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Going to the fair can be a lot of fun. But can it also be healthy?

Marin County Fair has proven that answer to be a resounding YES! The annual event,
slated this year for June 30 –July 4, also proves that big changes can occur in small (yet
significant) steps.

In 2004, “Play Fair Marin” replaced beer as the fair’s title sponsor, evolving into a policy
that eliminates alcohol advertising altogether. And the healthy changes didn’t stop there!

• 2007: The event became the first smoke-free fair in the nation.
• 2008: Food vendors were required to add healthier options to their menus, with
new items becoming so popular, some vendors have rolled them out to other
festivals.
• 2011: A 1k fun run was introduced.

The Play Fair program isn’t just about the health of fair-goers. Environmental well-being
is equally important, with efforts focused on recycling, conservation, valet bike parking,
and even compostable containers.

A Fun, Green, Healthy, Happy, and Award-Winning Event

Marin’s “Greenest Fair on Earth” features attractions such as the first solar-powered
carousel at a county fair, interactive exhibits that showcase sustainable themes, and a
concert stage powered by 99 percent vegetable oil biodiesel.

Even rides at the fair are going green. The Giant Ferris Wheel has been retrofitted with
the latest in LED, as have the Ring of Fire and Super Shot Drop Tower. As a result,
these rides are using 90 percent less power!

“Giving fair-goers healthy choices, eliminating smoking, being careful with advertising
and the sale of alcohol, offering a welcoming baby sanctuary for nursing moms, and
using totally compostable materials all make the fair more family-friendly, and healthier
for our community and the environment,” said Patricia Kendall, medical group
administrator at San Rafael Medical Center. “It makes the fair a gift to all who attend.”

Play Fair Marin now includes more than a dozen community partners and has drawn
accolades and national attention. Working collaboratively, Marin County Fair, Play Fair
Marin, and Healthy Marin Partnership* have received numerous awards from the
Western Fairs Association, including the prestigious Merrill Award. In addition, festivals
across California and in neighboring states have looked to the Marin model for making
their own events healthier.

The Wine Business Institute (WBI) at Sonoma State University (SSU) today announced that it will host the 10th annual Academy of Wine Business Research (AWBR) conference from July 25 – 28, with program activities taking place on its Rohnert Park campus and throughout Sonoma and Napa counties. AWBR is an international society of academics and scholars with research focus on economic, social, and management issues related to the global wine industry. Confirmed attendees represent academic institutions in Austria, Australia, Brazil, Chile, China, France, Germany, Italy, New Zealand, Spain, and the United States. WBI has been involved with the annual conference since its founding in 2003, and previously hosted the gathering in 2005.

The 2017 Wine Business Education Summit, designed as a prelude to this year’s conference, will assess industry needs related to education, hiring, and employee retention. The half-day Summit features executive directors from California wine and grower associations, human resources executives from the wine industry, and executives from local staffing companies.

A full conference schedule may be viewed here: http://web.sonoma.edu/sbe/awbr-conference-2017.html

“We’re pleased that the international Academy wants to bring its annual conference back to the North Coast and to Sonoma State University. It’s a statement of appreciation for California’s importance to the global wine industry, and recognition of the Wine Business Institute’s leadership role in research and education,” Ray Johnson, Executive Director of the Wine Business Institute said. “The wine industry continues to change here and abroad, and we expect both the education summit and conference sessions will offer valuable exchanges, discoveries, and powerful takeaways for industry professionals and academics alike.” 

“We look forward to welcoming our wine business academic colleagues from around the world, to share and discuss recent research on issues of importance to the global wine industry, including how to build profitable and responsible wine businesses,” Dr. Liz Thach, MW, Distinguished Professor of Wine said.

Conference sessions include paper presentations under the headings: “Online Purchases of Wine”; “Managing Uncertainty in the Wine Industry”; “Measuring Market Performance”; “Understanding Wine Purchasing Behavior”; “Supply Chain and Exporting Issues”; “Industry Perspectives on Sustainability”; “Managerial Approaches”; “Sustainability and its Link with Customers”; “Wine Ratings and Raters”; “Wine Industry Trends”; “The Role of Tradition in the Wine Industry”; “Case Study Workshop”; “A Better Understanding of Wine Consumers”; “Marketing of Wine Terroir and Territories”; “Wine Marketing in a Digital Age”; “Wine Tourism”; “Marketing and the Wine Purchase Decision”; “Management Strategy”; “Wine Sales and Trends”; “Wine Marketing in Eastern and Southern Europe”; and “Wine Industry Development and Growth.”

Round table discussions include, “Wine and Intellectual Property, Implications for Innovation, Legislation, Marketing and Branding”; “Defining and Measuring Performance in the Wine Sector, The Sustainability Factor”; “Wine Tourism Experience, the Role of Heritage and Terroir, and the Differences Between “Old World” and “New World” Wine Regions”; and “How California’s Universities and Colleges are Providing the Optimal Workforce to the Local Industry.”

The 2017 Wine Business Education Summit will begin with a morning session facilitated by Ray Johnson, WBI Executive Director, including small and large group discussions to inform workplace issues and future wine business programming at the Wine Spectator Learning Center and globally. The program also includes a Wine Industry Colloquium featuring a panel discussion on the topic, “Wine Business Education: Where Do We Go from Here?”. Moderator Anisya Fritz, proprietor of Lynmar Estate, will facilitate an exploration with panelists Amelia Ceja, President at Ceja Vineyards; Luke Jencks, President at NakedWines.com; Michael Moone, past President at Beringer Wine Estates; and John Williams, President at Frog’s Leap Winery.

For more information regarding the 10th annual Academy of Wine Business Research conference, please contact the Conference Administrative Coordinator, Christine MacMillan, at cmcmillandestinations@gmail.com. For information regarding the 2017 Wine Business Education Summit, please contact Ray Johnson, WBI Executive Director, at ray.johnson@sonoma.edu or (707) 664-3071. For information regarding wine business research, programs, and degrees in the School of Business and Economics at SSU, please call (707) 664-3235, email winebiz@sonoma.edu, or visit www.sonoma.edu/sbe.

BioMarin Pharmaceutical Inc. (Nasdaq:BMRN) today announced that the U.S. Food and Drug Administration (FDA) approved Brineura™ (cerliponase alfa) to slow the loss of ambulation in symptomatic pediatric patients 3 years of age and older with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase 1 (TPP1) deficiency. Brineura is the first treatment approved to treat children with CLN2 disease, a form of Batten disease.

CLN2 disease is an ultra-rare, rapidly progressive fatal brain condition, which affects less than one in one million U.S. residents, many of whom are undiagnosed.  Every year approximately 20 children are born in the U.S. with CLN2 disease.  These affected children completely lose the ability to walk and talk around 6 years of age.  During the later stages of the disease, feeding and tending to everyday needs become very difficult with death often occurring between 8 and 12 years of age.

In clinical trials, Brineura, an enzyme replacement therapy, was shown to slow the loss of ambulation in symptomatic pediatric patients 3 years of age and older with CLN2 disease. It is the first enzyme replacement therapy to be directly administered to the brain, treating the underlying cause of the condition by replacing the deficient TPP1 enzyme. Using an established technique most often used in oncology – intraventricular administration– the therapy is delivered directly into fluid surrounding the brain, known as the cerebrospinal fluid.

To support the community of families with children with neurologic disorders, BioMarin in partnership with a commercial lab is offering a no cost genetic testing program called ‘Behind the Seizure’ to support early testing for children who experience seizures.  In addition, BioMarin is investing in tools and resources to educate physicians on CLN2 disease in order to help them diagnose patients with this disease earlier and prevent them from being misdiagnosed during critical years when therapy could help slow the loss of ambulation.

In addition, BioMarin RareConnections™, a resource available to patients and families, provides a variety of personalized support services at no cost to patients, including education on CLN2 disease and Brineura, and coordination of additional services, such as information about financial assistance programs.

“We thank the FDA for recognizing Brineura’s potential to alter the course of CLN2 disease and its urgency in delivering this treatment to children as quickly and safely as possible. Brineura was approved in under four years from starting the first clinical trial to today, a significant achievement for a condition that progresses so rapidly,” said Jean-Jacques Bienaimé, Chairman and Chief Executive Officer of BioMarin. “Treating children with CLN2 disease requires an extraordinary amount of collaboration between families, hospitals, advocates and physicians. We are grateful for the partnership of all those involved and look forward to continuing to work together to make Brineura accessible to children who may benefit.”

“CNL2 is a devastating diagnosis that robs families of life with their children much too young,” said Emily de Los Reyes, M.D., attending pediatric neurologist at Nationwide Children’s Hospital and principal investigator for Brineura studies. “Today’s announcement gives my patients and their families hope.”

“The approval of Brineura is an extraordinary medical breakthrough for the CLN2 Batten community who have been waiting for this moment for more than a century when the condition was first described,” said Margie Frazier, PhD, LISW-S, Executive Director of Batten Disease Support and Research Association. “We appreciate BioMarin’s commitment and partnership to the CLN2 Batten community and investing the resources needed to bring this pivotal treatment to families.”

With this approval, the FDA also issued a Rare Pediatric Disease Priority Review Voucher, which confers priority review to a subsequent drug application that would not otherwise qualify for priority review. The rare pediatric disease review voucher program is designed to encourage development of new drugs and biologics for the prevention or treatment of rare pediatric diseases.

Brineura is expected to be available in the United States by early June, and BioMarin will begin promotion of Brineura immediately.

Last week, the Committee for Medicinal Products for Human Use (CHMP), the scientific committee of the European Medicines Agency (EMA), adopted a positive opinion for the company’s Marketing Authorization Application (MAA) for Brineura to treat children with Neuronal Ceroid Lipofuscinosis Type 2 (CLN2) disease, a form of Batten disease, which is also known as tripeptidyl peptidase 1 (TPP1) deficiency.  The CHMP’s recommendation is now referred to the European Commission (EC), which is expected to render its final decision by the second quarter of 2017.

Clinical Trial Results

The approval was supported by safety and efficacy data assessed over 96 weeks in a non-randomized, single-arm dose escalation clinical study of patients with CLN2 disease. Brineura treated patients were compared to untreated patients from a natural history cohort.

Patients were assessed for decline in the motor domain of the CLN2 Clinical Rating Scale. The scale measures performance of mobility with normal function being a score of 3 and no function being a score of 0.  Decline was defined as having a sustained 2-point decline or an unreversed score of 0 in the motor domain of the CLN2 Clinical Rating Scale.

Twenty-four patients aged 3-8 years were enrolled in the clinical study. One patient withdrew after week 1 due to inability to continue with study procedures; 23 patients were treated with Brineura every other week for 48 weeks and continued treatment during the extension.

Results from the 96-week analysis demonstrated the odds of Brineura-treated patients not having a decline were 13 times the odds of natural history cohort patients not having a decline (Odds Ratio (95% Confidence Interval): 13.1 (1.2, 146.9)).

Of the 22 patients treated with Brineura and evaluated for efficacy at week 96, 21 (95%) did not decline, and only the patient who terminated early was deemed to have a decline in the motor domain of the CLN2 Clinical Rating Scale. In comparison, 50% of patients in an independent natural history cohort demonstrated progressive decline in motor function.  Two Brineura treated patients with a maximum score were excluded from the analyses; they maintained that score throughout the study period.

In the clinical study, intraventricular access device-related infections were observed in two patients. In each case, antibiotics were administered, the intraventricular access device was replaced and the patient continued on Brineura treatment.  Hypotension was reported in 2 (8%) patients, which occurred during or up to 8 hours after Brineura infusion. Patients did not require alteration in treatment and reactions resolved spontaneously or after IV fluid administration.

Hypersensitivity reactions have been reported in 11 (46%) Brineura treated patients during the clinical studies.  The most common adverse reactions (≥8%) are pyrexia, ECG abnormalities, decreased cerebrospinal fluid (CSF) protein, vomiting, seizures, hypersensitivity, increased CSF protein, hematoma, headache, irritability, pleocytosis, device-related infection, bradycardia, feeling jittery, and hypotension.

About CLN2 Disease

Children with CLN2 disease typically begin experiencing seizures between the ages of 2 and 4 years old, preceded in the majority of cases by language development delay.  The disease progresses rapidly with most affected children losing the ability to walk and talk by approximately 6 years of age.  Initial symptoms are followed by movement disorders, motor deterioration, dementia, blindness, and death usually occurring between the ages of 8 and 12 years of age.  During the later stages of the disease, feeding and tending to everyday needs become very difficult. BioMarin estimates the incidence of CLN2 disease is approximately one in 200,000 with up to 1,200 to 1,600 children in the regions of the world where BioMarin operates, many of whom are undiagnosed.

The neuronal ceroid lipofuscinoses (NCLs) are a heterogeneous group of lysosomal storage disorders that includes the autosomal recessive neurodegenerative disorder CLN2 disease. CLN2 disease is caused by mutations in the TPP1 gene resulting in deficient activity of the enzyme tripeptidyl peptidase 1 (TPP1). In the absence of TPP1, lysosomal storage materials normally metabolized by this enzyme accumulate in many organs, particularly in the brain and retina. Buildup of these storage materials in the cells of the nervous system contribute to the progressive and relentless neurodegeneration which manifests as loss of cognitive, motor, and visual functions.

Arrow Benefits Group has allocated an unprecedented $100,000 in order to place AED (Automated External Defibrillator) machines with employers throughout the local community over the course of the next five years. This builds on its Arrow Wellness Initiative, which was established two years ago to offer free CPR and first-aid classes to its clients and the community.

“The Arrow Wellness Initiative has been utilized by businesses since its inception, and it was always our goal to build on this program,” said Arrow Benefits Group Principal Andrew McNeil. “Our clients see value in implementing employee benefits that go beyond the norm and that improve health and cost outcomes, as well as performance in the workplace. We are dedicated to helping our clients take proactive actions to keep their employees healthy and safe.”

Arrow’s funds will be donated to clients who wish to purchase AED machines for their business location. To help implement its CPR and first-aid training, as well as AED installation, maintenance and registration, Arrow has partnered with the Petaluma Health Care District’s HeartSafe Community initiative and Healthquest training center.

“HeartSafe Community was established to strengthen the community’s ability to respond to a cardiac emergency,” said Ramona Faith, CEO of the Petaluma Health Care District. “Arrow was one of our first HeartSafe Businesses and is pioneering how companies should be approaching wellness in the workplace. With regard to heart health in particular, businesses are on the front lines with cardiac response because cardiac arrest is the leading cause of death in the workplace. Arrow understands this and is doing amazing work in our community. Its pledge to buy AEDs for businesses throughout the region is unparalleled.”

Access to AED machines significantly increases the chance of survival in the event of sudden cardiac arrest, which is the leading cause of death within the workplace, claiming 10,000 lives each year. Immediate CPR and AED deployment within three-five minutes of collapse increases sudden cardiac arrest survival rates as much as 70%; without intervention, survival rates average 7%. The dramatic difference in survival rates with the addition of an AED device is what motivated Arrow to donate $100,000 and partner with North Bay employers to place these machines within workplaces throughout the region. By coupling the District’s HeartSafe Community initiative and Arrow’s donation, McNeil hopes to improve safety within the workplace, and, by extension, within the community. He adds, “We believe we can build a stronger community by building stronger employers. Stronger employers translate into a stronger local economy and happier, more productive citizens.”

“Arrow is the first company in our area to make this kind of a donation for AED machines,” said Stacy Gibbons, executive director of American Heart Association in the North Bay. “There are not enough in our community, and the contribution will not only help get them placed with employers, but it will save lives. The AED is critical to the survival of someone who has suffered a cardiac arrest – 10,000 Americans die on the job from cardiac arrest each year.”

For more information on the program contact Andrew McNeil 707-992-3789 or andrewm@arrowbenefitsgroup.com.

About Arrow Benefits Group

Arrow Benefits Group, the third largest benefits firm in the North Bay, is a proud partner of United Benefit Advisors (UBA), one of the largest benefits consulting and brokerage firms in the country.

Arrow Benefits Group is the single-source solution for managing the complexities of benefits with expert advice, customized programs, and personalized solutions. Arrow’s innovative programs control costs and give employees a greater sense of financial and emotional security. For straight answers to employee benefits call 707-992-3780 or visit http://www.arrowbenefitsgroup.com.