PI is an inherited medical condition that affects the cells, tissues and organs of one’s immune system. Because the immune system for those living with PI does not work as it should, people with the condition are more susceptible to common infections and germs, and consequently, secondary complications that can be severe.
According to the World Health Organization, there are more than 150 different types of PI, each affecting different parts of the immune system, but all leading to severe and frequent infections. While the more severe forms of PI are often diagnosed shortly after birth, according to the American Academy of Family Physicians, many people aren’t diagnosed until their 20s or 30s.
Living an "Extreme" Life despite Primary Immunodeficiency
Professional Supercross racer Carl Schlacht was diagnosed with Agammaglobulinemia, a disorder classified as primary immunodeficiency, at age one. "I decided early on that I would not let PI prevent me from competing in Supercross and achieving my lifelong goal of becoming a professional racer," said Carl, who turned pro in 2005.
Today, Carl’s one of approximately 80 racers who tour the U.S. each year competing in front of tens of thousands of fans at each venue. The sport, which consists of racing off-road motorcycles with steep jumps and obstacles, requires peak conditioning, daily practice and months of training leading up to the season.
"As a professional athlete I always have to be at my physical best," said Carl. "This means I have to manage my condition appropriately to ensure I have the right steady-state of immunoglobulin in my body. Immunoglobulin helps protect me against infections."
Carl manages his condition weekly with Hizentra®, Immune Globulin Subcutaneous (Human), from CSL Behring. Hizentra is an immunoglobulin replacement therapy, indicated for the treatment of primary humoral immunodeficiency, that is administered subcutaneously (i.e., under the skin).
The Jeffrey Modell Foundation has identified 10 general warning signs of Primary Immunodeficiency in children:
- Four or more new ear infections within 1 year
- Two or more serious sinus infections within 1 year
- Two or more months on antibiotics with little effect
- Two or more pneumonias within 1 year
- Failure of an infant to gain weight or grow normally
- Recurrent, deep skin or organ abscesses
- Persistent thrush in mouth or fungal infection on skin
- Need for intravenous antibiotics to clear infections
- Two or more deep-seated infections including septicemia
- A family history of PI
"Treating PI weekly keeps my immunoglobulin serum levels at a steady-state," said Carl. "This works for me. Due to the product’s low volume it only takes about an hour to an hour and a half a week to self-administer. The ability to manage my condition wherever and whenever I choose is really convenient as I lead a fast-paced life."
Carl added, "I’d recommend anyone experiencing the warning signs of PI to visit his or her doctor. I also encourage people who have PI or another serious medical condition to chase their dreams and not let their condition hold them back."
Carl, who is sponsored by CSL Behring, plans to take his own advice and continue competing at the highest level of his sport.
For more information about primary immunodeficiency, visit www.treatingPI.com, and to learn more about Hizentra, visit www.Hizentra.com.
Important Safety Information
Immune Globulin Subcutaneous (Human), Hizentra®, treats various forms of primary immunodeficiency (PI) in patients age 2 and over.
Hizentra should not be used if you have had serious negative reactions to immune globulin (Ig) preparations or a deficiency of an Ig known as IgA. Because Hizentra contains the amino acid proline as stabilizer, patients with hyperprolinemia (too much proline in the blood) should not take Hizentra.
Infuse Hizentra under your skin only; do not inject into a blood vessel.
Allergic reactions can occur with Hizentra. If your doctor suspects you are having a bad allergic reaction or are going into shock, treatment will be discontinued. Immediately tell your doctor or go to the emergency room if you have signs of such a reaction, including hives, trouble breathing, wheezing, dizziness, or fainting.
Tell your doctor about any side effects that concern you. Your doctor will monitor for potentially serious reactions that have been seen with Ig treatment, including thrombotic events (blood clotting); aseptic meningitis syndrome (brain swelling); osmotic nephropathy (a kidney condition); hemolysis (a blood problem) and transfusion-related acute lung injury.
The most common drug-related adverse reactions in the clinical trial for Hizentra were injection-site reactions (swelling, pain, redness, heat or itching); headache; back pain; diarrhea; tiredness; cough; rash; itching; nausea and vomiting.
Hizentra is made from components of human blood. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, cannot be completely eliminated.
Vaccines (such as measles, mumps and rubella) might not work as well if you are using Hizentra. Before receiving a vaccination, tell the healthcare professional that you are being treated with Hizentra. Also tell your doctor if you are pregnant or nursing, or if you plan to become pregnant.
For full prescribing information, visit http://www.hizentra.com/consumer/prescribing-information.aspx.
You are encouraged to report negative effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.
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Media Contacts:
Greg Healy
Senior Manager, Public Relations and Communications
US Commercial Operations
CSL Behring
1020 First Avenue
610-878-4841
greg.healy@cslbehring.com