SolarCraft Names Solar Industry Leader Ted Walsh as CEO

Novato and Sonoma-based SolarCraft, a leader in solar and clean energy system design and installation, has appointed Ted Walsh to serve as Chief Executive Officer.

Mr. Walsh, who formerly served as Vice President of Sales & Marketing at SolarCraft, takes the helm to guide the company’s strategic direction and fulfil the company’s mission of delivering the highest value solar and clean energy solutions. “Ted has done many impressive things in our industry over the last decade, and helped to grow local solar adoption to one of the most respected levels in the country,” Bill Stewart said. Stewart, SolarCraft’s Founder, former CEO and current Board Member continued: “Ted is committed to teamwork and holds a deep respect for our clients, partners and employees. We are excited to have Ted lead SolarCraft, and excited for the future as the company expands our technology and service offerings.”

Mr. Walsh has been a leader in the solar industry for more than 10 years, and has led several local solar companies to their most stable growth periods. Throughout his career, Ted’s local work in renewable energy has gained national recognition. In 2007, he executed the GoSolarMarin program, at the time representing the largest community purchase of home solar systems in the country. Successful programs GoSolarSonoma and GoSolarNapa soon followed, along with the development of hundreds of residential and commercial solar projects within the North Bay community. Significant projects include the deployment of the first two commercial-scale floating solar systems in the world for wineries in Sonoma and Napa Counties, the development of the country’s first solar-powered multiplex theater in Fairfax, as well as helping many of the Bay Area’s leading organizations transition to solar power, including Apple, eBay, Sutter Health, Jackson Family Wines, Gallo, Friedman’s Home Improvement, The Marin Mammal Center and many more. Mr. Walsh also gained recognition for his local client’s commitments to renewable energy from the Obama White House.

Prior to joining SolarCraft, Ted was President of Stellar Energy, a national commercial-scale solar provider based in Sonoma County, where he led their growth and eventual acquisition by the nation’s largest utility. Previously, Ted worked at Marin-based SPG Solar for six years, serving as VP of Sales & Marketing and contributing to the company becoming one of California’s leading residential and commercial solar providers before eventually being acquired by the world’s largest solar company.

Joining the SolarCraft team in February of 2017, Ted brings his passion for environmental leadership and local sustainability with his expertise and experience in renewable energy to SolarCraft. Ted combines a high level of integrity, customer perspective and personnel management experience with expertise in local energy markets, energy technology, and project finance. He believes that SolarCraft possesses the ideal balance of commitment to community, ‘client-first’ company culture, technical expertise and partnerships to best serve the Northern California market.

“Solar makes a difference, short and long term, locally and globally,” says Walsh, who has lived and worked in the North Bay for more than a decade. “SolarCraft energy solutions save clients money, keep that money within the community, and improve environmental health locally. Our North Bay community, and the larger Northern California region, must lead the way on environmental and economic responsibility. As a community, we can set an example that the rest of the state, the rest of the country and the world can follow.”

Mr. Walsh holds degrees in Economics, Managerial Accounting, and Chemistry from the University of Rochester, NY and the University of South Florida, respectively. A lifelong athlete, he competed in the decathlon and basketball in college and played professional basketball in Europe. He continues to coach youth sports locally, volunteers for local community and sustainability programs, and lives in Marin County with his wife and two young children in a solar-powered, environmentally low-impact home.

SolarCraft delivers Solar and Clean Energy Solutions for homes in the North Bay and businesses and non-profit organizations throughout Northern California. SolarCraft provides the most advanced solar technology with expert in-house design/build services, all back by comprehensive warranties supported by 34 years of local experience and dedicated service and maintenance teams. SolarCraft is 100% Employee-Owned and is proud to be one of the largest green-tech employers in the area. We are a certified Green Business, a Pacific Sun ‘Best in Marin’ Hall of Fame member, and consistently ranked as one of the best places to work by the North Bay Business Journal. Our team of dedicated employee-partners is proud to have installed more solar energy systems than any other company based in the North Bay, including many of our community’s most recognizable organizations. We invite you to learn more about SolarCraft and the economic and environmental benefits of solar at www.solarcraft.com

Marin’s ‘housing crisis on steroids’ focus of San Rafael forum

Former county supervisor Cynthia Murray kicked off a discussion on housing affordability in Marin with one question.

“Spoiler alert,” said Murray, now CEO of the North Bay Leadership Council. “How do people make ends meet in Marin? They don’t.”

Murray, speaking before the Marin Communications Forum on Monday, said the recent North Bay fires will worsen Marin’s longstanding affordable housing crisis. Robert Eyler, the chief economist of the Marin Economic Forum, and Robin Sternberg, the forum’s CEO, also addressed the housing issue and talked about the impact that housing ownership has on racial equity in Marin.

About 150 people turned out for the morning meeting at the Embassy Suites hotel in San Rafael. The Marin Communications Forum is a group of communications staffers from local agencies, organizations and service providers in Marin County.

“The housing that was lost in the North Bay is equal to all of the housing that has been built in the North Bay in the last few years,” Murray said. “We’ve had a housing crisis for decades. So now with the loss of 6 percent to 7 percent of the housing stock there we now have a housing crisis on steroids. This is a wake-up call. We have an emergency.”

She said the lost housing will likely inflate housing prices and rents in Marin further.

Murray said some 7,000 workers were displaced by the fires and there is no guarantee they will remain in the area.

“We’ve seen a big impact on our health care workers, our teachers, people who work as public employees, our child care workers and preschool teachers,” Murray said.

She said a worker shortage could become a serious issue for Marin, since about 60 percent of the county’s employees commute to work from outside the county daily.

“It is already increasingly hard to fill positions in Marin County,” Murray said.

She offered a number of prescriptions for the problem. She said changes in the California Environmental Quality Act need to be made to prevent its use as a “hammer” to squash project proposals. She said the county of Marin should explore ways to provide more funding for housing construction. She said more attention should be given to mixed-use projects and the creation of junior accessory and accessory units.

But, Murray said, perhaps most importantly, Marin needs “to develop the political will to approve more housing.”

“We have to accept that the only way to reduce the cost of housing is to build more housing,” Murray said. “The prices will always go up if the demand is more than the supply. We have been under-building for decades.”

To build political support for housing, Murray said she and others have formed the Housing Crisis Action Group.

“We have a rapid response team to go out and support projects and write letters and show up at hearings,” she said.

In addition to Murray, the organization’s steering committee consists of Diana Conti, president of the College of Marin Board of Trustees; Linda Jackson, a board member of Sustainable San Rafael and a former Transportation Authority of Marin planner; Robert Pendoley, a member of the Marin Interfaith Council and former Corte Madera planning director; and Kris Organ, former executive director of Service Employees International Union Local 949 that represents most Marin County Civic Center employees.

Sternberg, who became CEO of the Marin Economic Forum in August, said a report released last month showing that Marin is the most racially unequal county in California “set off alarms” for her.

The report produced by Advancement Project California, a Los Angeles-based civil rights organization, rated counties across seven categories. Marin’s poorest performance came in the housing category, where it also ranked first in racial inequity.

For example, 62.6 percent of whites in Marin own their own home, compared with 59.8 percent of Asians, 27.7 percent of African-Americans, 26.4 percent of Latinos and 8.5 percent of Native Americans.

“I feel this is posing the question to all of us: Are we going to continue to accept the status quo? I hope not,” Sternberg said.

Eyler, however, said the aims of housing everyone and increasing equity may be at cross purposes to some degree.

For example, both Eyler and Murray agreed that probably the biggest immediate impact on the housing crisis could be the proliferation of junior accessory and accessory units.

“They are probably the best play in the short term,” Eyler said.

But Eyler cautioned that these units, usually created by turning a spare bedroom into an autonomous living space, would be strictly rentals.

“There are generally benefits to home ownership,” Eyler said. “One of them is that you are building wealth.”

As a result, he said providing more rental housing in Marin would do little to close the gap in racial economic equity.

Eyler said raising education levels is a good way to increase incomes so people can afford more expensive housing and build equity. He cautioned, however, that in today’s world education needs to emphasize the academic disciplines of science, technology, engineering and mathematics, if it is be expected to pay economic dividends.

Sonoma Raceway Distributes More Than 4,400 Pounds of Food to North Bay Food Banks

Sonoma Raceway has taken the checkered flag on its 17th annual Thanksgiving Food Drive, distributing more than 4,400 pounds of non-perishable food to Friends in Sonoma Helping (FISH) and the Redwood Empire Food Bank (REFB).

This year’s 4,486-pound donation brings the raceway’s 17 year total to more than 36 tons of non-perishable food items for bay area food banks. In addition, the raceway also distributed more than $2,500 in contributions from community members to the food banks.

FISH has helped Sonoma Valley residents in times of need since 1970 and provides not only food, but clothing, transportation and emergency rental assistance to Sonoma Valley residents. The food collected through the Sonoma Raceway Thanksgiving Food Drive is used for the organization’s Holiday Food Basket program. Last year, FISH distributed more than 450 holiday food baskets, touching the lives of 1,700 adults and children.

“This donation helps fill the boxes for the holiday food baskets,” said longtime FISH volunteer Louise Bielfelt.  “You’re not making a dent, you’re making a really big addition to the baskets.”

Sonoma Raceway was not alone in its efforts. It was joined in the food drive by RKA Motorcycle Luggage in Windsor, Falck Northern California/verihealth, Levy Restaurants, Pedroncelli Mobile Bottling LLC, Save Mart and Lucky Supermarkets, Sonoma Drift and Wednesday Night Drags participants and spectators, Sonoma Gourmet, Sonoma Market and Glen Ellen Village Market, Sonoma Valley Fire and Rescue Authority, Schell-Vista Fire and Transcendence Theater Company.

The Redwood Empire Food Bank is the largest hunger-relief organization serving the Northern California Coast, from Sonoma County to the Oregon border. As a result of the fires in the Sonoma County region, the REFB has seen an increased need for food and nutrition. In response, they provide critical food to those impacted by the fires and work to ensure that those who needed assistance before the fires are still able to access help.

In addition to the food and funds donations, nearly 40 Sonoma Raceway employees volunteered at the REFB warehouse in Santa Rosa on Nov. 16, processing more than 17,000 pounds of food, which equates to 14,750 meals.

“The entire team at the Redwood Empire Food Bank is inspired by the community spirit and tremendous support we receive from Sonoma Raceway,” said Jen Oberti, REFB corporate and community relations manager.  “In addition to hosting an annual food drive for the REFB, their employee volunteer day provided much needed help in our warehouse, where their team helped glean, sort and pack over food for our neighbors in need.”

 

For more information about Sonoma Raceway community events, call 800-870-RACE (7223) or visit SonomaRaceway.com.

Midstate Construction Completes Santa Rosa Charter School for the Arts Project

Midstate Construction Corporation recently completed new construction of Santa Rosa Charter School for the Arts, a K-8 public school in Santa Rosa, CA.

Designed by Quattrocchi Kwok Architects, this project included demolition of existing structures and new construction of a K-8 public school campus with one story and two story classroom buildings which include a black box theater, dance studio and music auditorium.

This project received the North Bay Business Journal’s 2016 Top Projects Award.

Sonoma Raceway Kicks Off 17th Annual Thanksgiving Food Drive

Sonoma Raceway kicks off its season of giving with the 17th annual Thanksgiving Food Drive, which runs through Nov. 19.

Race fans and community members can donate at several North Bay locations, and all food collected through the drive will be distributed to two Sonoma County food banks: Friends in Sonoma Helping (FISH) and the Redwood Empire Food Bank in Santa Rosa

Sonoma Raceway’s annual food drive has generated nearly 34 tons of food over the past 16 years, including more than 1,600 pounds in 2016.

New for this year, non-perishable food items can be brought to the Sonoma Valley Fire Department, as well as the Schell-Vista Fire Department. In addition, Sonoma Raceway will collect both food and new, unwrapped toys at Sonoma Valley Fire and Rescue Authority’s Open House and pancake breakfast at the Albert C. Mazza Fire Station on Sunday, Nov. 12, between 9 a.m.-1 p.m.

Non-perishable food items for the Sonoma Raceway Thanksgiving Food Drive can be dropped off at the following locations:

  • Sonoma Raceway (at Gate 1 or the main office), 29355 Arnold Dr., 800-870-RACE (7223), www.SonomaRaceway.com.
  • Sonoma Valley Fire & Rescue Authority, 630 2nd Street West, Sonoma. (707) 996-2102, www.svfra.org.
  • Schell-Vista Fire Station,  22950 Broadway, Sonoma, CA 95476, (707) 938-2633, www.schellvistafire.com
  • Sonoma Market, 500 West Napa Street, Sonoma, 707-996-3411, www.sonoma-glenellenmkt.com
  • Glen Ellen Village Market, 13751 Arnold Dr., Glen Ellen, 707-996-6728, www.sonoma-glenellenmkt.com
  • RKA, 7694 Bell Road, Windsor, (707) 836-7659, www.rka-luggage.com.
  • Food items or donations can also be mailed to the raceway: Jen Imbimbo, 29355 Arnold Drive, Sonoma, CA, 95476.

RKA Motorcycle Luggage will continue its annual tradition of hosting a motorcycle group ride on Saturday, Nov. 11, which will include a pit stop at Sonoma Raceway to drop off collected food items and take a few laps around the Sonoma Raceway road course. For more information and to sign up for the ride, visit www.rka-luggage.com and click on the event schedule tab.

In addition to its regularly scheduled food distribution, REFB is distributing thousands of pounds of food to evacuation centers, shelters and other community resources helping fire victims.  The food bank has also implemented Station 3990, a drive-through, free grocery distribution at its warehouse and select other locations to help those impacted by the fires.

In the first 12 days after the fires began, REFB distributed 950,000 pounds of food: 114,000 pounds went to partner organizations, 281,000 pounds was distributed through REFB programs and a staggering 533,000 pounds (57%) was moved through Station 3990, car load by car load. The donations poured in during the first two weeks after the fires but are now beginning to dwindle. However, the number of people turning to them for help continues to increase. The capacity to continue to provide emergency food to the fire victims depends on donations to food and funds drives.

FISH (www.friendsinsonomahelping.org) has been helping Sonoma Valley residents in times of need since 1970 and provides not only food, but clothing, transportation and emergency rental assistance to Sonoma Valley residents in need. FISH has been a beneficiary of the raceway’s food drive since its inception in 2001.

For more information on the food drive, visit www.SonomaRaceway.com or contact Jen Imbimbo at Jennifer@SonomaRaceway.com or 707-933-3981.

Sonoma State University’s New Public-Private Partnership Bolsters Classroom-to-Career Pipeline

The School of Business and Economics (SBE) at Sonoma State University (SSU) today announced a strategic partnership and career services pilot program with Star Staffing, a leading regional employment agency with nearly 20 years of experience in employer relations and job search training. Beginning this month, the strategic public-private partnership will provide enhanced career support to business and economics students, alumni, and employers, including the development of existing programs, such as annual career fairs, alumni mixers, and the professional mentor program.

“This pilot program is intended to build on our progress and engage even more companies and create more opportunities for our students nationally and globally,” Dr. William S. Silver, Dean of the School of Business and Economics and Wine Business Institute said. “It allows us to continue creating extraordinary learning experiences for our students, while further developing a portfolio of career services in support of regional economic development and recovery. Our goal and expectation is that these enhancements will help ensure the educational, career, and life success of our students and alumni.”

“We’re excited about the opportunity to help students gain career-readiness skills and employment,” Nicole Smartt, Star Staffing Vice President said. “Employers across all industries are having difficulty finding enough qualified employees. By turning a spotlight on these talented students, we’ll help local employers see the value of hiring from this source and alleviate the hiring headache.”

Star Staffing will oversee the SBE Career Center’s services to students and employers, while working closely with Dr. Kyuho Lee, Professor of Marketing and current Internship Director; Dr. Karen Thompson, SBE Managing Director; and Joe Standridge, Professional Faculty in Accounting and advisor to the Accounting Forum, the largest academic student club at SSU. Currently, the SBE Career Center connects students to approximately 300 internships and nearly 100 professional mentorships each year, and the Accounting Forum helps place more than 100 students in accounting positions annually.

For more information regarding SBE Career Center services, please contact 707-791-5565 or sbe.careers@sonoma.edu. For information regarding the new public-private partnership with Star Staffing, or for information about SBE programs and degrees, please call 707-664-2377 or visit www.sonoma.edu/sbe.

BioMarin Highlights Breadth of Innovative Development Pipeline at R&D Day on October 18th in New York

BioMarin Pharmaceutical Inc. (NASDAQ: BMRN) updated the investment community on the Company’s development portfolio, which is focused on innovative therapies to treat rare and ultra-rare diseases.

BioMarin Pharmaceutical logo (PRNewsfoto/BioMarin Pharmaceutical Inc.)

“We are pleased to share the progress of our development programs in therapies to treat rare genetic diseases; hemophilia A, PKU, achondroplasia and our next IND into Friedreich’s Ataxia,” said Hank Fuchs, M.D., President Worldwide Research and Development of BioMarin.  “In the near term, we are expecting an FDA decision on pegvaliase to treat adults with uncontrolled PKU in the first half of next year, and we continue to be rapidly and decisively developing the potential first gene therapy for severe hemophilia A.”

BioMarin Selects BMN 290 for Friedreich’s Ataxia

BioMarin announced today that it has selected BMN 290, a selective chromatin modulation therapy, for the treatment of Friedreich’s Ataxia (FA).  FA is a rare autosomal recessive disorder with worldwide prevalence of approximately 15,000, which results in disabling neurologic and cardiac progressive decline.  Currently there are no approved disease modifying therapies for FA.  In preclinical models, BMN 290 increases frataxin expression in affected tissues more than two-fold.  BMN 290 is a second generation compound derived from a compound acquired from Repligen that had human clinical data demonstrating increases in frataxin in FA patients.  BMN 290 was selected for its favorable penetration into the central nervous system and cardiac target tissues, and its preservation of the selectivity of the original Repligen compound.  The company expects to submit the IND in 2H 2018.

BMN 270 Gene Therapy for Severe Hemophilia A

BioMarin announced today that the FDA has completed their review of the IND application for BMN 270, an investigational gene therapy treatment for severe hemophilia A, and concluded that it can proceed.  The IND application included 52-week data at the 6e13 vg/kg dose and the protocol for the Phase 3 study using the 6e13 vg/kg dose.  The protocol for the second Phase 3 study using the 4e13 vg/kg dose has also been submitted to the FDA.

BioMarin also announced today that the Phase 3 Clinical Trial Application was approved by the UK Medicines and Healthcare Products Regulatory Agency (MHRA).

The company expects to initiate the global Phase 3 program in the fourth quarter of 2017.

Data Update on Phase 1/2 Study of 4e13 vg/kg Dose

In addition, the company provided an update on the ongoing open-label Phase 1/2 study of the 4e13 vg/kg dose at up to 36 weeks of observation at the September 14, 2017 data cut.  Since the last data update provided during the Q2 earnings call on August 2, 2017, five of the six patients at the 4e13 vg/kg dose tracked to the low range of normal, and the sixth is in the mild range for Factor VIII levels.  Median annualized bleed and factor VIII use rates for 4e13 and 6e13 vg/kg were zero after Week 4.

Factor VIII Levels (%) of 4e13 vg/kg Dose Patients* by Visit (N=6)

Week**

4

8

12

16

20

24

28

32

36

4e13 vg/kg
Dose

n

6

6

6

6

6

6

6

3

3

Median
Factor VIII
Level*** (%)

4

15

21

29

34

28

31

51

45

Mean
Factor VIII
Level*** (%)

5

13

19

26

31

29

30

51

47

Range
(low, high)

(2,10)

(3,21)

(6,32)

(5,38)

(7,45)

(7,43)

(4,44)

(48,54)

(41,55)

*All patients had severe hemophilia A, defined as less than or equal to 1% of Factor VIII activity levels, expressed as a percentage of normal factor activity in blood.

**Weeks were windowed by +/- 2 weeks

*** Bolded numbers are in the mild to normal range of Factor VIII activity as defined by the World Federation of Hemophilia, http://www.wfh.org/en/page.aspx?pid=643 (link current as of Oct. 17, 2017). Factor VIII levels are determined by one-stage assay.

 

BMN 270 Reduces Bleeds and Factor VIII Use:  Summary of Mean Annualized Bleeding Rate (ABR) and FVIII Use Rate of 4e13 vg/kg Dose for Patients Previously on Prophylaxis (N=6) at September 14, 2017 data cut

Before BMN 270 Infusion

After BMN 270 Infusion

Median (mean, SD)

Median (mean, SD)

Annualized Bleeding Rate*
(bleeding episodes per
year per subject)

8.0 (12.2, 15.4)

0.0 (0.8, 1.9)

Annualized FVIII Use Rate*
(infusions per year per subject)

155.5 (146.5, 41.6)

0.0 (2.7, 6.7)

*Post-infusion data were based on data after Week 4

BMN 270 Generic Name is Valoctocogene Roxaparvovec

BioMarin was issued the International Nonproprietary Name (INN) valoctocogene roxaparvovec for BMN 270.  The World Health Organization (WHO) has approved the INN “valoctocogene roxaparvovec” for the Company’s gene therapy to treat hemophilia A.  International Nonproprietary Names (INN) identify pharmaceutical substances or active pharmaceutical ingredients. Each INN is a unique name that is globally recognized and is public property. A nonproprietary name is also known as a generic name.

Gene Therapy Manufacturing

BioMarin has constructed one of the largest gene therapy manufacturing facilities in the world, which is located in Novato, California.  Good Manufacturing Practices (GMP) production of BMN 270 has commenced and will support clinical development activities and anticipated commercial demand. This facility is capable of supporting approximately 2,000 patients per year, and the production process was developed in accordance with International Conference on Harmonisation guidance for Pharmaceuticals for Human Use facilitating worldwide registration with health authorities.

Vosoritide Data Update

BioMarin provided an update on its open-label Phase 2 study of vosoritide, an analog of C-type Natriuretic Peptide (CNP), in children with achondroplasia, the most common form of disproportionate short stature in humans.

Vosoritide for achondroplasia demonstrates sustained increase in average growth velocity over 30 months of treatment in 10 children, who completed 30 months of daily dosing at 15 µg/kg/day.  Over this period of time, patients have experienced mean absolute growth increase of approximately 4 cm over what their baseline growth velocity would have predicted.

The sustained increase in annualized growth velocity was accompanied by sustained improvements over time in height compared to age- and gender-matched unaffected children as measure by z-scores.  In addition, treatment with vosoritide shows continued improvement over time in proportionality as measured by a ratio of the upper and lower body measurements or U/L ratio.

Pegvaliase Program Update

The Pegvaliase Biologics License Application (BLA) remains on Track for FDA Action during the first half of 2018.  The company plans to submit a Marketing Authorization Application (MAA) to the European Medicines Agency (EMA) in Q1 2018.

2017 Full-year Total Revenue and Non-GAAP Guidance reaffirmed

Today, the Company commented on their Total Product Revenue and Non-GAAP trends for the third quarter and full-year 2017.  In terms of the overall commercial business, BioMarin stated that sales of products in markets throughout most of the world are performing at or above internal expectations.  However, the Company said the one exception is Brazil, where a slowdown in Federal purchasing orders has extended into the third quarter of this year.  As a result, third quarter revenues are expected to be negatively impacted.  For the fourth quarter, if orders are placed in Brazil as expected, Total Product Revenue for full-year 2017 is anticipated to be in the mid-point of guidance.  However, if sales to Brazil continue to be slow in the fourth quarter, full-year 2017 Total Product Revenue may be at the low end of guidance.  Regardless of Brazilian ordering patterns for the remainder of the year, and based on careful expense control, the Company still expects to be in the mid to high-end of Non-GAAP profitability guidance for full-year 2017.

About BioMarin and Disease Information

BioMarin is a global biotechnology company that develops and commercializes innovative therapies for patients with serious and life-threatening rare and ultra-rare genetic diseases. The company’s portfolio consists of six commercialized products and multiple clinical and pre-clinical product candidates. For additional information, please visit www.biomarin.com. Information on BioMarin’s website is not incorporated by reference into this press release.

About Friedreich’s Ataxia

Friedreich’s ataxia (FA) is a progressive, neurological disorder that affects approximately 15,000 people in the United States and Europe, typically resulting in wheelchair dependence in young adulthood and early death due to cardiac failure. It is caused by mutations in the FXN gene, and is inherited in an autosomal recessive manner. FXN mutations result in reduced expression of frataxin protein, manifesting in progressive neurological and cardiac damage. Major neurological symptoms include muscle weakness and ataxia, a loss of balance and coordination. These symptoms typically appear between 10 and 15 years of age, but FA has been diagnosed in people from ages 2 to 50 with earlier onset associated with a more severe course.

BMN 270 Safety

Overall, BMN 270 has been well-tolerated by patients across all doses, including the two patients that received the lowest doses of 6e12 and 2e13 vg/kg, respectively.  No patients developed inhibitors to Factor VIII and no patients withdrew from the study.  The most common adverse events (AEs) across all dose cohorts were alanine aminotransferase (ALT) elevation (11 patients, 73%); arthralgia, aspartate aminotransferase elevation, and headache (7 patients each, 47%); back pain and fatigue (5 patients each, 33%).  Two patients reported Serious Adverse Events (SAEs) during the study.  One patient was hospitalized for observation after developing Grade 2 pyrexia with myalgia and headache within 24 hours of receiving BMN 270.  The event resolved within 48 hours following treatment with paracetamol, an over-the-counter treatment for pain and fever.  The event was assessed as related to BMN 270.  The other SAE was assessed as not related to BMN 270, attributed to a planned knee surgery to treat hemophilic arthropathy, and Grade 1 in severity.  No complications were reported.

About Hemophilia A

Hemophilia A, also called Factor VIII (FVIII) deficiency or classic hemophilia, is a genetic disorder caused by missing or defective Factor VIII, a clotting protein. Although it is passed down from parents to children, about 1/3 of cases are caused by a spontaneous mutation, a new mutation that was not inherited. As an X-linked disorder, hemophilia A mostly affects males, occurring in approximately 1 in 5,000 male births. People living with the disease are not able to form blood clots efficiently and are at risk for excessive bleeding from modest injuries, potentially endangering their life. People with severe hemophilia often bleed spontaneously into their muscles or joints. The standard of care for the 43% of hemophilia A patients who are severely affected, is a prophylactic regimen of Factor VIII infusions three times per week. Even with prophylactic regimens, many patients still experience microbleeds and spontaneous bleeding events that result in progressive joint damage.

Vosoritide Safety

Vosoritide was generally well tolerated at all doses. The majority of adverse events (AEs) were mild and no serious AEs were reported as study drug-related. Across all doses, injection site reactions and hypotension were the most common drug-related AEs.  All injection site reaction events were transient. AEs of hypotension were mild, transient and resolved without medical intervention, and the majority were asymptomatic and reported in context of routine blood pressure measurements. No new safety findings were observed at the 30 µg/kg/day dose.

About Achondroplasia 

Achondroplasia, the most common form of disproportionate short stature in humans, is characterized by failure of normal conversion of cartilage into bone, which results in disproportionate short stature. This condition is caused by a mutation in the fibroblast growth factor receptor 3 gene (FGFR3), a negative regulator of bone growth. Beyond disproportionate short stature, people with achondroplasia can experience serious health complications, including foramen magnum compression, sleep apnea, bowed legs, mid-face hypoplasia, permanent sway of the lower back, spinal stenosis and recurrent ear infections. Some of these complications can result in invasive surgeries such as spinal cord decompression and straightening of bowed legs. In addition, studies show increased mortality at every age.

More than 80% of children with achondroplasia have parents of average stature and have the condition as the result of a spontaneous gene mutation.  The worldwide incidence rate of achondroplasia is about one in 25,000 live births.  Vosoritide is being tested in children whose growth plates are still “open,” typically those under 18 years of age.  This is approximately 25 percent of people with achondroplasia.  In the United StatesEuropeLatin Americaand the Middle East, there is currently no licensed medicines for achondroplasia.

Pegvaliase Safety

The safety data set for all pegvaliase trials includes exposure up to seven years and approximately 680 patient years of treatment, 300 of which are from the Phase 3 program.  Most Adverse Events (AEs) were mild or moderate in severity.  8.5% of patients reported AEs leading to study withdrawal.  The most common Adverse Events (AEs) were arthralgia (69.5%), injection site reaction (65.1%), headache (51.9%), nasopharyngitis (41.1%), and rash (40.2%).

Pegvaliase treated patients had acute systemic hypersensitivity adverse events (4.6%) as defined by the broad National Institute of Allergy and Infectious Disease and the Food Allergy and Anaphylaxis Network (NIAID/FAAN) (Sampson’s) criteria for anaphylaxis by expert external adjudication.  Hypersensitivity adverse events and acute systemic hypersensitivity events mainly occurred in the first year of treatment.  With prolonged exposure, AE rates declined for almost all categories.  Using high-sensitivity assays, anti-drug IgE was undetected in the observed acute systemic hypersensitivity events.  Eight of the 13 patients with acute systemic hypersensitivity events were re-dosed and six of the eight re-dosed patients continued therapy.

About Phenylketonuria

Phenylketonuria (PKU) is a genetic disorder affecting approximately 50,000 diagnosed patients in the developed world and is caused by a deficiency of the enzyme PAH.  This enzyme is required for the metabolism of Phe, an essential amino acid found in most protein-containing foods. If the active enzyme is not present in sufficient quantities, Phe accumulates to abnormally high levels in the blood and becomes toxic to the brain, resulting in a variety of complications including severe intellectual disability, seizures, tremors, behavioral problems and psychiatric symptoms. As a result of newborn screening efforts implemented in the 1960s and early 1970s, virtually all individuals with PKU or PAH deficiency under the age of 40 in developed countries are diagnosed at birth and treatment is implemented soon after. PAH deficiency can be managed with a Phe-restricted diet, which is supplemented by low-protein modified foods and Phe-free medical foods; however, the strict diet is difficult for most adult patients to adhere to the extent needed for achieving adequate control of blood Phe levels.  To learn more about PKU and PAH deficiency, please visit www.PKU.com. Information on this website is not incorporated by reference into this press release.

Forward Looking Statement

This press release contains forward-looking statements about the business prospects of BioMarin Pharmaceutical Inc., including, without limitation, statements about its development programs and regulatory actions related to these programs, including the expected timing of the filing of the MAA for pegvaliase and an FDA decision on pegvaliase, the timing of its anticipated submission of an IND for BMN 290, BioMarin’s BMN 270 program generally, the timing of the initiation of the global Phase 3 program, the expected design and size of the Phase 3 studies and a Phase 1/2 study in subjects with pre-existing antibodies against AAV5, expected regulatory actions related to BMN 270, the expectations of total BioMarin revenues for the third quarter and full year 2017 and the financial performance of BioMarin as a whole, and statements about the anticipated capacity of the Company’s gene therapy manufacturing facility.  These forward-looking statements are predictions and involve risks and uncertainties such that actual results may differ materially from these statements. These risks and uncertainties include, among others: results and timing of current and planned preclinical studies and clinical trials of our product candidates, the continued clinical experiences of the patients in the current clinical studies; the content and timing of decisions by the U.S. Food and Drug Administration, the European Commission and other regulatory authorities; the content and timing of decisions by local and central ethics committees regarding the clinical trials; our ability to successfully manufacture our product candidates for the preclinical and clinical trials; the ordering patterns for our commercial products, particularly orders from Brazilian governmental entities; and those other risks detailed from time to time under the caption “Risk Factors” and elsewhere in BioMarin’s Securities and Exchange Commission (SEC) filings, including BioMarin’s Quarterly Report on Form 10-Q for the quarter ended June 30, 2017, and future filings and reports by BioMarin. BioMarin undertakes no duty or obligation to update any forward-looking statements contained in this press release as a result of new information, future events or changes in its expectations.

Sonoma County manufacturers reach out to students on industry’s career appeal

Local manufacturing facilities recently opened their doors to 40 students from Cloverdale, Windsor, Santa Rosa and Rancho Cotate high schools as part of an effort to showcase the range of local career opportunities available in manufacturing.

On Oct. 6, students toured facilities at Viavi Solutions and Amy’s Kitchen, explored the makerspace and machining shop at Santa Rosa Junior College, engaged with industry professionals in career pathway discussions, and took part in hands-on learning activities at 180 Studios, the new community makerspace in Santa Rosa.

Manufacturing Day brought together education and industry leaders to inspire the next generation of Sonoma County manufacturers and to raise public awareness of the diversity and economic importance of local manufacturers.

“The manufacturers of Sonoma County provide a wide range of employment and career opportunities to its residents,” said Bill Oakes, chair of the North Bay Society of Manufacturing Engineers, one of the event’s leading partners. “A vibrant and growing manufacturing sector within the North Bay provides a healthier standard of living for people of all educational backgrounds.”

Other partners included Medtronic, CamelBak, Six Sigma, Straus Family Creamery, Keysight Technologies, Small Precision Tools, P & L Specialties, EMG Pickups, Max Machinery, and the North Bay Leadership Council.

Manufacturing Day is an annual national event executed at the local level and supported by thousands of manufacturers as they host students, teachers, parents, job seekers and other local community members at open houses, plant tours and presentations designed to showcase modern manufacturing technology and careers.

2017 MFG Day Sonoma County was launched by the Sonoma County Office of Education, Career Technical Education Foundation, and Sonoma County Economic Development Board.

Comcast Gives a $500,000 Cash Donation to North Bay Fire Relief Fund

Comcast NBCUniversal today announced it will donate $500,000 in cash to the North Bay Fire Relief Fund, and almost $1 million worth of in-kind support for the affected communities.

Comcast will produce advertising spots and donate advertising time on its cable systems to build awareness and solicit donations to the North Bay Fire Relief Fund.  The Fund has been established to provide much needed assistance to residents affected by the North Bay Fires in Northern California.

“These fires are the most deadly and devastating in California’s history and our hearts go out to all of who have been impacted,” said Dave Watson, Senior Executive Vice President of Comcast Corporation and President and CEO of Comcast Cable. “With this contribution to the fire relief efforts, Comcast is supporting the communities we serve and providing aid in the rapid recovery and restoration of the affected areas.”

In addition to opening up its Xfinity Wi-Fi hotspots throughout the Northern California region, Comcast is also providing a range of Internet, Wi-Fi, Wireless, Phone and Video services to evacuation/community support centers throughout the area, installing TVs with video services at evacuation centers and donating technology services to numerous Emergency Response and Fire Command centers.

Throughout the areas impacted by the fires, Comcast is working in partnership with local authorities, as well as utility and other infrastructure and service providers to ensure that service restoration efforts can take place quickly, while working within safety guidelines and other incident response protocols.

For more information about our overall response and support during the Northern California wildfires, visit http://california.comcast.com/.

Sonoma State University Starts #Nomacares Intiative

hen the recent fires struck Sonoma County, thousands of students, faculty and staff were personally affected. Many were forced out of their homes, including students on campus who were temporarily relocated from the residence halls. Others lost loved ones, were injured or had their houses burned. Nearly all of us know someone who was struck by this tragedy.

Now we’re asking Noma Nation to help.

Get involved by donating to a special fund that will provide direct assistance to the many victims at Sonoma State University. You can also find ways to help through the Noma Needs Facebook group, by providing things like rides, temporary housing, or other assistance.

The fires knocked us down. But we’re back up, and we’re working hard to help those who need it.

#NomaCares

See below for a list of FAQs, latest updates, resources and other ways to get involved.