On November 9th, The Noah’s Hope – Hope 4 Bridget Foundation hosted its annual A Fifth Season event in Chicago, Illinois. The Hope 4 Bridget Foundation was established in honor of Bridget Kennicott in order to bring awareness and raise funds for research for Batten’s disease.

Batten’s disease is an “orphan” disease, meaning it affects so few people that drug companies have little incentive to invest in research. Therefore, promising research and clinical trials are being put on hold due to a lack of funding. In order to support the scientists and universities who are conducting research on lysosomal storage diseases, like Batten disease, Bridget’s family partnered with Noah’s Hope, another family foundation established in honor of their children, Noah & Laine. Noah’s Hope – Hope 4 Bridget Foundation is a non-profit organization whose mission is to provide funding for research and education in efforts to find a cure for Batten Disease.

As well as to provide support to children and families affected by a horrible disease. The children who are recognized through this foundation all fought an extensive battle, and are represented in honor of this. Bridget’s strength was inspiring, and despite her struggles she always shined bright.

Bridget Kennicott, born on November 11, 2004, was always an energetic, lovable, spirited little girl who wanted to go, go, go. Bridget was beyond active and she loved to dance. She began school at the Early Learning Center at the age of 3 and received an individualized education plan.

At the age of 3 years, 2 months, Bridget had her first seizure. The ER and later pediatrician ruled it a febrile seizure and told us only 6% of children will have another. Unfortunately, she did. And then there was another and another, each seizure gaining momentum, length and severity. Two months after the first seizure, she began medication. Sadly, there was no medication that stopped them from occurring.

Bridget was diagnosed 15 months after her first seizure with Late Infantile Neuronal Ceroid Lipofuscinosis or CLN2 Batten disease on April 20, 2009. Bridget continued to decline and lose motor coordination, the ability to communicate and eat. By 5 years old, she had a g-tube and was wheelchair-bound. During these years, she continued to attend school at Countryside School.

Again, the staff met all her needs with understanding and love. It was a long slippery slope of tests, neurologists and waiting. She lost her battle to this horrific disease on February 15, 2017, at the young age of 12. Barrington schools staff honor Bridget and her memory with two butterfly gardens. One was dedicated to her at Countryside school where she ended her school career in 5th grade, as well as the Early Learning Center who dedicated their butterfly garden to her this fall, 2019.

Batten disease, or Neuronal Ceroid Lipofuscinosis (NCL), is a family of rare diseases caused by autosomal recessive genetic mutations. These genetic mutations disrupt the cells’ ability to dispose of wastes. Cells are thrown out of balance with the build-up of proteins and lipids (fats).

There are 14 known forms of Batten disease and you will often hear them referred to as CLN1-CLN14. It is estimated that 2-4 births per 100,000 in the U.S. are affected by Batten disease, though some researchers in the field suggest these numbers are low. These disorders generally include a
combination of vision loss, epilepsy, and dementia.

A Fifth Season Fundraiser began to help raise much-needed funds for clinical research in CLN2 Batten disease. The name was given to the event reflecting the parents and families, who desperately wish there were more time in the year to find a cure, a fifth season.

This year they celebrated the fundraiser’s 9th year. A Fifth Season fundraiser provides a casual yet, purposeful atmosphere, featuring music, an impressive live and silent auction, dinner, and dessert. Kristina Anderson, a good friend of the Kennicott’s, attended the event.

When asked why, she said “I attended the Fifth Season even because Batten is a tragic, always terminal, rare childhood illness that has no cure. It has stolen the lives of children in our community. And because it is extremely difficult to get funding to cure rare diseases, it is desperately necessary to raise money to support the scientist trying to find a treatment or cure for a rare disease like Batten.”

All funds raised will go towards CLN2 Batten research via Noah’s Hope – Hope 4 Bridget. In 2017, the FDA and the European Medicines Agency (EMA) have each approved the first-ever CLN2 Batten disease enzyme replacement therapy called “Brineura”.

While this is exciting news, new donations will allow us to continue searching for additional treatments and a CURE for CLN2 Batten disease. Over the past several years, the foundation has raised over 2 ½ million dollars toward the fight against Batten disease. They have funded over 27 scientific research projects for the advancement in discovering treatments for this disease.

This year, Sara Kennicott, mother of Bridget and founder of the original Hope 4 Bridget Foundation, started a junior board of student members who have a person connection to Bridget and her family, including Harrison Kennicott, Libby Shannon, Amelia Cook, Maggie Bruce, Mary Kaelin Puccio, Morgan Scariano, Zach Bromet, Daniel Langlois, Daniel Hesselbein, Ian Bouchard and myself. Together this junior board has worked together to promote and publicize the Noah’s Hope – Hope 4 Bridget Foundation and the A Fifth Season Event, as well as set up additional fundraisers and events that students could get involved in.

Although the children whom the foundation was started for could not benefit from this progress, other children like Noah, Laine, and Bridget can. “We will always have HOPE,” Sara Kennicott, Bridget’s mom, said. Bridget, and children like Bridget, are dependent upon donations to fund critical clinical research studies to find a cure for Batten disease.

Visit www.hope4bridget.com, www.noahshope.com, or www.bdsra.org for further information, and ways you could participate and support the foundation.