Primary Immunodeficiency Can Isolate People from World, Loved Ones

(Family Features) - Imagine living in a world where even common germs can trigger extreme illness that won't go away. This is the reality for nearly 10 million children and adults living with primary immunodeficiency (PI). PI encompasses a group of disorders, usually genetic, that result from having a dysfunctional immune system. People with PI are often unable to fight off dangerous infections caused by germs that would leave most other individuals unaffected.

10317bTed Sandilands has been dealing with PI his entire life, though he only recently knew that to be the case. For years, he experienced persistent allergy and gastrointestinal symptoms. He visited several physicians, but the relief that their treatments provided was only temporary. Sandilands had to adjust his life to avoid being exposed to germs that would make him ill again; as a result, he was unable to spend the time that he wanted with his grandchildren.

"Eventually my ear nose and throat doctor conducted initial tests that suggested I might have problems with my immune system," Sandilands said. "My allergist and immunologist then determined that I have primary immunodeficiency."

PI can be very difficult to diagnose because it brings symptoms that resemble other conditions, including asthma, sinus infections, allergies and gastrointestinal disorders. Often, doctors and patients will go through a series of treatments before arriving at a diagnosis of PI.

According to the National Institutes of Health, PI treatment falls into three stages: 1) treating the current infection, 2) avoiding germs and preventing exposure to new infections and 3) correcting the immunodeficiency.

Once diagnosed, Ted began receiving immunoglobulin (Ig) therapy and saw a marked improvement in his symptoms. His course of treatment led him from intravenous immunoglobulin (IVIg) to subcutaneous immunoglobulin (SCIg).

In 2010 the United States Food and Drug Administration approved Hizentra(r), (Immune Globulin Subcutaneous [Human], 20% Liquid), from CSL Behring. Hizentra is the first and only 20 percent SCIg approved in the U.S. for the treatment of people with PI. As an active person who travels frequently, Sandilands needed a therapy that would allow him to self-administer on his schedule. He inquired about Hizentra.

"Because Hizentra can be stored at room temperature (up to 25°C [77°F]) for up to 24 months, I can administer my treatment without warming and I don't have to worry about refrigerating the product when I'm traveling," said Sandilands. "I find this to be convenient and efficient, as my infusion time has been reduced from my previous therapy."

While Sandilands is committed to managing his condition weekly, he no longer has to schedule his life around treatment. He and his wife recently moved from Colorado to New York so they could spend time with their grandchildren.

"I spent many years of my life being sick, but now I'm out in the world doing more than I could have imagined," said Sandilands. "I'd encourage anyone who is frequently ill to be tested for primary immunodeficiency."

For more information on PI, please contact the leading PI patient advocate groups in the U.S., the Immune Deficiency Foundation at www.primaryimmune.org, and the Jeffrey Modell Foundation at www.info4pi.org.

Important Safety Information

Hizentra®, Immune Globulin Subcutaneous (Human), is indicated for the treatment of patients with primary immunodeficiency (PI).

Hizentra is contraindicated in individuals with a history of anaphylactic or severe systemic response to immune globulin preparations or components of Hizentra, and in persons with selective immunoglobulin A deficiency who have known antibody against IgA and a history of hypersensitivity. If anaphylactic reactions are suspected, administration should be discontinued immediately and the patient treated as medically appropriate. Because Hizentra contains the stabilizer L-proline, it is also contraindicated in patients with hyperprolinemia.

Hizentra is derived from human plasma. The risk of transmission of infectious agents including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, cannot be eliminated completely.
The most common drug-related adverse reactions, observed in 5 percent or more of subjects in the clinical study, were local injection-site reactions, headache, vomiting, pain, and fatigue.

Monitor patients for reactions associated with IVIg treatment that might occur with Hizentra, including renal dysfunction/failure, thrombotic events, aseptic meningitis syndrome (AMS), hemolysis and transfusion-related acute lung injury (TRALI).

For more information about Hizentra visit www.hizentra.com, for full prescribing information visit http://www.hizentra.com/prescribing-information.aspx.

Source

CSL Behring