November - December 2008

It wasn’t planned, but the theme of this month’s Alpha-1 Canada Community News is gifts. And given the time of year it somehow seems more than appropriate.

This newsletter will serve as a combined November and December issue.

Season’s Greetings from the President

Dear Alpha-1 Family and Friends:

The holiday season is fast approaching, such a poignant time of year; a time of hustle, anxiety, joy, sharing, reflection, thanksgiving, friendship, families, gatherings, resolutions and memories. As we gather in mind, body or spirit, know that we at Alpha-1 Canada join you across the miles in community and celebration. Know that we are hard at work for you building our Canadian Alpha-1 Family.

As we reflect on a very busy year we would like to extend our sincere gratitude and share a few special highlights with you:

• Jim Mundy, our Executive Director, a fabulous addition to our team – Thank you Jim for your many accomplishments thus far
• Creation of our new web site – Thank you Talecris and staff
• Our new monthly newsletter – thank you Jim
• Significant inroads into our “Awareness Campaign” – Thank you Talecris and GlaxoSmithKline
• Continued growth of our support group and support efforts – thank you Support Group Volunteers
• Sudbury Medical/Patient Education Series – thank you Northern Ontario School of Medicine
• Establishment of our Medical Advisory Board – thank you Doctors Kenneth Chapman, Jean Bourbeau,  Diane Cox and Simon Ling
• Forging international partnerships, new sponsors and friends – Thank you – without you I would hate to think where we could be
• New and existing members of the Board of Directors – thank you for all that you do

Resolutions and challenges give rise to many questions, answers, solutions and dollar boundaries with respect to testing, finding new alphas, standardization of orphaned drugs, improvements to our quality of life and much more. We look to you for your assistance. Building upon the wealth of individual and family information you hold, we can continue to provide support and education. We hope your New Year’s Resolution is to become active in your alpha-1 community.

Lest we forget our Alpha Angels, here is a toast to those who have gone before us, fought the battle, and left us behind to build new memories.

On behalf of the Board of Directors, our Medical Advisory Board, Jim and myself we extend our best wishes for a healthy and prosperous new year.

Warm Regards,

Mimi McPhedran
President and Chairperson

Sharon’s Extreme Medical Adventure

Sharon is taking a month off from writing about her transplant journey. She has moved back home now and is settling in to her new routine. Many of you have told us what a gift Sharon’s story has been to you. If you would like to return the favour and have us share your story with your community, call or write. We’d love to help you share your story. You can read more about her journey in the new year.

Read chapter one and two if you missed them.

Gifts of Information and Education

Physician and Patient Awareness

Alpha-1 Canada is about to launch the biggest awareness campaign in its history. Finishing touches are being applied to a program to mail physician brochures, posters and a supply of patient brochures to every family physician, respirologist, hepatologist and medical geneticist in Canada. English and French versions of all materials will be available.

GlaxoSmithKline and Talecris Biotherapeutics are contributing a combination of funding and services to create these materials, print, package and mail them. We are very thankful to these two companies not only for making this long planned project possible, but also for recognizing the importance of increasing awareness of alpha-1 antitrypsin deficiency in Canada.  We believe that this national exposure to the medical community will stimulate testing, diagnosis and early detection and treatment.

We will let you know when the mailing goes out so that the next time you visit your physician you can ask if they have received their package, ask for their comments and contact us with their feedback and yours. It’s always helpful in the long term to provide our funders with evaluation comments and success stories. Extra copies to distribute to your families will be available for the asking.

Parlez-vous français?

We have also begun the process of becoming a bilingual organization by undertaking a project to create a French version of our web site. Soon, when you go to www.alpha1canada.ca, the first page you see will ask whether you would like to visit our English or French web site.

The number of known diagnosed Alphas in Québec is very low and most experts agree that the reason is not that French Canadians are less likely to have Alpha-1, but rather that little information is available in French.

Thanks for making this possible go out to Talecris Biotherapeutics.

This Holiday Season exercise your heart!

The holidays will soon be here and many of us are trying to figure out what to give those hard to buy for people on our list. This year, why not give your loved ones a less materialistic gift by making a donation to Alpha-1 Canada in their name.

Alpha-1 Canada has a number of programs and services that can benefit from your support:

• the patient support hotline at 1-888-669-4583 is available to patients and their caregivers for information on both a regular and emergency basis
• the medical advisory board meets regularly providing the board of directors and staff with information on the latest advances in research and treatments
• the web site (www.alpha1canada.ca) is continually updated with the latest information
• the monthly newsletter keeps patients and their caregivers up-to-date on current events and offers stories about fellow Alphas
• in addition to the web site and newsletter, printed materials are distributed and are available to anyone, printing and postage are very expensive
• patient support is offered through a variety of methods including e-mail, the hotline and a number of support groups
• in 2009 we will be launching a new  “drop-in support group” that will meet twice a month by telephone conference offering you the ability to ask other Alphas how they deal with things you may also be dealing with and guest speakers when available, depending on participation this could become a very helpful, but expensive, resource
• through Alpha-1 Canada’s membership in the Canadian Organization for Rare Disorders (CORD) we keep informed about government activities related to healthcare and drug coverages and participate in supporting those initiatives that will benefit the Canadian Alpha-1 Community

These are just a few of the programs and services you can support and help others during this time of love and giving and receive a tax write-off in the process. We will send you a gift card for easy giving (click to see a full size version of the card)

Knowing their gift is a selfless contribution that made someone else’s life brighter can put the season in perspective for you and the person in whose name you make a donation, perhaps even your own.

Find out how you can give gifts that don’t need to be wrapped and provide information, education and support to people who could really use your help. Contact Alpha-1 Canada at 1-888-669-4583 or jim.mundy@alpha1canada.ca for more information.

Carlton University Students’ Association withdraws support for Cystic Fibrosis

In a move universally criticized, the Carleton University Students' Association (CUSA) has voted to drop a cystic fibrosis (CF) charity as the beneficiary of its annual Shinearama fundraiser, supporting a motion that argued the disease is not "inclusive" enough.

According to the Canadian Cystic Fibrosis Foundation, “Cystic fibrosis (CF) is the most common, fatal genetic disease affecting young Canadians. CF is a multi-organ disease affecting primarily the lungs and the digestive system.”

The resolution adopted unanimously by CUSA read, in part, that CF "has been recently revealed to only affect white people and primarily men"

If the motion were accurate, it would be difficult to disagree with people like the National Post’s Jonathan Kay who suggested it is racist. However, the resolution is simply wrong. Approximately 1 in 25 people of European descent is a carrier of the cystic fibrosis gene mutation. Although CF is less common in these groups, approximately 1 in 46 Hispanics, 1 in 65 Africans and 1 in 90 Asians carry at least one abnormal CFTR gene. Cystic fibrosis is diagnosed in males and females equally.

At Alpha-1 Canada, we know as well as anyone how difficult it is to raise funds for rare genetic diseases. When organizations such as CUSA don’t do their homework it makes it difficult for all of us.

The Alpha-1 Foundation Awards Two More Research Grants

The Alpha-1 Foundation in the US announced recently that it has awarded two new research grants for projects at the University of Florida and Brandeis University. The grants were made possible through an unrestricted charitable donation from Talecris Biotherapeutics to the Alpha-1 Foundation.

The first grant, given to Sihong Song, PhD, of the University of Florida, will enable him to develop a line of mice with alpha-1 antitrypsin deficiency. No mouse models currently exist for studying the functions of the alpha-1 antitrypsin (AAT) protein. Dr. Song will use genetic engineering techniques to replace the gene for alpha-1 protein in the mice being studied. The goal is to develop a line of transgenic mice which express the mutant “Z” alpha-1 protein – mice with alpha-1 antitrypsin deficiency (Alpha-1). The AAT-deficient mice will be studied for the development of lung and liver disease. “We hope that these AAT -deficient mouse models will reveal novel functions of AAT and be useful in evaluating treatments for AAT-related human diseases,” said Dr. Song.

The second grant, to Brandeis University in Waltham, Massachusetts, will be used to fund the work of Anne Gershenson, PhD, who plans to map the structure of AAT to aid in designing new treatments for Alpha-1. AAT, the alpha-1 protein, is mainly made in the liver. Normal AAT is a single protein called a monomer. In people living with Alpha-1, the single AAT proteins link together to form long chains called polymers. The long polymer chains cannot be transported normally into the bloodstream, so a deficiency of AAT develops in the blood. This is considered a root cause of lung disease in Alpha-1. The AAT polymer chains accumulate in the liver cells, and in some Alpha-1 patients, this leads to liver disease. Dr. Gershenson’s study will attempt to determine the molecular structure of AAT polymers by measuring and mapping the polymers, using a technique called electron paramagnetic resonance.