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Russell Hampton
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Salin Low
Getting to Know our Members
Getting to Know Peter Vignati

Peter Vignati has fond memories of growing up in East Hartford. He says he is “the classic middle child” amongst five siblings. Peter attended the East Hartford public schools and went on to Bates College, where he majored in chemistry and biology. At Bates he really enjoyed playing golf and was the Maine intercollegiate champion one year.

After graduation, Peter went to work for Enthone, a West Haven, Connecticut manufacturer of specialty chemicals for the plating, electronics and aerospace industries. He started in sales, first in Wisconsin and then returned to Connecticut. Later he became an International Marketing Manager, traveling worldwide establishing relationships with overseas companies to license Enthone’s technology to them and also to license some of their technology. Being single, it was an exciting time for him to see and experience different people and their cultures. He went on to earn a Master of Business Administration from the University of Connecticut, taking classes at night.

In the meantime, Peter was introduced to Susanne “the love of my life” on a blind date and now his wife of almost thirty years. “Best thing I ever did”, he says. They lived in a beach condo in East Haven after they were married and then relocated to Farmington to raise their family. Peter and Susanne lived for two years on the Miss Porter’s campus rent-free as “house directors”. It was a fun time for them and they met many great young girls attending the school, including Oprah’s niece who lived in their house. Oprah later delivered a commencement speech at graduation.

Peter then spent seven years in Marlboro and Freehold, New Jersey working for a chemical company with a colleague that he had known over the years. That company was bought out by a Cleveland firm, but he didn’t want to relocate again for work. They now had three little boys and had enjoyed their proximity to New York City and New Jersey’s great beaches. So it was time for a career change and a move back to Connecticut where he started a business for Edward Jones, which he continues to run today. Never in his wildest dreams did he ever think he would become a financial planner, but he does enjoy helping families achieve their goals and get on a path to a secure retirement.

Peter and Susanne have always been active in their church, whether it be the Church of Saint Ann in Avon or their current church, Sacred Heart Church in Bloomfield, where they relocated three years ago. He says “I’d like to think that we are generous with our time and talents to help those in need and my children are also engaged in similar activities.” None of their boys are married yet, but he is thankful that all are in school or gainfully employed. Peter and Susanne enjoy hiking with their two Jack Russell Terriers, Bailey and Finn. They also enjoy biking, skiing and travelling. They look forward to trips to the Canary Islands and Iceland this year.

Peter is in his seventeenth year with Rotary and is a Past President and Paul Harris Recipient.

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Dog Star Rescue
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Aug 16, 2019
Oil Drum Art-an NFP to Benefit Haiti
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Executives & Directors
President
 
President Elect
 
Immediate Past President
 
Treasurer
 
Secretary
 
Sergeant-at-Arms
 
Foundation Chair
 
Membership
 
Board Member-at-Large
 
Board Member-at-Large
 
Asst Treasurer
 
Interact Chair
 
EarlyRiser Presentations
The Annotated Guide to Ending Polio
GLO Fall 2017 Mission Trip
Eastern Europe Trip to Vienna and Prague Presentation
Avon Village Center Project Presentation
Club Information
Rotary of Avon-Canton - Founded 1973
Avon-Canton
Service above Self
We meet Fridays at 7:30 AM
Avon Old Farms Hotel
279 Avon Mountain Rd.
Avon, CT  06001
United States of America
DistrictSiteIcon District Site
VenueMap Venue Map
Upcoming Meeting Speaker
Deene Morris
Servant Leadership and Psychological Safety
 
 
 
 
Sergeant-at-Arms Report
February 22, 2019
 
Members Present: 41
 
Make-Ups: None  
Guests: Lisa Wright (guest of Gary Hyde; inducted later in the meeting), Joe DiNicola (son) & Ketil Osland ) (plans to rejoin club) (both guests of Robin DiNicola).
Visiting Rotarians: Prasad Menon - Simsbury-Granby Club
Happy Dollars: $20
Raffle Winner: Capri Frank Brighenti
 
Birthdays
 
Gary Miller
 
 
Gary has been a member of Rotary for nearly 10 years. He was sponsored by Phil Worley and succeeded Phil as Executive Director of the Canton Chamber of Commerce. He is also involved with the Canton Lions, the Farmington Valley Trails Council, and the ski patrol at Ski Sundown. He is Co-Chairman of the Polio Plus Committee of the District Foundation Committee and is Webmaster for the club. He was President of the club in 2015-2016.  In this picture he is giving is annual donation to the Rotary Foundation, although he also contributes monthly to the Foundation.
 Happy Birthday, Gary.
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Rauf Majidian
 
 
 
 
 
Rauf celebrated his 30th birthday last week, an event that is but a foggy memory for many of our members. He is a native of Tajikistan, no doubt the first from that country to be part of our club. He came to the United States when he was about 20 and became a citizen in the spring of 2018. He says he now looks forward to Fridays when he comes to our meetings. We are glad that he is strengthening our club. Happy Birthday, Rauf!
                                                                                                                                                    
 
 
 
 
 
 
 
 
 
Happy Dollars
 
Prasad Menon reminded us that Rotary would be 114 years old on February 23.
 
Gary Miller gave $3 for the three grandkids visiting him. They are 6, 9, and 11 and are hard to keep up with. They have become fairly good skiers which pleases Gary.
 
Peter Vignati was happy that his sister has responded well to chemo therapy to treat her pancreatic cancer. Her tumor has shrunk which makes surgery possible. She is a strong athlete, and she gets meals from Sarah Leathers's Healing Meals. She loves the meals and is sure that they are helping her to stay strong.
 
Capri Frank, aka Mrs. Brighenti, was glad to be back. She and John were married in January with all five of their sons about to be present. They took a minimoon for five days in Naples, Florida. They saw Nancy Nation's daughter Liz and her huband John who are "hanging in" trying to support Nancy and husband Craig while Nancy goes back and forth from treatment in Houston.
 
Larry Haber wasn't really happy about the idiot who had run into him the day before the meeting.
 
Robin DiNicola thanked Peter Vignati for giving her the opportunity to pursue a new career. She has been visiting other clubs to push Tastes of the Valley and has gotten ideas from other clubs. The Southington club is having a Mardi Gras celebration.
 
Don Bonner had made 60 additional stickers for FAVARH vans, which he thinks will last until we have completed our commitment to them. He went into the new building and was amazed at the various rooms for therapy, meetings, and activities for FAVARH's clients.
 
Paul Mikkelson was happy about the success of the recent bike conference, especially since Les Zinner had shown up and bought a bunch of attire and accessories. He encouraged all of us to keep Nancy Nation in our thoughts while she is in Houston, attempting to get in a drug trial for cancer. Paul and wife Annie will be spending two weeks in Florida.
 
Gary Hyde has an interesting joke about Anthony Weiner.
 
 
Announcements
 
Rotary Foundation chairman, Joanne Santiago, seeks nominees for our annual Community Paul Harris Fellow recognition. Please contact Joanne or any of the Rotary Foundation Committee members with your suggestions. We typically honor 2-3 citizens each from Avon and from Canton.
 
Gary Miller is seeks a few members to purchase Rotary website ads, at the excellent price of $100/yr. This funding supports our use of ClubRunner software and our website subscription. The ads cycle every few seconds at the upper left-hand corner of the website and are a good way to promote member businesses. Contact Gary if interested.
 
Past President Don is planning the President's Dinner. He would like to have it at Farmington Gardens for the first time. The catch is that it would have to be on a Thursday, June 6. He wants to determine if that works as well for members as the usual Friday. Please let him know if Thursday doesn't work for you.
 
 
Calendar of Upcoming Events
 
March 22th: Club meeting at BEANZ - behind New England Pasta on Rt. 44 in Avon.
 
March 30th: Tastes of the Valley will be held Saturday, March 30 at 6 PM. 
 
May 3rd & 4th: The Rotary District 7890 District Conference will be held May 3rd to 4th  at the Doubletree Hilton Hotel in Danvers, MA. Five districts including 7890 will be represented. The event starts with the Rotary Foundation luncheon on Friday. For a full schedule and registration information follow this link: District Conference Registration
 
May 16th: Rotary Night at Hartford Yard Goats, Thursday May 16; Game time 7:05 PM. Contact Paul Mikkelson for details.
 
Induction of New Members
 
Lisa Wright
 
 
Gary Hyde introduced Lisa whom he is sponsoring for membership. She is a true New Englander, originally from New Hampshire. She has been club manager of the Golf Club of Avon for about a year and was a great help to our club during the golf outing last year. She is a member of the New England Club Managers' Association. She has two grown children, a son and a daughter, and each of them has children. Welcome to the club, Lisa!
 
 
 
 
 
 
 
 
 
 

Community Service Grants
 
None this week.
 

 
Presentation
Dr. Arnold Goldman
"X-Linked Myotubular Myopathy"
(Notes and slides provided by Dr. Goldman)
 
 
 
(Arnie clarified that this presentation is about labs - that is, laboratories, not labradors. Maybe next time we'll get that one.)
 
 
Rotarian and veterinarian Dr. Arnold L Goldman gave a presentation on the general subject of gene therapy for inherited disease, with the specific example of a muscle disorder found in male puppies and little boys called "X-Linked Myotubular Myopathy" or "XL-MTM". The presentation was based upon a more detailed presentation previously given by researcher Dr. David Mack of the University of Washington, Institute for Stem Cell & Regenerative Medicine, located in Seattle. Dr. Goldman had seen the presentation at the National Animal Interest Alliance Conference twice over the last few years and made it a point to meet the speaker.
 
The Joshua Frase Foundation helps to support research on XL-MTM, a disease that affects the X chromosome, the one chromosome that males possess just one copy of. Having no second X chromosome, males do not have an alternative set of potentially normal X chromosome genes to take over for those that might be defective on the first X chromosome. As females normally have two X chromosomes, and two copies of all X-linked genes, they can be carriers but are not affected by the disease.
 
 
In this case a gene that "codes" for an important muscle protein known as "myotubularin" is defective and little to no myotubularin is created. As a result, muscle cells, also known as fibers, in all the skeletal muscles of affected puppies and boys, remain in an undeveloped state, one which leads to extreme dysfunction.

 
XL-MTM is a terrible disease with an eventually fatal outcome for 100% of affected patients. Worldwide the incidence is 1 in 50,000 live born, male infants, with boys born "floppy", with severe muscle weakness and a characteristic long narrow face. Greater than 50% will die before their 2nd birthday, with just a few surviving into their teens. Death is due to respiratory failure as the muscles of respiration are affected and eventually the compensatory mechanisms of the body are overwhelmed. In the case of the little boys, before they succumb, the care required of their parents and caregivers is extreme and most commonly includes 24-hour respiratory support with a ventilator, an intervention that is costly and labor intensive. That is in addition to, all the other needs of any growing child. The impact on families is devastating with a heavy burden of physical, emotional and financial consequences.
 
Alison Frase the heroic mother of one such boy, Joshua Frase, and also the wife of now-retired NFL player Paul Frase, would not be deterred in her efforts to help find a way to save her son Joshua, who was affected by the disease. With encouragement from Joshua's doctors, she scoured the nation, and the world, for similar disease victims among other species, so that they might be studied in the hunt for a treatment for her son.

 
Mrs. Frase learned from a San Diego veterinary pathologist about a litter of puppies that had died shortly after being born, and with similar signs. The mother dog was owned by a Saskatchewan farm family, and Alison flew to Saskatoon that very night, arriving unannounced at the farm house doorstep after midnight. After explaining who she was, and that her son might have the same inherited illness as the farmer's dog, the farmer gave her the dog, named Nibs, to bring to the scientists to study. Alison returned to North Carolina with Nibs and soon put Nibs into the University of Washington researcher's hands.
 

 
The dog lived with the researcher's family in Seattle and not long after arriving, Nibs gave birth to another litter of 12 puppies. This litter included 1 affected male and 5-affected female carriers, and thus the study colony, and the critical research, was begun. As the research progressed, healthy puppies were placed with the human patient's families, so that they could have a personal connection to the research.
 
 
Barely 1 year later, one of the many collaborators in this research, Dr. Alan Beggs of Harvard University, published the results of a study identifying the gene "MTM1" as the gene that was defective in Nibs, her puppies and the affected little boys, including young Joshua Frase.
 
The next step was to pursue a treatment. The strategy was to develop a method to insert a normal copy of the MTM1 gene into abnormal muscle cells, causing the protein myotubularin to be produced, and then hope muscle structure and function would return to normal. To do that, a benign virus that normally targets muscle was used to deliver a normal MTM1 gene into cells that lack one. The process first depends on selecting a virus with a natural affinity for muscle, like the kind that causes muscle aches, so the functional gene "cargo" is delivered where it needs to go, and can be absorbed by the cell's own mechanisms into its chromosomes.

 
This method of viral gene delivery was first tried in mice engineered to have the defective MTM1 gene, and then in the next phase, in naturally affected dogs. The results in the dogs were more successful than could possibly have been imagined, with prolonged survival, restored muscle strength, reversed structural abnormalities, and no adverse immune system reactions to the virus or gene, and all that after a single treatment! What's more, 5 years on, the first two treated dogs remain healthy.
 
 
After publishing the successful outcomes in the treated dogs, the scientists approached the FDA for permission to treat the little boys, and soon received it. At a minimum it would be transformative if this therapy could restore even some respiratory function to these boys.
 
Dr. Goldman showed Rotarians video of young, affected dogs both before and after receiving this gene therapy. While for privacy and legal reasons he was unable to show us similar video of untreated & treated boys, he informed the audience that at least three boys have since been treate. He reported that all three are vastly improved and for the first time, functioning normally. Many other such boys are now in line for similar treatment in the near future.
 
 
 
Some important questions remain, questions that gene therapy scientists have yet to answer. Will one dose last for the lifetime of a patient?;   Will the virus persist in muscle & cause trouble later?; In infants, does therapy weaken as the baby grows to an adult?; If one dose doesn’t suffice, can the exact same virus/gene combination be administered again without adverse consequences?; Will there then be immune reactions to the virus, the gene or both?; Will the virus persist & evolve?; Will that be harmful?; Clearly, Dr. Goldman said, there is so much more work remaining to be done.
 
 
He continued, saying "often in veterinary practice, I'm asked what I think about the use of animals in biomedical research, for example beagle dogs, and whether its unethical to use them."  "I tell those who've asked, that research using animals benefits both people & animals and is essential to solve many health problems that animals and humans share. These so-called, animal “models” of human disease are essential for progress to be made, as there is no inanimate substitute for this kind of research. Computer simulations and tissue culture have their uses, but cannot model a living organism in all its complexity. Further, so called “rare” diseases often have common characteristics, sometimes allowing similar approaches to study & treatment. Finally, precisely because this research saves human lives, not doing it is what would be unethical."

Dr. Goldman's final point was that it had cost 18 million dollars a month for 5 years, to get to this point with
XL-MTM.  There are dozens of other diseases suitable for this form of treatment that are being studied with lives at stake. Please support and promote biomedical research, and also the Joshua Frase Foundation.
 

 
Mail Bag
 
No mail this week.
 
Photo Credits 
Photographs courtesy of Phil Worley unless otherwise noted.
 
Editor's Notes
Submission Deadline: Members are kindly encouraged to submit all materials for each week's Early Riser as early as possible. Please note that some editions may be published and distributed as early as the Saturday following our meetings, and during those weeks further contributions to the Early Riser will be included in the subsequent week's edition.
“The FOUR-WAY TEST of the things we think, say or do”:

1. Is it the TRUTH?
2. Is it FAIR to all Concerned?
3. Will it build GOODWILL and BETTER FRIENDSHIPS?
4. Will it be BENEFICIAL to all concerned?