ALS Society of Nova Scotia
www.alsns.ca careandhope@alsns.ca
454-3636
October 2011

Terri Cooper – Client Services Manager clientservices@alsns.ca
Kimberly Carter – President/CEO careandhope@alsns.ca
John Forsyth – Chair of the Board c/o careandhope@alsns.ca

ALS – Anyone’s Life Story - The Story of Darrell


At the Anyone's Life Story evening on September 21st at City Hall in Halifax, Darrell Cottam shared his love of photography with us. Since his ALS diagnosis Darrell's speech has deteriorated and he has had to leave his job in sales. However, this has meant that he is free to pursue his passion of photography. This is what his wife, Deana has to say about his photo entitled Fisherman's Cove, NS, June 2011 "This photograph represents Darrell's quiet nature, his art, and his love of life. It represents his strength and stubbornness and his determination to be forever who he always was". To see Darrell’s extraordinary photos including “Fisherman’s Cove” visit www.Easternlight.ca.

Circle of Friends
People living with ALS and their families are surrounded by the most amazing circle of friends across the province. These friends work tirelessly to ensure the ALS Society has the financial resources to keep on providing equipment and support.
In September alone:
Soda Shoppe Gang - $12,40, Live Drive (British Automobile Touring Association NS) - $7,177, In Memory of Mary (Sissy) Royal - $7,000
Our hearts are overflowing with appreciation!



2011 Walk for ALS
www.walkforals.ca
From June – September 7 walks for ALS were held in different communities across the province. Literally hundreds of people raised $96,113 for research (40%) and care (60%). There were barbecues and fish fries, bake sales, music, silent auctions, and so much more. Thank you to team captains, walkers, pledgers, donors, volunteers, sponsors and so many more who helped make these walks a huge success.
Halifax $46,682
Sheet Harbour $3,000 +56% from 2010! Sydney $17,878 +34% from 2010!
South Shore $12,313 +40% from 2010! Truro $2,577
Western Shore $9,554 +33% from 2010! North Hants High School $898
Other funds were raised from sponsorship and the Running Room race - $3,211.


West Jet Draw

What would you do if you still could?
Take to the skies and fulfill your dream of a special vacation , a trip to spend time with family and friends, or participate in a long planned for adventure! Also makes a great Christmas gift for students, teachers, hairdressers, co-workers, babysitters! Prize includes a trip for two to any one of West Jet’s 55 destinations. Includes all airfare, taxes, and surcharges. Tickets – $10 apiece or 2 for $15. To purchase tickets or for further information. careandhope@alsns.ca 454-3636
Draw Date: January 09, 2012 – Tickets may be booked up until January 2013.

Quotes and Notes

We like to share with you some quotes from people who have used the services of the ALS Society. It’s not about “blowing our own horn” but it is about connecting you to the people who use our services. We want you to know how your support helps us help people and their families with ALS. You may never meet some of the people we help but every time we help them you have a huge part in it. Thank you!
From a sister who lives far away from her sister living with ALS.” I truly appreciate your concern and time you have taken to visit with my sister and to respond to me.”
“We are very fortunate to have such a caring and loving ALS society.”
“I also want to thank you for the scooter it really made my vacation much better, I was able to preserve my energy and not miss out on anything.”

Team Nova Scotia - 2011

The Inaugural Team Nova Scotia for ALS was introduced at the Anyone’s Life Story event on September 21st at Halifax City Hall.
Team Nova Scotia is a widely expanding group of people right across this province who either volunteer 50 hours in service, assist with events, or fundraise directly for people living with ALS, lead a group or host an event raising over $5,000 for ALS, or who significantly raise the profile of the organization.
We simply could not do it without this circle of friends.
2011 Inductees:
Walk Coordinators: Liz Arsenault, Nannette Clarke, Rodney Clarke, John Garroway, Maureen Hope, Nelson Murphy, Anne Savoy
Volunteers Extraordinaire: Peggy Amyotte, Donna Sutherland
Super Fundraisers: Andrew James, Cindy James, Craig James, Mark James, Shelly Brown, Jillian Darroch, Nancy & Stephen Dugas, Roger Phinney, Crystal Sparrow, Jordan Sparrow Ray Whittley
Speaking for and Supporting People with ALS: CTV (Mike Elgin, Liz Rigney, Tina Simpkin), Anthony Cooper (Hour Glass Media),Pamela Gray & Brian Penny, Emily Meehan, Susan Rahey, HRM Councillor Sue Uteck , Michael, Brenda, Michelle, Kim Watson
Equipment Program Partner: Paula Gallagher, Deloitte Touche Halifax
ALS NS Board Members & Staff: John Forsyth, Don Todd, Alison MacRae, Chris MacRae, Troy Yeo, Stephen Dugas, Brian Smith, Dr. Brian O’Brien, Dr. Victor Rafuse, Jill Taylor, Dana Clements, Terri Cooper, Patrick Little, Kimberly Carter
If you would like to become a member of Team Nova Scotia please email careandhope@alsns.ca for a brochure and application. The more the merrier!

Care – The work of ALS NS
The ALS Society of Nova Scotia is currently serving 72 families in over 30 communities across the province. In addition to our Equipment Loan Program over the last two months we have been able to help people:
• Find out what is involved in bringing a family member with ALS back to Canada from the US, making plans for his transition.
• Helping a person living with ALS do her errands.
• Helping a caregiver arrange for financial support for family member living with ALS.
• Weekly visits to people with ALS in hospital.


Hope – Research/The Work of ALS Canada
Landmark studies identify new gene as the most common cause of ALS and Frontotemporal Dementia (FTD)

Two studies published in the September 21 online issue of Neuron report the identification of the long-sought genetic abnormality which authors say is the most common cause of two different but related forms of neurodegenerative disease: FTD and ALS.

The first study, entitled “Expanded GGGGCC hexanucleotide repeat in non-coding region of C9ORF72 causes chromosome 9p-linked frontotemporal dementia and amyotrophic lateral sclerosis” was led by neurogeneticist Rosa Rademakers, PhD, at the Mayo Clinic in Florida. The researchers identified a short DNA sequence repeated hundreds to thousands of times in almost 12 per cent of familial FTD and more than 22 per cent of familial ALS of samples studied. Ian Mackenzie, MD, neuropathologist at Vancouver General Hospital and professor in the department of pathology and laboratory medicine at the University of British Columbia (UBC) also served as a lead investigator in this study.

“This is the most common genetic defect, in both familial and sporadic cases, identified to date in these two different, but equally devastating neurodegenerative conditions, and confirms an overlap between FTD and ALS at the molecular level,” says Dr. Mackenzie.

Using invaluable samples from a number of well-characterized BC families with FTD and ALS, Dr. Mackenzie identified a pattern of pathology in the post-mortem brain tissue of patients that accurately predicted the presence of the mutation. This work proved critical in demonstrating the impact of the mutation on brain cells by demonstrating abnormal patterns of protein expression and the presence of the potentially toxic accumulations of mutant RNA.

Dr. Mackenzie has been a featured speaker at the ALS Canada Research Forum and you can read more about his work in the Summer Edition of Research News - http://www.als.ca/researchnewsletter

The second study, entitled “A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD” was led by scientist Bryan Traynor, MD, Laboratory of Neurogenetics, National Institute on Aging, Maryland. This study took advantage of the genetically homogeneous Finnish population to identify the GGGGCC expansion in the first intron of the same gene in familial ALS and sporadic ALS patients, as well as in FTD patients, and further confirmed the finding in familial ALS cases of outbred European descent. Toronto-based investigators Lorne Zinman, MD, medical director of the ALS/Neuromuscular Clinic at the Sunnybrook Health Sciences Centre and chair of the Canadian ALS Trials and Research Network and Ekaterina Rogaeva, associate professor, Centre for Research in Neurodegenerative Diseases, were co-authors on this study.

Findings reported today represent the culmination of years of genetics studies of familial ALS and ALS-FTD including seminal work by groups led by Guy Rouleau, MD, PhD, professor, department of medicine at the University of Montreal, Robert H. Brown Jr., MD, DPhil, professor of neurology at Harvard Medical School and associate neurologist at the Massachusetts General Hospital, and Christopher E. Shaw, MD, department of neurology, King's College London School of Medicine.

Last year, two separate studies employing the genome-wide association approach confirmed linkage of ALS to the region of chromosome 9 in which the newly reported gene C9ORF72 resides. (See story https://mail.als.ca/exchweb/bin/redir.asp?URL=http://www.als.ca/_news/4472.aspx)

What does today’s breakthrough discovery mean for the ALS community?

One immediate consequence will be the benefit of precise genetic screening to aid in the genetic counselling process for the affected families (FTD and ALS). Further, the reported observations of the variability of presenting symptoms in both ALS and FTD patients bearing the mutation can provide both impetus and opportunity to learn more about patient care and management in individuals presenting with what appear to be separate clinical entities.

Finally, the substantial import of these new study results is the opportunity to learn more about mechanisms of degeneration related to both ALS and FTD. The gene mutations account for a significant proportion of patients with both clinical (as well as the combined) syndromes, and thus further study of the mechanisms can be expected to contribute to meaningful therapeutic development. Both studies support the likelihood of more than one potential downstream mechanism resulting from the hexanucleotide expansions. And, both studies agree that disruption of RNA processing, particularly in the nucleus, is highly likely to be one of those downstream disease mechanisms. Altered regulation of RNA function is already a proposed mechanism in motor neuron degeneration (See story https://mail.als.ca/exchweb/bin/redir.asp?URL=http://www.als.ca/_news/4419.aspx), which continues to receive support from experimental models of ALS. Today’s new findings will add momentum to this important line of research.

Taking a step back from the excitement generated by these two new studies, Denise Figlewicz, PhD, vice president research for the ALS Society of Canada says:

“2011 has been an exceptionally productive year for research related to ALS. Looking at the field from the outside, someone might wonder ... New genes! New mechanisms! Significant new findings related to established mechanisms! What is really important with respect to ALS and moving towards treatments? The answer is ... all of these studies are important.

Over the past decades it has frequently been said that there is no known cause of ALS. This is not correct. In the cases where ALS is inherited as a familial trait, we do know the causes for many of these. Mutations in a number of genes, beginning with SOD1 and ending with the C9ORF72 gene announced today, account for a large fraction of those familial cases. In addition, studies in recent years have demonstrated the involvement of many of those genes in cases of sporadic ALS. This means that experimental models based on those mutant genes must be providing very important information about mechanisms of motor neuron degeneration which occurs as a result of the primary causes. And, the phenomenon of disease progression from one region of the body to another, corresponding to one region of the nervous system to another, is being intensely investigated at the level of the motor neurons and their neighbouring cells (glia) as well as inside the motor neurons at the molecular level.

Halting the progression of ALS is the expected outcome of therapies which effectively target any of these pathways and mechanisms currently being studied. Targeting more than one of these at the same time may prove to be the most effective strategy.

In conclusion, there has never been more reason to be hopeful and optimistic that research will bear fruit and provide the badly needed therapies for those living with ALS.”




Upcoming Events
February 08th The Bucket List Event
Can you help us out with an event we’re having next February (details below) to raise money and awareness for people living with ALS?
The Bucket List Dreams Night
February 08th, 2012
Proceeds for the ALS Society of Nova Scotia
If you’re interested in finding out more call or email Kimberly Carter – careandhope@alsns.ca
(902) 454-3636.

What would you do if you still could? Would you clean the house or build a sandcastle with a child? Would you choose the same career path or try a different one? Would you paint the house or paint a picture? Would you write a complaint letter or write a love note to someone special? Would you work overtime or take that adventure vacation? Would you drive a sedan or a sports car?
Here’s what we’re planning on February 08th. We want to raise awareness and money for people and their families living with ALS. We want to remind people that life is precious and so we’re hosting The Bucket List Dreams Night.
It’s a pajama party/movie night featuring a showing of, you guessed it The Bucket List movie with Morgan Freeman and Jack Nicholson. And yes, there will be pop corn, soda, Milk Duds . . . no healthy snacks!
We’re asking well known Nova Scotians to paint their dreams on aluminum buckets that will be auctioned off with certificates of “gifts of experience” in them. Example: a fancy camera with photograpy lessons, an opportunity to guest host a radio or television segment, learn to sky dive, drive a fancy sports car for a day complete with gourmet picnic, host a cooking show, take an adventure vacation.
So, if you see yourself sponsoring this event, donating a prize, working on our organizing committee, donating a prize, painting a bucket or buying tickets – contact Kimberly today!
Please feel free to pass this on.