Alpha-1-To-One Magazine Spring 2011

Vol. 9, No. 1 • SPRING 2011

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ALPHAPractical advice, personal experience, and

A MAGAZINE OF THE ALPHA-1 FOUNDATION

pertinent news for people touched by ALPHA-1

The

Crucial Study

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NONPROFIT ORG U.S. POSTAGE PAID MIAMI, FL PERMIT # 8124

Alpha-1 Future

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COPD Genes

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End of Life

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FOCUS ON THE FOUNDATION

Progress on many fronts We introduced aggressive new programs in 2010.

And we’re making good progress in each one of them. ven though 2010 was a difficult year economically, the Alpha-1 Foundation maintained our levels of funding for Alpha-1 research, and also launched several vital new programs. We’re happy to report good progress in all of these programs: • The Alpha-1 Project.

The ultimate mission of the Alpha-1 Foundation is research for a cure.

For that reason, we created The Alpha-1 Project. This is the Foundation’s venture philanthropy company whose goal is to invest directly in promising new Alpha-1 therapies developed by biotechnology companies. The Foundation is considering interesting proposals from two different companies, and this year we expect to announce our first investment in therapeutic development for Alpha-1.

• It’s All in the Family.

It’s All in the Family is our program to help Alphas and Alpha-1 carriers encourage their families to consider testing for Alpha-1. The program’s success has brought a significant increase in free and confidential testing, funded by the Foundation, through the Alpha-1 Coded Testing (ACT) Study. Thanks to ACT, Alphas have one of the very few rare-disease communities which has confidential testing available. You or your family members can apply directly online for ACT testing at http://tinyurl.com/actquestionaire • Alpha-1 Liver Disease Initiative.

As part of our Liver Initiative, we formed a panel of clinical and research experts to advise us on our future efforts on liver disease. The group has met several times and the Foundation has awarded its chair, Jeff Teckman, MD, a one-year planning grant to prepare a grant application to the National Institutes of Health for a largescale study of the natural history of Alpha-1.

Such a study could find genetic and environmental factors that may be responsible for the different severity of disease in Alpha-1. It could stimulate research and clinical testing of new therapies for both liver and lung disease in Alpha-1.

The Liver Initiative is one of many vital research areas being investigated by the Foundation. Last year we awarded $1.3 million in new research grants to 11 scientists. We will announce our new research awards for 2011 at the American Thoracic Society conference in Denver in May. As always, none of this would be possible without the many contributions of the Alpha-1 community, and we are grateful for your continued generous support.

For information about organizing a fundraising event to benefit Alpha-1 research, contact Angela McBride at 1.888.825.7421, Ext. 233, or amcbride@alpha-1foundation.org. To learn more about methods of giving, or to make an online donation, visit www.alpha-1foundation.org/help/.

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Practical advice, personal experiences, and pertinent news for people touched by Alpha-1 VOL. 9, NO. 1 Published by the Alpha-1 Foundation 2937 SW 27th Avenue, Suite 302 Miami, FL 33133 1.877.2.CURE.A1 (228.7321) www.alpha-1foundation.org Alpha-1 Foundation Board of Directors Executive Committee Ab Rees*, Chair William J. Martin II, MD, Vice-Chair Elaine Alfonzo +, Secretary Kenneth A. Irvine +, Treasurer Greg Hules*, Past Chair David A. Brenner, MD, Scientific Advisor Members Gordon E. Cadwgan, Jr., PhD* John S. (Jack) Reid + Joe Reidy* Stephen I. Rennard, MD Edwin K. Silverman, MD, PhD Director Emeritus Marilina V. Fernandez+ Editorial Board Bettina Irvine* Michael Krowka, MD William J. Martin II, MD Jane M. Martin, BA, CRT Miriam O’Day Ab Rees* Bruce C. Trapnell, MD John W. Walsh* Executive Editor Marcia F. Ritchie Managing Editor Bob Campbell Contributing Editors Laura Fleming Angela McBride Linda Rodriguez Robert A. Sandhaus, MD, PhD, FCCP Advertising If you are interested in advertising, please contact Bob Campbell at 888.825.7421 ext 230.

ALPHA-1 is published by the Alpha-1 Foundation with the support of our advertisers. No part of ALPHA-1 may be reproduced in any form by any means without prior written permission of the Alpha-1 Foundation.

The contents of ALPHA-1 are not intended to provide personal medical advice, which should be obtained directly from a physician. The Alpha-1 Foundation is not responsible for the accuracy of information expressed in advertisements in this publication.

Letters to the Editor. ALPHA-1 would like to hear from you.

Please send letters to the editor at the Foundation or e-mail us at editor@alpha-1foundation.org. Letters may be edited for clarity and length.

The Alpha-1 Foundation is dedicated to providing the leadership and resources that will result in increased research, improved health, worldwide detection, and a cure for Alpha-1 Antitrypsin Deficiency. * Diagnosed Alpha-1 Antitrypsin Deficient + Diagnosed Family Member

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Vol. 9, No. 1 • Spring 2011

A MAGAZINE OF THE ALPHA-1 FOUNDATION

Inside ALPHAFeatures

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SPOTLIGHT: Augmentation’s past and future The pivotal study Mark Wewers did the study that brought us augmentation therapy The future of a protein The inhaled augmentation study; new formulations and uses for Alpha-1 The Brantly Bunch The 1989-1996 longitudinal study of 1,129 Alphas led to a community

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FRONTIERS The COPD genes The many kinds of COPD, and why some Alphas get sicker than others

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IN YOUR INTEREST Working with the FDA Helping to speed the development of new therapies Speak up, the easy way The Action Center makes it easy to express your opinion to legislators

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THE END OF LIFE: A special section Talking about it How to avoid much stress, fear and family battles when a loved one dies The legal documents What to do, where to find the legal documents you need A gentle death For Marta Strock, it was all about quality of life. So she made her own choices.

A doctor’s advice Tom Petty, MD, on family conversations and medical care at the end of life

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ALPHA 411 The miracle dog Justin and Jamie Truett will get the specially-trained dog they need Two wonderful lives Ed Brailey and Dennis Pollock became Alpha-1 leaders after lung transplants Celebrating 20 years A new, improved Celeste

19 On the Web: ALPHA-1 LUNG DISEASE: Everything you want to know about Alpha-1 lung disease, including a Q&A page, on the Alpha-1 Foundation website, http://tinyurl.com/a1lung

www.alpha-1foundation.org

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SPOTLIGHT

The Pivotal Study Mark Wewers, MD, published one of the first studies showing the “biochemical effectiveness” of augmentation therapy in treating Alpha-1. is study, published in the New England Journal of Medicine in 1987, used concentrated and purified alpha-1 protein from the pooled blood plasma of healthy human volunteers. Made by Cutter Labs, this product later became Prolastin. Launched in 1988, Prolastin was the first augmentation therapy approved by the Food and Drug Administration (FDA).

“The 21 Alphas in this study were really pioneers, accepting what could have turned out to be substantial risks for augmentation infusions,” Wewers says.

The pivotal study found that augmentation had demonstrated “biochemical efficacy,” which the FDA accepted as a rationale for approving the drug in December, 1987.

Wewers’ article also said it would take an estimated 300 to 500 Alphas in both arms of a doubleblinded study for three years to show clear levels of clinical effectiveness for augmentation therapy.

“I still find it a difficult discussion with my Alpha-1 patients, when we talk about the reasons why they might consider augmentation – since there’s still no proof that this will slow down their loss of lung

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function. I believe there is no harm in augmentation, other than the economic harm. The cost of infusions is high.”

WEWERS ON TODAY’S ALPHA-1 CLINICAL RESEARCH • On inhaled therapy: “My bias is, I think it’s stepping out a little too far. If you give an IV of the protein, as we do today, it raises both the protein levels in the lung alveoli and in the blood. If you give it by inhalation, it doesn’t raise blood levels. We see most of the lung destruction in Alphas as basilar emphysema – damage in the bottom of the lungs. The lung bases get the best blood flow, due to gravitational flow. I can imagine ultimately finding out that the alpha-1 protein in the blood is necessary to protect the lungs.

“Aerosol therapy is less invasive and you probably need a lot less protein to raise the level in the lungs. But I think in order to prescribe it for my patients, I’d want to see a study showing it works – by actually slowing the loss of lung tissue.”

(The FDA has made it clear that such studies will be required for approval of inhaled Alpha-1 therapy in the United States.)

• On unsung heroes of early Alpha-1 research: “Jim Gadek, my predecessor at the NIH, was a real pioneer in Alpha-1. He was one of the first to propose the idea of augmentation therapy. Everything we’ve accomplished since wouldn’t have happened without his work.”

Gadek published the first augmentation study ever, “Replacement Therapy of Alpha-1 Antitrypsin Deficiency,” in 1981. He used a crude early version of augmentation that raised blood levels of Alpha-1 to 35 percent of normal in ZZ Alphas, showing “the feasibility of this approach” to Alpha-1 treatment.

“Ron Crystal was the force behind all this.” Crystal, then chief of the Pulmonary Branch, National Heart, Lung and Blood Institute of the NIH, was a champion of research on augmentation for 1.877.2.CURE.A1 (228.7321)

Wewers in his office at Ohio State.

Alpha-1. He was the senior author of both Wewers’ 1987 study and Gadek’s in 1981.

• On monthly infusions: A 1988 study of 28-day infusions for Alpha-1 at a rate of 250 mg/kl, by Richard Hubbard and Crystal at the NHLBI, found that “monthly administration is… a rational alternative to weekly therapy.”

Wewers says, “The goal is to imitate the body’s natural alpha-1 protein defenses as closely as practical.” He primarily recommends weekly infusions for his Alpha-1 patients who choose treatment, but only after careful consideration of the pros and cons. “I accept infusions every two weeks for some.

I don’t prescribe monthly infusions.”

• On Alpha-1, COPD and lung damage: “I don’t think we understand the whole story yet. I think we’re quite naïve about the whole process whereby alpha-1 deficiency causes lung damage and disease. There is still a lot to know www.alpha-1foundation.org

about Alpha-1 and its role in lung host defense.

I’d like to see more study of how the lung’s natural defenses against infectious challenge, the innate immune response, contribute to lung destruction, particularly in Alpha-1 subjects.

There is great potential to use modern technology to find new therapies.” _____________ Today, Wewers is not actively involved in Alpha-1 research. He has asked his residents (young doctors doing postgraduate work) at Ohio State University College of Medicine to research the early NIH study of Alphas done from 1989-1996. He believes that a modern review of that study could still find much valuable information.

“That early study showed that quitting smoking slowed the rate of loss of lung function in Alphas more than anything else. It was incredibly dramatic, much more than anything else, including augmentation treatment.”

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SPOTLIGHT 6

Inhaled Alpha-1 Inhaled therapy in Europe study shows good safety and tolerability in trial’s first year n February, Israeli biopharmaceutical company Kamada announced the interim report of its Phase II and III trial of an inhaled form of augmentation therapy for Alphas.

“The report, which includes information on 13 months of treating dozens of patients, shows an excellent safety and tolerability profile,” Kamada said.

The Phase II-III trial is the most advanced stage a company has ever reached with inhaled augmentation therapy.

The trial is designed to investigate both the safety and effectiveness Photo courtesy of Pari Pharma of alpha-1 protein taken by a nebulizer.

Several sites in Europe are participating, including Germany, the United Kingdom, the Netherlands, Denmark and Ireland.

The European Medicines Agency (EMEA), the European equivalent of the US Food and Drug Administration (FDA), has reviewed the trial.

Phase III of a clinical trial typically takes several years to complete and longer to receive approval from the EMEA or FDA.

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Alpha-1 protein: New formulations, and new therapies to be discussed Leading scientists from the United States and Europe will meet in June to talk about recent research on using alpha-1 protein to treat rejection of organ transplants, diabetes, cystic fibrosis, COPD and other diseases.

A number of studies have raised the possibility that the same protein now used in Alpha-1 augmentation therapy may prove useful in many other medical treatments.

Such new uses for the protein could eventually put a severe strain on supplies. All current products are intravenous treatments, produced by purifying human blood plasma from healthy donors.

For that reason, another major topic will be new formulations of Alpha-1 therapy. This includes inhaled therapy and possible recombinant (genetically engineered) forms.

The event, in Bethesda, MD, June 24, is the 12th Gordon L. Snider Critical Issues Workshop. Chairs are Mark Brantly, MD, and Basil Golding, PhD. The scientific committee consists of Adam Wanner, MD, Robert A. Sandhaus, MD, PhD, and Brantly.

It is sponsored by the Alpha-1 Foundation, Baxter Healthcare, CSL Behring, and the Talecris Center for Science and Education.

For information, contact Randel Plant at rplant@alpha-1foundation.org or 305-567-9888, Ext. 252.

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The study that led to an Alpha-1 community rom 1989 to 1996, a total of 1,129 Alphas were enrolled in a National Heart, Lung and Blood Institute (NHLBI) study of Alpha-1.

Mark Brantly was a member of the steering committee for the study, ran the central phenotyping and was also a principal investigator who saw more than 120 Alphas. (Many of those Alphas called themselves “the Brantly Bunch.” He called them “the Brantly Brats.”)

Recently he talked about the NHLBI study and its lasting effects on the Alpha-1 community and many physicians, including Brantly himself.

It was partly a longitudinal (conducted over a long period) study of the natural history of Alpha-1. It was also a Phase IV study, reMark Branley quired by the Food and Drug Administration for the approval of Prolastin, the first augmentation therapy. It compared treated and untreated Alphas.

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THE LESSONS AND IMPACT What was valuable about the study?

“We got the first evidence that augmentation might be efficacious. It was not proven, but it was pretty good evidence,” says Brantly. “We also got the first glimpse of the frequency of rare alleles [that cause severe Alpha-1 deficiency].”

Brantly says “one of the most lasting and significant achievements” is that the study established a group of doctors who were interested in treating Alphas.

“It was the first time we doctors interested in Alpha-1 met, shared our passion, and became a www.alpha-1foundation.org

group that’s largely intact today. All the major Alpha-1 centers in the United States can be traced back to their involvement in the study.”

Just as important, says Brantly, “It also tied together patients. Newsletters and support groups evolved from the study, and many of today’s Alpha-1 community leaders, including John Walsh. We had patients and Alpha-1 experts come together to help solve problems. In those respects, it had a huge impact.”

THE BEST OF BOTH WORLDS The study had an obvious and lasting impact on Brantly’s career. He is now a professor of medicine and chief of Molecular Genetics and Microbiology at the University of Florida Department of Medicine, where he runs the Alpha-1 lab.

He still sees “plenty of Alphas from the NHLBI study. I either follow them in my clinic, or I see or hear from them all the time. I have one who was a 17-year-old boy when the study began, and he’s in his 40s now, with a family.”

His original intent in life was to be a family practitioner. “But in my heart, I’m a scientist. What the Alpha-1 community has made possible is marrying those two passions. I have this family practice of Alphas, in addition to studying what I think are important scientific questions.”

WANTED THE FIRST HEROES OF ALPHA-1

were you in the first Alpha-1 Registry?

From 1989-1996, the NIH Registry followed 1,129 Alphas.

Many called themselves “The Brantly Bunch,” after mark brantly, md, the doctor who worked closely with them.

These Alphas made augmentation therapy possible. No one knows how many survive today. the alpha-1 Foundation is exploring the possibility of an “Alpha-1 Pioneer reunion” in late 2011.

If you were in the nih registry, and are interested in a reunion, call Angela McBride at 1- 888 - 825 -7421, Ext. 233, or amcbride@alpha-1foundation.org

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FRONTIERS

The

many kinds of COPD

COPDGene study hopes to find out why we have so many differences in the disease labeled ‘COPD’ wo years ago, we wrote about a new COPDGene study.

The COPDGene project is funded by a $37 million grant from the National Heart, Lung and Blood Institute. One of the largest COPD research studies ever done, it involves more than 20 medical centers around the country.

The study leaders hope to identify the different genes involved in COPD and find new, more effective therapies. Alpha-1 is the most important known genetic factor in COPD, and COPD is usually the first diagnosis given to Alphas with lung disease.

“COPD is largely not understood,” James Crapo, MD, of National Jewish Health in Denver, a coprincipal investigator for the study, said at the time.

“It’s made up of a bunch of different diseases which express themselves differently in a patient. Treating it as one entity doesn’t make sense. You have to separate it into different groups before you can find specific treatments.”

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THE MANY DIFFERENCES IN COPD What is the most notable finding of COPDGene so far?

How many differences there are in people with COPD. “We knew that all COPD subjects were not the same, but the great variability in the disease has been 8

remarkable. This has come out in some interesting ways. COPDGene has identified two groups – individuals with interstitial lung abnormalities, and people with unclassified spirometry patterns – that had not been widely studied before,” says Edwin Silverman, MD, PhD, of Harvard Medical School and Brigham and Women's Hospital in Boston.

What’s an unclassified spirometry pattern? “We currently diagnose and grade the severity of COPD based on spirometry. Most people fall into the categories of “normal” or “COPD” based on these tests, but a substantial percentage (likely about 10%) don't fit into either category. We have been studying those previously ignored individuals,” says Silverman.

Silverman is the other co-principal investigator for COPDGene. He is also a member of the Alpha-1 Foundation board.

The COPDGene project recently hit its national recruitment goal of 10,000 participants.

Now Silverman hopes to move on to working fulltime to analyze the huge amount of data the study has collected. By examining the genetic background of study participants, researchers hope to understand why some people get COPD. Most of the 12 million identified Americans with COPD are or have been smokers – but only a minority of smokers gets COPD. “We'll be looking to find determinants that relate to why some people get COPD and others don't,” said Silverman. Researchers also want to know “why some people get emphysema and others don't; and why some people with the same severity of COPD get frequent exacerbations and others don't.”

KEY QUESTIONS ABOUT ALPHAS The study also may help explain why some Alphas develop more severe emphysema than others, and why Alphas with similar lung capacity can have very different ability to exercise. Silverman and Crapo will 1.877.2.CURE.A1 (228.7321)

Ed Silverman, MD, PhD

James Crapo, MD

be presenting some of these findings at the upcoming conference of the American Thoracic Society in May. “It's also going to be interesting to see how many previously undiagnosed Alphas are within the COPDGene population,” said Silverman. “People with known Alpha-1 were asked not to participate in the study. But this is a way to estimate how many undiagnosed Alphas are in the COPD population… Not just how many, but what their characteristics are.”

Since everyone in the study gets a CT scan of their lungs, the project has taken steps to improve the accuracy and efficiency of CT scans.

The COPDGene project held an imaging workshop in February 2010, with the Alpha-1 Foundation and COPD Foundation among the co-sponsors. Radiologists and pulmonologists at the workshop reviewed CT scans from the study. They analyzed

them to come up with what they hope will be more standardized analysis of imaging for COPD. “CT scans give us a unique opportunity to see what's going on almost at an anatomical level in someone's lungs,” said Silverman. But a major problem lies in how different each scanner is, he said. Different brands and models of CT scanners produce different results. This presents challenges for coming up with standardized measurements of lung disease.

Silverman hopes that in the future, as a result of the imaging workshop, the analysis of CT scans will be more uniform across the board. This will allow doctors to better understand a patient’s COPD and treat it more effectively.

The Alpha-1 Foundation helped in recruiting for COPDGene and paid for the Alpha-1 phenotyping at Silverman’s study site.

www.alpha-1foundation.org

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IN YOUR INTEREST 10

Working with FDA to accelerate new Alpha-1 therapies The Alpha-1 Foundation has always worked closely with the US Food and Drug Administration’s (FDA) Center for Biologics Evaluation and Research (CBER) and Center for Drug Evaluation and Research (CDER).

From about 1998 to 2003, the Foundation had frequent meetings with the FDA under the auspices of a “liaison group.” These meetings were attended by FDA representatives to discuss overcoming obstacles to the development of additional Alpha-1 augmentation Atinthe is study,products. published the time, there were periodic New England Journal of shortages Prolastin, Medicine inof1987, used the only augmentation prodconcentrated and purified uct then available.

By esalpha-1 protein from the tablishing theplasma liaisonof pooled blood group, the Foundation alhealthy human volunteers. lowed an open line of Miriam O’Day Made by Cutter Labs, this communication between product later became Prolastin. Launched in 1988, all stakeholders the FDA; therapy scientists; Prolastin was theincluding first augmentation ap-clinicians; consumers and industry. proved by the Food and Drug Administration (FDA).

The achieve approval of ac“Themeetings 21 Alphashelped in thistostudy wereFDA really pioneers, the augmentation products Zemaira and Aralast, cepting what could have turned out to be substantial which to market infusions,” in 2003. The comparative risks forcame augmentation Wewers says. trials that led to both new drugs were made possiTheby pivotal study found that augmentation ble the biological division of Bayer, then had the demonstrated “biochemical efficacy,” whichProlastin the manufacturer of Prolastin, which provided FDA for theaccepted trials. as a rationale for approving the drug in December, 1987.

The Foundation plans to return to regular FDA liaiWewers’ articleAalso saidgoal it would take an son meetings. major is helping to estimated develop 300 to 500 Alphas in both arms of a doublebetter, more reasonable and effective design of cliniblinded for three years to showSuch clearimproved levels of cal trialsstudy of potential new therapies. clinical effectiveness for augmentation therapy. trials could accelerate the development of these therapies, inhaled Alpha-1 therapy.

The “I still findincluding it a difficult discussion with my Alpha-1 Foundation alsowe would like tothe discuss some conpatients, when talk about reasons why they cerns requirements for Phase IVthere’s trials ofstill cur-no mightabout consider augmentation – since rent proofaugmentation that this will products. slow down their loss of lung function. I believe there is no harm in augmenta-

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HELP CHANGE THE RULES Currently, the Foundation is working on influencing the rule writing for the Affordable Care Act (often called Healthcare Reform), to make sure our Alpha-1 community continues to receive valuable protections as the rule writing is done to implement the law. Provisions of the new law are rolling out steadily, with many already effective and more coming into effect this year.

The government website on the Act now carries a time line of changes in health care and insurance coverage provided in the law.

Some notable changes in the law include a 50 percent discount on brand-name drugs for those in the Medicare “doughnut hole,” expanded coverage for young adults, small business tax credits for providing health care coverage, and insurance plans for those with pre-existing conditions.

Many more changes are coming through 2014, and the time line is easy to read and understand. (See it here:http://www.healthcare.gov/law/timeline) HELPFUL NOTES The “Improving Access to Clinical Trials Act” (IACT) went into effect April 3.

This new law allows those with Alpha-1 or other rare diseases who receive Supplemental Security Income (SSI) to accept up to $2,000 in compensation for participating in a clinical drug trial without losing their federal medical benefits.

The Foundation joined many organizations representing those with rare diseases in lobbying for the new law, which passed Congress and was signed into law in 2010. _____________ The Centers for Medicare & Medicaid Services (CMS) has improved the Medicare Physician Directory tool with new information about physicians and other healthcare workers in their communities and the services those professionals provide.

The new feature is called Physician Compare.

The site, which was required by the Affordable Care Act, contains information about physicians enrolled in the Medicare program, which include doctors and other types of health professionals who routinely care for Medicare beneficiaries, including nurse practitioners, clinical psychologists, registered dietitians, physical therapists, physician assistants, and occupational therapists.

See Physician Compare site: http://tinyurl.com/drsearch 1.877.2.CURE.A1 (228.7321)

Speakup, the EasyWay Action Center lets anyone tell Congress what Alphas need now n 2008 COPD became the third leading cause of death in the United States, yet our top public health agency has done little about it. In Washington, DC, and in state capitals across the country, those that you elect make policies that should be in your best interest – but most know very little about Alpha-1, COPD and the unique needs of the community.

The Alpha-1 Foundation, the COPD Foundation, and the Alpha-1 Association are working together to change this, but we need your help. The only way to guarantee that your elected officials know about Alpha-1, COPD and your needs is to tell them yourself!

For those who have yet to visit the COPD Foundation’s Action Center, there is no better time than now to pay a visit and get involved.

The Action Center makes it easy to be an advocate – no matter how technically savvy you are or how much you know about politics. It is more than just an informational tool; it’s a one-stop shop for becoming an advocate at home.

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www.alpha-1foundation.org

Take a first, easy step to becoming an Alpha-1 advocate by signing up for the Action Network.

When there is a critical issue, you will know about it. All you need to provide then is your address and the Action Center will display your elected officials at the state and federal levels.

You can introduce yourself by using the “email your governor, house representatives and your senator” options on the home page. Tell them you’re a constituent; explain Alpha-1; and tell them you are relying on them to make sure the needs of the Alpha-1 and COPD communities are met.

After that, if you wish, you can do a few simple things: • Check out the action alerts and send a pre-written letter asking for action on a specific issue like “Joining the COPD Congressional Caucus” or supporting the “Medicare Respiratory Therapy Initiative.” We give you the information; you come armed with your zip code and your passion and it becomes easy to express your feelings and opinions.

• You can also amplify your voice by alerting your friends that you need their help using the “tell a friend” feature, post on your Facebook page, and tweet about it to make sure others follow your lead.

Some want to do even more. The Action Center can help with that.

See “Capitol Hill Basics,” tips on meetings, phone conversations, and letter writing. You do NOT need to go to Washington to be an advocate. All 435 members of the House of Representatives have offices near the home towns of people they represent; the Action Center will help you find them and set up a meeting.

Imagine the effect, if everyone with Alpha-1, COPD, and their friends, family and healthcare providers sent just one message!

Visit the Action Center at www.operation435.org and create change that improves the lives of everyone affected by Alpha-1 and COPD for generations to come.

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One place.

All things Alpha.

CareZ is the place to go to find all of the support you want to meet your specific needs.

With a comprehensive array of Alpha-1 support services, we focus on the whole you and provide a customizable community of care built around your Alpha-1 and your life.

To find out more, contact the CareZ community at:

1-888-415-2167 Monday-Friday, 8:00 AM to 5:00 PM CT

Please see Brief Summary of full Prescribing Information on following page. Š2010 CSL Behring LLC 1020 First Avenue, Avenue, PO Box 61501, King of Prussia, P PA A 19406-0901 USA www.CSLBehring-us.com www.CSLBehring-us.com 09-ZMR-041 6/2010

Your Y our home for Alpha-1 help

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.

END OF LIFE 14

How to talk about End of Life When someone you love is dying, here's how to avoid a lot of stress, fear and family battles ane Martin is a respiratory therapist and author who has been teaching classes on advance directives and end of life issues for many years. Here is her advice to Alphas, their families, and just about everyone else.

I suggest that family members approach the topic like this: “Mom, I know it’s not fun to think about this, but if you’re ever unable to speak for yourself regarding what you want for health care, it’s important that your wishes be carried out. Let’s talk about what you want, not what I want or my brother wants, your lawyer wants – or anybody else. Just what you want. When you decide, write it down so everybody knows, then we can just forget about it, never need to think about it again.”

I tell patients much the same. It’s basically the same thing that I’ve been telling them in many years of teaching an advance directives class to people with severe lung disease.

It’s about choice and dignity.

Choice – deciding what you want for yourself. Not leaving it up to others to decide, guess or speculate, what you would have wanted or what they think is best for you.

And dignity – not losing control of everything that happens to you at a very important time in your life, when you are near death. You don’t want a doc-

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tor, or your most aggressive family member, your lawyer or a judge deciding for you.

I tell patients that even the closest, most loving families can have intense emotional battles when they talk about decisions that must be made when a parent is clearly near death. I see it all the time. So I always say, “You must have Jane Martin an advocate who will agree to follow your wishes, even if they are very different from the wishes of the advocate. You might have a son who says, ‘No, Mom, I could never pull the plug on you. I couldn’t live with myself if I did that.’

He’d never sign the paperwork; he’d never be willing to do what you want. That’s OK; the son’s feelings must be accepted and honored. But you must get a different person to be your advocate who can, in good conscience, do what you wish.”

BE SURE TO TALK TO YOUR DOCTOR And talk to your doctor about it! Bring it up at your next appointment, or make a special appointment to discuss it. Doctors might have no idea what their patients want when end of life decisions are being made.

Everybody has their own idea of what living is. And this is your chance to make your own choices about what you consider life to be, and have everybody respect that. No matter how sick you become, you’ll still have control over your own life.

When someone you love is dying, think about care and comfort. Think about that, and it covers a lot.

The subject of morphine is huge. Doctors and nurses are nervous because morphine can suppress breathing. But so what? If the patient is dying, what are we trying to accomplish? If the patient is 1.877.2.CURE.A1 (228.7321)

near death, morphine, painkillers, comfort is what he or she needs. That’s why getting all these things written down when the patient is lucid and comfortable is so important.

Every adult, even those who are young and healthy, should have a living will. It makes your wishes known about life-prolonging medical treatments.

Among other documents I always recommend highly are an Advance Directive and a Durable Power of Attorney for Health Care – this names your Patient Advocate, the person or persons legally empowered to speak for you when you are no longer able to speak for yourself.

This is a terrible time for families. It’s always a very sad time. Why add to that by letting these decisions become so stressful? When everything is decided in advance, a family can grieve together and comfort each other without the unnecessary stress and even emotional battles.

Jane Martin tells more about end of life issue in her blogs like this one on Health Central: http://tinyurl.com/endcopd

End of life: What to do, where to find the documents you need Here is a “To Do List” suggested by Tom Petty, MD: • Discuss your hopes, fears, and anxieties about end-of-life issues openly and honestly with your loved ones.

• Prepare legal documents such as a living will, advance resuscitation directive, and durable medical power of attorney.

• Share your spiritual beliefs with your doctor and healthcare professionals. Surveys show that up to 80 percent of all patients employ prayer to help them reach medical decisions.

DOCUMENTS, GUIDANCE You may want to talk to a lawyer about preparing documents concerning your wishes at the end of your life, especially if you already have a personal attorney.

Here are some places to find essential legal documents such as a living will and durable medical power of attorney, recommended by Petty and other experts. The best are written in language intended to be clear to average people. Be sure the documents meet the specific legal requirements in your state. Many are offered free.

Caring Connections offers a wide variety of free documents and downloadable brochures. They include a legal guide for the seriously ill and advance directives that are specific to your state. http://www.caringinfo.org/i4a/pages/index.cfm?pageid=1 The Center for Practical Bioethics offers free downloadable information on end of life, including helpful guides to families on how to talk about it.

Their website is at http://www.practicalbioethics.org/cpb.aspx?pgID=886 Full Circle of Care, a program of the Triangle J Area Agency on Aging of North Carolina, has extensive information for caregivers, families and patients. Their website: http://www.fullcirclecare.org/index.shtml Nolo, a 40-year-old company whose motto is “law for all,” offers free and low-cost legal forms and information, as well as a lawyer directory at www.nolo.com.

www.alpha-1foundation.org

15

END OF LIFE 16

A gentle death “For Marta it was all about quality of life. In all the 10 years we were together, being able to enjoy life was the big thing for her.” arta Strock was an energetic AlphaNet coordinator in Sarasota, FL, when she was diagnosed with breast cancer in June, 2001. She had a mastectomy.

Chemotherapy was very hard on her. She had constant fatigue and flu-like symptoms; she lost all her hair, lost breathing capacity, went on fulltime oxygen. But she recovered and returned to work.

She was cancer-free for over six years.

Then the pain came, and then the news in December, 2007: the cancer was back.

Sue Fair, her life partner, tells the story.

Marta got a second opinion at Moffitt Cancer Center. They recommended even more aggressive treatment than she’d been offered by her Sarasota oncologist. The side effects could be very severe.

She turned it down.

“I don’t want to spend the rest of my life feeling sick and in bed all the time,” she said.

She didn’t want to be too sick to watch a polo game – she loved polo – or go out to eat, or enjoy the company of friends. A neighbor gave her a batterypowered chair. She loved to tool around the neighborhood and I could take her to the flea market or the mall, places she couldn’t go without the chair.

She was even able to kayak and go horseback riding, her little Helios oxygen bottle strapped to her.

She began to give people things, like pieces of her jewelry, that she wanted them to have.

She took chemo tablets. Her oncologist monitored

M

her carefully with X-rays and CAT scans. The tumor didn’t grow, but her cancer markers kept rising. She was reluctant to go on IV chemo because it had been so toxic for her.

After a year on tablets, her markers began to soar, so she switched to IVs. The markers kept going up.

She came back from her oncologist’s one day, sat down to have a cocktail with me, and said, “Sue, you know I’m not long for this world.”

I said, “Yes, I know. And whatever decision you make about treatment, or no treatment, you know I’ll support it.”

We’d always had these frank conversations. Every January, we updated our health record and choices in a folder. It had our wills, Living Wills, health surrogates, Do Not Resuscitate orders, our prescriptions, and the names of friends and family to contact in an emergency.

Now when I took Marta to the hospital, I just grabbed the folder and took it with us.

In late April, she was hospitalized to have fluid drained from around her lungs. When she came home, she was sitting outside, just relaxing in the sun, drinking a Warsteiner, her favorite beer.

Dr. Diener arrived. Howard Diener had been treating her for COPD for many years. They were friends. He came to tell her that the fluid taken from around her lungs contained a large number of circulating cancer cells. She asked him, “Does this mean my time is getting limited?” And he said, “Yes.” He suggested she register with hospice, and we did.

Marta told me, “I spend 85 percent of my life in treatment so I can live the other 15 percent. I don’t want to do this anymore.” She stopped her chemo and Alpha-1 infusions.

Thanks to hospice, a registered nurse came in once a week to organize her medications; a nurse’s aide came in every day; a personal groomer came in twice a week to help her shower and wash her hair.

We signed all the hospice paperwork on a Monday.

The following Sunday, Marta had the emergency that made her decide to go into hospice for the first time. It was cut very close. I tell everyone, you never expect these things to happen as fast as they do.

That morning, while she was alone, Marta just couldn’t get enough oxygen. I came home and called 911. The paramedics couldn’t raise her oxygen saturation to safe levels. When she was stabilized in a hospital emergency room, she chose to enter the hospice for the first time.

1.877.2.CURE.A1 (228.7321)

Marta Strock

Sue Fair

She did well there. One evening she had a gathering of friends in her hospice room. She had someone sneak in a bottle of Warsteiner. Shortly after that, she was able to go home with 24-hour nursing care.

I had promised that when her time came, she wouldn’t die alone, or in pain, and she wouldn’t die gasping for breath the way her brother did.

[Marta’s brother Randy, also an Alpha, had died after two lung transplants.]

For Marta it was all about quality of life. In all the 10 years we were together, being able to enjoy life was the big thing for her. After she was cancer-free for five years, she had high hopes of a lung transplant. She planned to see Dr. Rolfe at the Tampa General Hospital transplant program. When the cancer came back, it just shattered her world. She knew the transplant would never happen.

But she went on with life, enjoyed it, and was always hopeful. Even after her first visit to hospice, she still hoped she could get strong enough to see her Alpha friends at the national conference in June.

The nurses were impressed with how well Marta did at home. She could get up, eat at the table, do most normal things. But after a few days she got

badly confused and disoriented, and had to go back to the hospice room.

Then she reached another key point. She was always anxious and uncontrollably restless – they call it terminal restlessness. Besides morphine, hospice wanted her on Thorazine. That was end-of-life sedation for her, and they wouldn’t give any more nourishment. I was very upset; I felt they were starving her. But the hospice doctor and Dr. Diener made it clear to me that withholding nourishment at that point is much kinder.

She died June 2. I soon gave away her Helios to an Alpha, her power chair to another Alpha. It was six months before I could go into her closet, give away her clothes and change her bedroom.

I released Marta’s ashes into the Gulf of Mexico in October. It was a beautiful, peaceful, sunny day.

I chartered two boats and took out 10 other people.

I spoke a few words and dedicated a poem to her.

Once I was out on the water, especially after I released her ashes, I felt a tremendous sense of peace.

Then we all went to the Salty Dog to offer up a few toasts and share some stories of the life and times of Marta Strock. I still miss her so much.

www.alpha-1foundation.org

17

END OF LIFE 18

TomPetty’s advice on end of life Suffering can almost always be avoided, with the doctor’s help om Petty invented pulmonary medicine as we know it today,” says Bernard Levine, MD, an Arizona pulmonologist.

“When he started… in the early 1960s… COPD was a disorder that nobody wanted to talk about or treat.”

Petty, who died in 2009, had COPD himself in later life. He was a pioneer in the use of medical oxygen. This article is condensed from COPD Digest.

Most patients with advanced COPD die of this common respiratory disease.

They're burdened by shortness of breath, chronic cough and mucus production, diminished activity, anxiety, depression, and despair. Many have asked me, their physician, about endof-life matters.

Tom Petty I can empathize with these dying patients. I have faced death myself on four occasions after urgent open-heart surgery. I share my deep feelings with them. All physicians should do so when facing any dying patient. I've discussed end-of-life issues with patients and their families clearly, emphasizing that all life inevitably ends by design. I also stress that medical techniques can both postpone death and allow patients and their families to make it pleasant and meaningful.

Most COPD patients do not particularly fear death.

“T

Accepting it as fact, they receive comfort and assistance from oxygen and other prescribed medications. Patients worry most about how they will die.

They imagine themselves suffocating in anguish and delirium. This often panics them. Most do not want to spend the rest of their lives on a mechanical ventilator or have their death extended by it.

All patients – and their loved ones – must understand completely that suffering is rare and can almost always be avoided. As COPD progresses, carbon dioxide builds up as a normal consequence of impaired breathing. In fact, carbon dioxide retention has a calming, narcotizing effect.

TWO BIG MISTAKES In some cases, doctors try to reduce elevated carbon dioxide levels by mechanical maneuvers or by stimulating the patient's breathing with drugs. This is a big mistake. The kidneys compensate for the acid caused by carbon dioxide build-up, keeping acid levels in the normal range well into extreme limits of life.

Nor should doctors limit sedatives, tranquilizers, and painkillers for fear of suppressing respiration.

This is another common mistake. Many doctors and nurses feel that these drugs will suppress the drive to breathe, and death will occur as a result. In fact, an excessive drive to breathe exists in most patients.

This increases the work of breathing and creates respiratory distress. Thus, trying to keep carbon dioxide down to normal levels actually torments the patient.

Rest and normal sleep usually accompanies the skillful use of pharmacologic agents. Enjoyable food, ample fluids, and pleasant drink (including wine or substitutes) are perfectly appropriate.

Most patients can willingly face their impending death in the comfort of their home with their families, sometimes for months or even years. Hospice services help. They focus on comfort care, which should be the goal for all who serve these patients.

Institutional hospices serve mostly patients near death. Many patients deemed appropriate for hospice care live far beyond the previously limited sixmonth length of stay, just because they learn to adapt to their disease. One of my hospice patients even drove his car for two years, accompanied by his hospice worker. He was happy to participate in some pleasurable activity.

In COPD – as in other illnesses – death is not the enemy. The true enemies are fear of abandonment, loneliness, and pain and the anxiety that accompanies such fear. Proper medical care and loving attention can keep those enemies at bay.

1.877.2.CURE.A1 (228.7321)

The Truetts are going to train with the dog that will change the family’s life for the better witness a miracle every day,” says Desiree Truett.

The Arizona Alpha needed $25,000 to pay for all the costs of a specially-trained dog for her adopted son, Justin. His “silent seizures” can be life-threatening. The whole town of Maricopa has gotten behind Desiree and her husband Tim in the effort; a town carnival raised $13,000.

That’s where the miracles began.

The first one came after she and Tim appeared on local television. On the show, Tim wore a shirt with the emblem of the major league baseball team Arizona Diamondbacks. The Diamondbacks president saw the show and donated $5,000 – and seven season passes.

A family from the area saw the TV spot and paid for the Truetts’ hotel and rental car expenses. They got donations of $10 and $20 from people who live on fixed incomes. American Airlines agreed to donate their travel expenses, and will allow the dog to ride in the cabin with them.

Getting ready for the dog is very complicated. The family made “tons of videos” of themselves, their home, and the schedules they follow and sent them to 4Paws for Ability in Ohio, the only organization in the country that trains service dogs for children with special needs. 4Paws trainers picked two or three dogs they thought might match the family.

Two years of training is required for the dog to do

“I

www.alpha-1foundation.org

Justin Truett

its job. Although the family has pictures of the dogs chosen, they will not be told which specific dog will be theirs until two weeks prior to going to Ohio.

This is to prevent families from falling for one dog, then finding out the dog wasn’t right for them.

In mid-September, Desiree, Tim, Justin, and Jamie will travel to Xenia, Ohio, for two weeks. They’ll meet the dog chosen for them and spend 10 days in intensive training, 10 hours a day. The family will be trained to give the same commands in the same way, so the dog won’t get confused.

Justin and Jamie will go because the dog must bond with the children. Justin, five, is the child to whom the dog must bond most strongly. For Jamie, eight and legally blind, this will also be a guide dog. She’ll be put on the list for her own dog.

Their dog is primarily trained in detecting Justin’s seizures. The dog will detect seizures and alert Desiree and Tim to deal with them. The dog is also trained to bring toys, turn on lights, and help in more general ways.

The Truetts’ dog had to be even more special. Since Desiree is allergic to dogs, she gets allergy shots.

Only a few mixed breeds of hypoallergenic dogs are available.

Getting this dog is much like having a baby. They don’t know who it will be, how it will turn out, or even what its name is. But Desiree says, it’s “the best thing ever.”

ALPHA LIFE

dog for Justin and Jamie A

19

ALPHA 411

In memory:

Alpha leaders Ed Brailey, Dennis Pollock d Brailey and Dennis Pollock, both lung transplant recipients who became nationally known Alpha-1 community leaders, both died recently.

Brailey, 58, died Jan. 3, 2011.

After his lung transplant in May 2002, he became involved almost immediately in fundraising with “Team Brailey,” leading a team of firefighters, police officers and friends to many “Escape to the Cape” bike rides on Cape Cod, MA. In Brailey's honor, Team Brailey will lead Team Alpha-1 at the 2011 “Escape to the Cape” Bike Trek this fall, said Fred Walsh, longtime member of Team Ed Brailey Alpha-1 and participant at the annual bike trek. “Team Alpha-1 will be calling itself the Brailey Brigade this year in Ed's honor,” he said.

Brailey and his wife JoAnne led a Connecticut Alpha-1 support group for nearly 10 years. He was a frequent advocate in Washington for Alphas and others with rare and chronic diseases. He was a member of the Alpha-1 Association Board for five years and became chair in 2010. The Braileys moved in 2010 to North Carolina.

E

DENNIS POLLOCK Dennis Pollock, who led an Alpha-1 support group in Oklahoma that played a key role in the passage of the Genetic Information Nondiscrimination Act (GINA) in 2008, died Oct. 4, 2010. He was 51.

Pollock, who received his transplant in August,

20

2004, had his wife Shelley, family and friends with him when he died.

“Dennis had an amazing ability to articulate the importance of understanding Alpha-1,” said Alpha1 Foundation president and CEO John Walsh. “We have GINA protections today largely thanks to his unwavering commitment.”

THE QUEST FOR GINA On one day’s notice in 2007, Pollock traveled to Washington, DC, from Oklahoma, learned quickly about the proposed GINA bill, and went out to lobby for it. He decided to visit the office of Oklahoma Sen. Tom Coburn, who had placed a hold on the bill, preventing it from coming to a vote on the Senate floor. An aide told him that Sen. Coburn would not release the hold. “I thought that was arrogant,” Pollock said. “I was so frustrated.”

He started calling his Oklahoma support group members on his cell phone. “I called about 10 people. I asked them to call the senator’s office, and to ask 10 of their friends to do the same. Within 45 minutes, I got a call from Sen. Coburn’s office. They asked me to tell the Alphas to stop calling. They were getting so many calls, they couldn’t get anything done.

I said, ‘Will you release the hold on the GINA bill?’

They said no. I said, ‘Then I won’t stop the calls.’”

The calls and emails continDennis Pollock ued for months. Finally, Coburn released the bill and the Senate passed it 95-0. When President Bush signed GINA into law on May 21, 2008, Pollock was there, representing the Alpha-1 community.

The Genetic Alliance will honor Pollock with its Art of Advocacy Award June 25.

Pollock was a member of the board of the Alpha-1 Association. He was one of the Association's first Peer Guides and was a member of the Support Group Leader Advisory Council. With his support group, he ran the Alpha Okies Golf Classic every year as a fundraiser for the Alpha-1 Foundation's research programs. Pollock believed that organizing the golf tournament changed him personally, and changed his support group. “The golf tournament brought us together. It feels a lot like a family.”

1.877.2.CURE.A1 (228.7321)

Time to celebrate 20 years of national Alpha-1 conferences his year’s National Education Conference will mark the 20th anniversary of the Alpha-1 Association! In 1991, a group of Alphas in Minnesota created the Alpha-1 Association to stay in contact and keep other Alphas informed about research and treatment.

The first national conference was held in 1992, with an expected attendance of 75.

To the shock and delight of the organizers, close to 150 people came. Nineteen years later, attendance has grown to more than 600.

For those who are looking to open new doors or find new ways to get involved with the Alpha-1 community, and those curious to learn the latest on research and treatment from the leading Alpha-1 doctors and healthcare professionals, Saint Paul, MN is the place to be on June 10-12.

The conference honors the traditions set by the first conference by blending a family reunion atmosphere with educational topics on vital Alpha-1 issues. Some of this year’s speakers include local Minnesota physicians Michael J. Krowka, MD, Marshall Hertz, MD, and Julie Heimbach, MD; as well as Charlie Strange, MD, Jeffrey Teckman, MD, Michael Campos, MD and Robert Sandhaus, MD. (Sandhaus has attended all 20 national conferences.)

For Alphas with children, there will be a supervised indoor children's program at the hotel with games, crafts, and movies sponsored by Alpha-1 Kids, with separate activities for teenagers.

We’ve also scheduled activities for before and after the conference: Thursday, June 9, you can go on the Twin Cities Highlights Tour. From 11 am – 3 pm, a guide will provide commentary on the sights of Minneapolis, Saint Paul, and the area. Monday, June 13, you can take a river boat tour of Stillwater – birthplace of Minnesota – from 9:30 am – 3:30 pm.

T

www.alpha-1foundation.org

The tour will include time to spend in this enchanting old river town.

Thanks to the generosity of CSL Behring and Talecris Biotherapeutics, the Alpha-1 Association has established an Oxygen Travel Fund that will provide oxygen and equipment for Alphas in financial need to travel to their physicians, hospitals and Alpha-1 educational events.

Travelling with supplemental oxygen can be daunting, challenging and expensive. This program will enable Alphas to travel with an appropriate portable oxygen concentrator, approved for carry-on to airline flights. Equipment will be provided either by their own home health oxygen provider or a national company specializing in travel oxygen. A staff member will assist Alphas in organizing their supplemental oxygen requirements.

To learn more about how to apply for support from this fund, email or contact Cathey Horsak, director of community programs, at chorsak@alpha1.org or (877) 346-3212. We must have a minimum of 30 days’ notice to arrange travel with supplemental oxygen under this program.

Looking for extra cash?

Want to work from home?

Have a computer, high-speed internet and a telephone?

FREE online job skills + assistance with job placement

It’s easy: go to “Job Seekers” at www.vtsystems.org to apply today!!

The COPD Foundation is a founding charity of Virtual Training Systems.

In order to qualify, you must be eligible to work in the US and be one or more of the following: disabled, veteran, caregiver, and/or a military spouse.

21

ALPHA 411

“ excited to be

Iwakeup ” living After two transplants, a “new, improved Celeste”

eleste Morris is an AlphaNet coordinator who loves travel and life in the city of Chicago. You would never guess this woman has had a lung transplant. In fact, she’s had not one, but two.

As is common, Morris visited three doctors who never mentioned or tested for Alpha-1. (They suggested she “get in shape” to treat her breathlessness.) It was a neighbor – not any of the three doctors – who suggested she see a pulmonologist at Loyola University Medical Center in Chicago. Then

C

she was properly diagnosed, 22 years ago. She received augmentation infusions for 13 years, until her first transplant.

In 2002, she received a double lung transplant at Loyola after her COPD grew severe due to her Alpha-1. Then in 2008, her ongoing organ rejection led to her second transplant, this time a single lung.

“Having two transplants has definitely been a journey, but one I would do again,” she says.

Morris attributes her success to the support her friends and family – she has two grown children – have given her, physically and emotionally.

She shares her own optimism and support as the Illinois AlCelesteMorris phaNet coordinator.

“I’m the ‘new and improved’ Celeste. My life has changed dramatically in the years following transplant. ‘Don’t sweat the small stuff’ are definitely words I live by. Every day is a blessing, so easy to navigate, I don’t think too much about the breathlessness I used to encounter with the smallest of tasks, such as talking and laughing,” says Morris.

“I wake up excited to be living instead of trying to exist.”

Calendar of Events 2011 May 7

4th Annual George Washington Bridge Walk

New York, NY

May 14

Alpha Okies Golf Classic

Oklahoma City, OK

May 26

“Get the Scoop on Alpha-1”

Denver, CO

June 4

Alpha Run: Mohawk Ramblers Motorcycle Club

MA

June 5

All Aboard for Alpha-1

Connecticut River

July 16

“Get the Scoop on Alpha-1”

Des Moines, IA

Aug 13

Let’s Cure Alpha-1 Walk

Fairmont, MN

Aug 13

Leadville Trail 100 Mile Bike Race

Orem, UT

Sept. 8

Greenwich Golf Event

Greenwich, CT

Sept. 30 – Oct. 2

Team Alpha-1 Escape to the Cape

Cape Cod MA

Joe Reidy joereidy@verizon.net Sherri Bowler Sherri.bowler@hobbylobby.com Judy Simon saidsimon@comcast.net Frank Lumbis fubarkiss@live.com Sandy Ringgard oakhamsix@yahoo.com Peg Iverson pegiver@mchsi.com Julie Liljenquist julie@norwexwithjulie.com DC Young dcytoquer@hi-speed.us Ken Irvine Airvine3@optonline.net Angela McBride amcbride@alpha-1foundation.org

For fundraising event information, contact Angela McBride at 888.825.7421 ext 233 or amcbride@alpha-1foundation.org.

22

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The effect 1 -proteinase inhibitor inhibitor (alpha (alpha11-PI) -PI) on on pulmonary pulmonary of therapy with any any alpha alpha11-proteinase -antitrypsin exacerbations and on the the progression progression of of emphysema emphysema ininalpha alpha1-antitrypsin 1 defi ciency has not been been demonstrated demonstrated ininrandomized, randomized, controlled controlled deficiency has not clinical trials.

PROLASTIN-C may may contain contain trace trace amounts amounts of of IgA. IgA. IgA Patients with patients known PROLASTIN-C deficient antibodies to IgA, which can be present in patients with selective or with antibodies against IgA should not receive PROLASTIN-C due to the risk severe IgA deficiency, have a greater risk of developing potentially severe of hypersensitivity. hypersensitivity and anaphylactic reactions. IgA deficient patients with antibodies against IgA notduring receiveclinical PROLASTIN-C to the risk The most common sideshould effects trials withdue PROLASTIN-C of hypersensitivity. were chills, a general feeling of being unwell, headache, rash, hot flush, and itching.

The most reactiontrials observed during clinical The most common sideserious effectsadverse during clinical with PROLASTIN-C PROLASTIN-C was an abdominal and extremity one studies with were chills, a general feeling of being unwell, headache, rash,rash hot in flush, subject. and itching.

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