Wheezing, shortness of breath and chronic bronchitis are often associated with asthma or chronic obstructive pulmonary disease (COPD). However, they are also symptoms of a serious genetic form of emphysema called Alpha-1 Antitrypsin Deficiency, also known as Alpha-1.

For Peggy Iverson and her family, the symptoms and risk of this life-threatening condition are all too familiar.  

Alpha-1 is a hereditary condition in which the body does not produce a protein known as alpha-1 antitrypsin, which protects lungs from damage. Alpha-1 has been identified in nearly all populations and ethnic groups, and it is the most common known genetic risk factor for emphysema. The National Institutes of Health estimate that more than 100,000 Americans may have Alpha-1; only 10 percent of these cases have been properly diagnosed. Up to 3 percent of people diagnosed with COPD may have undetected Alpha-1.

The importance of early diagnosis and intervention
Alpha-1 is commonly first misdiagnosed as asthma or COPD. In fact one study shows that 43 percent of Alpha-1 patients see at least three doctors before receiving a correct diagnosis – and that it takes an average of nearly eight years from first symptoms to diagnosis. During that time, lung damage progresses.

Peggy’s mother, Helen Kline, was 45 years old when she began experiencing shortness of breath. She learned she had lung damage, even though she had never smoked. Further testing soon revealed Helen had Alpha-1. Unfortunately, Helen’s lung function continued to decline, and she passed away from Alpha-1 in 1984, at the age of 54, just nine years after her diagnosis. Because of her mother’s diagnosis, Peggy was tested for Alpha-1 in her mid-20s and learned that she too had the condition.

For Peggy, this early diagnosis made all the difference. It allowed her to make lifestyle changes to protect her lungs, which included seeing a pulmonologist yearly.

Peggy was able to begin treatment immediately when her lung function began to decline in 2000. Nowadays, Peggy treats her condition with Zemaira® (Alpha-1 Proteinase Inhibitor [Human]), from CSL Behring, which works to restore the alpha-1 antitrypsin protein her body lacks.

Peggy, who is now 61 years old, is striving to help others with Alpha-1 find the education and support they need to manage their condition and lead happy lives.

Inspired to help
In 2006, Peggy attended her first Alpha-1 national conference and was inspired to begin an Alpha-1 support group in her hometown in Iowa. After running the local support group for five years, Peggy became a coordinator with AlphaNet, a not-for-profit organization serving the specific needs of those with Alpha-1.

As an AlphaNet Coordinator, Peggy provides support, education and resources to others with Alpha-1, on an ongoing basis, to help them manage their condition. Most important, though, she gives others hope.

“It is possible to live a great life with Alpha-1 due to the many resources and treatments available,” said Peggy.

This initiative is part of the Alpha-1 Disease Management and Prevention Program developed by AlphaNet to support those with Alpha 1, their families and healthcare providers. The goal of the program is to help those with Alpha-1 improve their quality of life and efficiently manage health resources.

“I feel blessed to serve those with Alpha-1 every day,” said Peggy. Peggy also heads fundraisers for Alpha-1 research and enjoys spending time with her husband, Pete, and two sons, both carriers of the Alpha-1 gene.

CSL Behring is a global biotherapeutics company committed to the Alpha-1 community. For more information about CSL Behring or Alpha-1, visit www.zemairacarez.com.

Who Should Be Tested for Alpha-1?
The American Thoracic Society (ATS) recommend Alpha-1 screening for people who have:  

• COPD, emphysema or irreversible asthma
• Recurrent pneumonia or bronchitis
• Unexplained liver disease      
• A brother or sister diagnosed with Alpha-1

Alpha-1 can be diagnosed through a simple blood test conducted by a healthcare professional.

Important Safety Information
Alpha1-Proteinase Inhibitor (Human), Zemaira® is indicated to raise the plasma level of alpha1-proteinase inhibitor (A1-PI) in patients with A1-PI deficiency and related emphysema. The effect of this raised level on the frequency of pulmonary exacerbations and the progression of emphysema have not been established in clinical trials.

Zemaira may not be suitable for everyone; for example, people with known hypersensitivity to components used to make Zemaira, those with a history of anaphylaxis or severe systemic response to A1-PI products, and those with certain IgA deficiencies. If you think any of these may apply to you, ask your doctor.

Early signs of hypersensitivity reactions to Zemaira include hives, rash, tightness of the chest, unusual breathing difficulty, wheezing, and feeling faint. Immediately discontinue use and consult with physician if such symptoms occur.

In clinical studies, the following adverse reactions were reported in at least 5% of subjects receiving Zemaira: headache, sinusitis, upper respiratory infection, bronchitis, fatigue, increased cough, fever, injection-site bleeding, nasal symptoms, sore throat, and swelled blood vessels.

Because Zemaira is made from human blood, the risk of transmitting infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, cannot be completely eliminated.

Please see full prescribing information for Zemaira.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call1-800-FDA-1088.

Photo courtesy of Getty Images (patient and physician)

Sponsored by CSL Behring/Zemaira