April 2023                                                                            Volume 34, Issue 4

Receiving gifts, no matter the value or occasion, is most often meaningful. The giver expresses appreciation, and the receiver reciprocates. Both feel good when the exchange is made. At BDSRA, we love receiving gifts in the mail and sharing our appreciation, but sometimes we cannot reciprocate. In Betty Wetherbee’s case, we want to share our appreciation with our BDSRA community.

Betty Wetherbee grew up in a large family but was never able to have children of her own. She was close with her sister and treated her sister’s children as her own. When Betty’s sister became a grandmother, Betty enjoyed the same benefits. Betty’s niece, Judy Dahl, gave birth to the first grandchild, David, her great nephew.

Betty remained close with her family, celebrating college graduations, weddings, and births. She also celebrated the birth of her niece Judy’s first grandchild, her great, great nephew and son to Dave and Corrina, Clifford. She kept in touch with Dave and his family even after his mother passed away. It was shortly thereafter that Clifford’s vision became a severe problem and a swift diagnosis of Batten disease (CLN3).

In the world of philanthropy, Betty wasn’t a close friend to BDSRA but was clearly made aware of us through her great, great nephew’s battle with Batten disease. She had given to us in the past, but her biggest gift was yet to come.

Just after Christmas 2022, BDSRA received a six-figure gift from Betty for support and research of Batten disease. We want to express an abundance of appreciation for the impact this gift will have on our Batten community because we weren’t able to tell Betty ourselves. We’ll share more about Betty her husband Joe, and their legacy in our Impact Report coming this fall.

Bequests of every value are meaningful to BDSRA, and we would like to express our appreciation while you are living! If you or someone you know is making a will or estate plans, and BDSRA is included in any way, please share that information with us. A form on our website will soon be available to send. If we can help you with the planning, let us know.

‘Tis better to give than to receive, but to express gratitude as well makes the exchange even sweeter.

Amy Fenton Parker, BDSRA President & CEO

In-person and virtual registration are open for this year’s BDSRA Annual Family Conference!

The conference will take place in Denton, Texas From July 14-16.

Click the button below for information on registration, sponsorship information, FAQs, travel accommodations, and more.

Come “Give Batten the Boot” with us!

Because of forward-thinking and generous donors who lost their children to Batten disease, each year, there is a limited amount of stipend funding available for families based in the US or Canada who need assistance to attend the annual BDSRA Conference.

Priority is given to families who have not received funding in the past for the conference, new families, and those who have been unable to attend for several years due to financial stress.

Click the button below to apply.

Join us as we celebrate families around the world and raise awareness and funds! BDSRA is hosting the 2023 International Batten Disease Awareness Day Virtual 5K on Friday, June 9th, 2023.

Our goal is to honor and support those affected by Batten disease and to raise awareness and funds so BDSRA can continue to advocate for all CLN types.

Sign up, join a team, and raise funds today! Deadline for T-shirt orders is before midnight on Thursday, May 11 (limited to U.S. families only).

We are halfway towards our $10,000 goal!

Watch the tutorial below for details on how to register and click the button below to visit the official event page.

We’d like to take a moment and thank our wonderful Batten community for their participation in the International Batten Disease Awareness Day T-shirt contest. 

Design submissions doubled during the final weekend prior to the deadline!

The next step in this contest is narrowing the submissions down to three finalists, which BDSRA will do in the coming days. From there, public voting will take place from May 3-7 and the winner will be announced on Wednesday, May 10th.

Don’t forget the deadline to order this year’s International Batten Disease Awareness Day T-shirts is Thursday, May 11th.

The power of advocacy can be defined by Tracy VanHoutan’s work.

VanHoutan, husband to Jennifer and father to Noah, Laine, Emily, and Colette, was described by former BDSRA Executive Director Margie Frazier as a “driving force” behind Brineura, described by its manufacturer BioMarin as, “indicated 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.”

It’s the first enzyme replacement therapy (ERT) to be directly administered into the fluid of the brain and became FDA-approved six years ago. It’s the first and so far, only FDA-approved treatment for any form of Batten disease.

Suzette James, a BDSRA Foundation Board of Directors member and late-onset CLN2 Batten mother to Maya and Xavier, recently served as a panelist for the FDA’s Center for Biologics Evaluation and Research (CBER) Office of Therapeutic Products (OTP) public virtual workshop titled, “Clinical Trials: The Patient Experience.”

In this Q&A, James recaps her presentation and unveils her advocacy plans moving forward.

Libby and Steve Stenzel were married on December 22, 2022. 

In lieu of wedding gifts, the couple asked for contributions to BDSRA Foundation. Their friends and family delivered by donating a total of $1,560.60 to BDSRA for research. 

Libby is a mother to Anna Weber, a 12-year-old who has CLN3 Batten disease. Weber plays drums in the band and sports, including volleyball and track & field. 

“Steve and I met two years ago. Steve understood the complexity of Anna’s disease. He knew of a young man who was diagnosed with Batten and had passed approximately 10 years earlier,” Libby wrote in a letter to BDSRA.

“Knowing how utterly drastic and then completely devastating this disease can be, Steve promised to research, love, and support Anna, myself, Anna’s brothers Ethan and Aricin, Anna’s sister Sophie, and Anna’s niece Stella through all of the emotional trials and success this disease will bring.” 

BDSRA thanks the Stenzel family and donors for investing in its mission and for their gratitude.

The 2023 BDSRA Annual Hybrid Family Conference will take place from July 14-16 at the Embassy Suites by Hilton Denton Convention Center in Denton, Texas! Book a room by clicking the button below!

Follow our website and social media channels for both in-person and remote registration information this spring.

Click below to read the letters BDSRA signed on to in April.

• Support for Texas SB 1408 – to establish a Rare Disease Advisory Committee in Texas
• Support HF 2261, which would prevent Medicaid in Minnesota from creating overly restrictive coverage criteria for newly approved rare disease therapies
• Support for  H.B. 2478 and S.B. 1697, Texas Newborn Screening Legislation
• Support of FY 2024 appropriations request letter for CDC

Batten families of all CLN types need medical equipment to support their journey. Covering the cost is so often a struggle, but BDSRA can help.

In recognition of Earth Day in April, BDSRA featured the Palermo Family’s story of how they benefitted from BDSRA’s Equipment Exchange Program. The family received a wheelchair for daughter Amelia, CLN1, and gifted orthotic equipment to a fellow Batten family.

Read more about their story in the images above and below this text and click on the button below to learn more about the program and apply.

The BDSRA Foundation is committed to supporting and advocating for those affected by Batten disease to raise awareness and give this community a platform to share their stories.

Sharing your story allows BDSRA to help raise awareness for Batten disease and advocate for improvements and developments for our three pillars — Support, Research, and Advocacy.

Please contact BDSRA Foundation Marketing & PR Coordinator Patrick Kotnik at patrick@bdsrafoundation.org if you’re interested in sharing your story with BDSRA.

Shared your story with local media? Let us know! Email Patrick and we will share!

In case you haven’t heard, BDSRA Foundation has moved offices!

With that comes a new address to send mail to BDSRA. For any mail and shipments to BDSRA, please use the address below. 

P.O. Box 30049
Columbus, OH 43230

Do You Follow BDSRA on Social Media?

Want to see more BDSRA content? Don’t miss out on upcoming events, information, campaigns, and community posts! Follow @BDSRA on Facebook, Instagram, LinkedIn, Twitter, and YouTube to stay in the know on all things happening in our Batten Community!

Click below to follow all of our accounts: 

Courageous Parents Network, a non-profit organization and educational platform that orients, empowers, and accompanies families and providers caring for children with serious illnesses, will host another virtual workshop on Wednesday, May 17th featuring the art of storytelling.

Shaping and telling a powerful story takes time, work, and practice. Join CPN’s team of Bloggers in Residence, author Ann Schrooten, and podcaster Effie Parks to explore the When, Who, What, Why, and How of finding and telling your story.

This is a great opportunity to help your community harness their own story-telling skills to understand their journey and/or to benefit others in their community.

This workshop is free to attend and will take place on Zoom.

Attention Medicaid and CHIP consumers, renewals are coming back! Many were paused due to COVID-19.

Be sure you get your renewal letter by making sure your state has your current mailing address NOW.

Research Updates from Industry Partners

Visit the Research tab on our website to keep up with new and investigational studies! bdsrafoundation.org/research/clinical-studies/ 

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April 2023 Facebook Fundraiser Spotlight:

Thank you for sharing and supporting our mission on social media! You can visit each fundraiser by clicking on the names above. Thank you!

In Loving Memory 

Remember those we have lost to Batten disease. We work daily to build a brighter future for families in their honor and memory.

Brody Bradley Koslowski | July 30, 2015 – March 25, 2023

Samuel Russell Engman | September 24, 2011 – April 25, 2023

To have your loved one’s name placed in The Illuminator please email info@bdsrafoundation.org.

Thank You BDSRA Community of Donors!

Thank you for investing in our mission. We exist to provide support, research, and advocacy for families with all forms of Batten disease. We want to acknowledge the donors who gave so generously last month to our work. Together, we are Batten Advocates for a Cure.

To view the list of recent donors, please click the button below!

We connect your donation to Support, Research, and Advocacy for families of all CLN types.

BDSRA Foundation

P.O. Box 30049
Columbus, OH 43230

(800) 448-4570 | info@bdsrafoundation.org | www.bdsrafoundation.org

View The Illuminator online