Rare Reflections
The official blog of Noah’s Hope
Giving Tuesday 2023
This GivingTuesday, Noah’s Hope-Hope 4 Bridget is raising money to support Batten disease research. Our goal is to raise $10,000 —will you help?
Greetings!
As we head into the holiday season, we hope you are enjoying time with family and friends. It is a time to appreciate our fortune and hope for better times for those who are having challenging ones.
Please consider supporting Batten disease research through Noah’s Hope-Hope 4 Bridget by donating to our GivingTuesday campaign. You’ll be doing more than just donating—your kindness will make it possible as we we continue our fight against CLN2 Batten disease for children like ours: Noah, Laine and Bridget.
CLN2 Batten disease is a rare, fatal, inherited disorder of the nervous system that typically begins in early childhood. Unfortunately, there is currently no cure for Batten disease.
The Noah’s Hope-Hope4Bridget foundation is dedicated to raising awareness and funds for research of CLN2 Batten disease. We believe that every child deserves a chance at exploring life while living with Batten disease.
While we are grateful for the enzyme replacement therapy (ERT) treatment, we continue to searching for additional treatments and a CURE for CLN2 Batten disease.
Current Projects funded by NH-H4B
- GOLD Sponsor: 18TH Int’l Congress of Neuronal Ceroid Lipofuscinoses
- Rush Chicago – Pediatric Neurology Translational Neuroscience
Sanford at the Weimer Lab
- “Explore GPI and metabolome as biomarkers of CLN2R208X disease course”
- “Determine short term efficacy of CLN2 base editors in CLN2R208X pigs”
Washington University at the Cooper Lab
- “Combined Treatment of Central and Somatic CLN2 Disease in Mice”
With your generous support, we can help make a difference in the lives of these brave children and their families.
Peace & hope to your family this holiday season!
With gratitude,
Sara & Dave Kennicott
Jen & Tracy VanHoutan
Everyone can have an impact on #GivingTuesday!
Donate online or checks may be mailed to:
NH-H4B
P.O. Box 54
Downers Grove, IL 60515
Visit Noah’s Hope Online | Visit Hope 4 Bridget Online
Noah’s Hope – Hope 4 Bridget is a 501(c)(3) registered charity supporting Batten disease research and recognized by the IRS.
2022 NH-H4B Annual Update
Greetings!
We hope you and your family are well! We wanted to share a few updates with you about our mission for CLN2 Batten disease.
With generous donations from you, our sponsors, and supporters, we have been able to fund over $2 million to scientists and research initiatives since 2009. Our projects led to meaningful discoveries in the Batten community. Please consider a donation today.
A Fifth Season
Last November, we hosted the 10th year of celebrating our children’s lives and continued fundraising for Batten disease at A Fifth Season. While it has been such a fun event, we have started working on a different type of event for 2023. Noah’s Hope-Hope 4 Bridget’s mission remains steadfast in funding early detection methods, innovative research to advance treatments, and to ultimately find a cure for CLN2 Batten disease. We will share a save-the-date for A Fifth Season early in 2023.
Project Updates
We are pleased to share that in September, A novel porcine model of CLN2 disease that recapitulates patient phenotypes publication was released. This project was one of our largest projects that we expect will help treatment development for CLN2 disease.
On November 3-4, we sponsored & attended the 7th TranslationalResearch Conference for the Management of NCLs (Batten disease). This meeting began in 2010 and was focused entirely on treatment approaches for Batten disease CLN1 and CLN2. This conference was started by several “family foundations”, including Noah’s Hope-Hope 4 Bridget. This year the program evolved to include all forms of Batten and provided a forum to spark critical ideas, conversations, and further research for this devastating disease with academia, the biotech industry, and advocacy groups. During the conference, we heard updates from NHH4B projects:
- Heather Adams, Ph.D.; University of Rochester, “Characterizing sleep dysfunction in individuals with NCL Disorders.”
- Elizabeth Berry-Kravis, MD, Ph.D.; Rush University Medical Center, “Challenges to Translation of Disease-Targeted Therapies in Rare Neurogenetic Diseases”
- Jonathan Cooper, Ph.D.; Washington University in St. Louis,“Defining and treating disease outside the brain”
Treatment, not a cure
In 2017, an enzyme replacement therapy (ERT) for CLN2 Batten disease, Brineura was approved. While grateful for this treatment, Noah’s Hope – Hope 4 Bridget continues searching for additional treatments and a CURE for CLN2 Batten disease. We are always exploring new funding opportunities and research projects.
Our inspiration
We keep our children, Noah, Bridget, and Laine close with the work we do for other children around the world who are living with Batten disease. On behalf of our children, thank you for your kind donation and continued support to Noah’s Hope-Hope 4 Bridget, a non-profit 501©(3) organization – Federal Tax ID #47-2609776.
Yours in hope,
Jen & Tracy VanHoutan (Noah & Laine’s parents) NoahsHope.com
Sara & David Kennicott (Bridget’s parents) Hope4Bridget.com
Donate online or checks may be mailed to:
Noah’s Hope – Hope 4 Bridget
P.O. Box 36
Barrington, IL 60010
Noah’s Hope-Hope 4 Bridget is a 501(c)(3) registered charity supporting Batten disease research and recognized by the IRS.
A Rare Life & a Lotus Flower
Written February 2019
If we measure life in moments, Rare Disease Day is a time of progress, hope, and celebration. Together, hundreds of patient organizations will tell the world that rare diseases are more common than you might believe. Together, countless people will push for more research, better policies, and effective treatments.
For most families living with a rare disease, there are also days that can only be described as overwhelming, challenging, and downright murky and dark. Our family is no different. Three years ago, we had four beautiful children. Today, we have two at home and two angels.
Noah, our oldest child, experienced his first seizure at age three. More seizures followed, along with a serious and progressive loss of developmental milestones. We consulted doctors at five different institutions over 16 painful months before receiving a diagnosis of CLN2 Batten disease. For most people with a rare disease, the journey to diagnosis is even longer: an average of five years.
In children like Noah, a mutation in a key enzyme impacts the body’s ability to process intracellular waste. Essentially, “trash” builds up, particularly in the central nervous system and retinas. The devastating impact is only realized after the first few years of life. Unfortunately, children with CLN2 Batten typically succumb to their disease by age 12, before treatment was available.
With knowledge of this rare genetic disease, we tested our three-year-old twins and learned that one of the girls, Laine, also had CLN2 Batten disease. We faced a murky future of unknowns: Will we be able to stop the disease? Will we be able to slow it down? How can we enjoy our family as we spiral into this Batten world?
Like many parents in similar situations, my husband Tracy and I had no training in research, medicine, or public policy. Yet, we found ourselves working to fund research, raise awareness within our community, and lobby for changes at the federal and state levels. In order to sustain Noah and Laine’s exhausting medical routines – and to cope with many sleepless nights – we looked for ways to keep ourselves healthy and happy.
About three years into our Batten journey, I started practicing yoga and heard the story of the lotus flower. The most interesting symbolic aspect of this plant is how it comes to life. Typically, the lotus is born from muck and mire. Yes! From sludgy, slimy waters, this brilliant blossom emerges. I learned we can make the best of our crummy environment and rise above our challenges.
During those long Batten days, I focused on the beauty that my children bring to me. Noah, Emily, Laine, and Colette are each my lotus flowers rising from the murky and unpredictable waters of CLN2 Batten disease. This beautiful image gives me the perspective needed to get up every day, enjoy my children, and advocate for others. For the most part, I don’t sink into murky waters.
I draw energy from participating in meetings and conferences like the Global Genes Patient Advocacy Summit. When I attended my first Rare Disease Week on Capitol Hill, I met Chris Smith from SmithSolve during his break-out session on “Sharing your Story.” Chris helped prepare us to speak with members of Congress and their staff, so they could hear first-hand the daily challenges of living with a rare disease.
As I return to Washington DC this week, it will be the first Rare Disease Legislative Advocates (RDLA) conference without Noah or Laine. While I ponder the bitter-sweetness of our life over the last ten years, I remind myself to look for the many lotus flowers surrounding us: The FDA approval of Brineura, the first treatment shown to stall the progression of CLN2 Batten disease. The tireless work of researchers searching for treatments and cures for CLN2 Batten disease and other rare disorders. The friends and family who continue to provide their unwavering support. And the perspective I gain by learning to love and appreciate every moment of this murky, slimy, beautiful life.