that explain in fun, friendly, easy-to-understand terms what your child can expect at that first infusion.

Bring any favorite games, books, portable DVD players, etc, to make the infusion time go faster.

Download the following Comic Books, or Register to receive print copies in the mail

Awesome Adventures of AndiBody

Sally's First Infusion

The source for the information in this section was the IDF Patient & Family Handbook for Primary Immunodeficiency Diseases, 4th Edition.

View the entire book online at the Immune Deficiency Foundation Web site.

Important Safety Information for GAMUNEX

Gamunex, Immune Globulin Intravenous (Human), 10% Caprylate/Chromatography Purified, is indicated for the treatment of primary humoral immunodeficiency disease (PI), idiopathic thrombocytopenic purpura (ITP), and chronic inflammatory demyelinating polyneuropathy (CIDP).

Immune Globulin Intravenous (Human) products have been reported to be associated with renal dysfunction, acute renal failure, osmotic nephrosis and death. Patients predisposed to acute renal failure include patients with any degree of pre-existing renal insufficiency, diabetes mellitus, age greater than 65, volume depletion, sepsis, paraproteinemia, or patients receiving known nephrotoxic drugs. Especially in such patients, IGIV products should be administered at the minimum concentration available and the minimum rate of infusion practicable. While these reports of renal dysfunction and acute renal failure have been associated with the use of many of the licensed IGIV products, those containing sucrose as a stabilizer accounted for a disproportionate share of the total number. Gamunex does not contain sucrose. Glycine, a natural amino acid, is used as a stabilizer.

Gamunex is contraindicated in individuals with acute severe hypersensitivity reactions to Immune Globulin (Human). It is contraindicated in IgA deficient patients with antibodies against IgA and history of hypersensitivity.

There have been reports of noncardiogenic pulmonary edema [Transfusion-Related Lung Injury (TRALI)], hemolytic anemia, and aseptic meningitis in patients administered with IGIV.

Thrombotic events have been reported in association with IGIV. Patients at risk for thrombotic events may include those with a history of atherosclerosis, multiple cardiovascular risk factors, advanced age, impaired cardiac output, and/or known or suspected hyperviscosity. Hyperproteinemia, increased serum viscosity, and hyponatremia may occur in patients receiving IGIV therapy.

Gamunex is made from human plasma. Because this product is made from human plasma, it may carry a risk of transmitting infectious agents, e.g., viruses, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.

In clinical studies, the most common adverse reactions with Gamunex were headache, fever, chills, hypertension, rash, nausea, and asthenia (in CIDP); headache, cough, injection site reaction, nausea, pharyngitis, and urticaria (in PI); and headache, vomiting, fever, nausea, back pain, and rash (in ITP). The most serious adverse reactions were pulmonary embolism (PE) in one subject with a history of PE (in CIDP), an exacerbation of autoimmune pure red cell aplasia in one subject (in PI), and myocarditis in one subject that occurred 50 days post-study drug infusion and was not considered drug related (in ITP).

Please see accompanying GAMUNEX full Prescribing Information for complete prescribing details.

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.

Reference:

Fauci AS, Braunwald E, Kasper DL, et al, eds. Harrison's Principles of Internal Medicine. 17th ed. New York, NY: McGraw-Hill; 2008.