While the ALS Ice Bucket Challenge has spawned video after video of folks being doused with icy water and also raised money for research while encouraging people to learn more about the disease, for one family in Bucksport, this progressive neurodegenerative disease is all-too-familiar.
But the Klenowski family of Bucksport doesn’t like to spend much time thinking about their chances of developing amyotrophic lateral sclerosis, also known as ALS or Lou Gehrig’s disease. Instead, the possibility has given the family of four a cause and spurred them to live fully every day. They’re glad to see the disease in the spotlight thanks to thousands of people participating in the Ice Bucket Challenge to raise money for ALS research.
ALS is a progressive neuromuscular disease that causes patients to slowly lose the ability to move, speak and eventually, breathe. It is not typically hereditary, and there is no known cause or cure.
However, for the Klenowskis, it appears the disease that took the life of Paul Klenowski Jr.’s father and grandmother may be hereditary, making them part of 5 to 10 percent of patients who pass the gene, according to Gregory Cox, an associate professor at Jackson Lab.
ALS is a particularly challenging disease for families such as the Klenowskis because even though body function deteriorates, the patient’s mind remains intact, making him or her painfully aware of changing physical capabilities.
“You know how much function you’ve lost from one day to the next, you know what you could do yesterday that you can’t do today and that’s really sad,” Cox said. “It’s particularly hard on caregivers, whole families are affected.”
Because of the genetic connection, the family has met with scientists at Jackson Lab who asked for a DNA sample from Paul Klenowski Sr. and talked with them about their likelihood of a diagnosis and genetic sequencing that could determine whether or not they have the gene. Paul Klenowski Jr. has a 50/50 chance of being diagnosed, and if he is, his two sons, Hunter, 12, and Moxen, 7, also have the same chance.
Heidi Klenowski said she and her husband decided he would not take a test.
Instead, they want to spend their time focused on how they can help expedite the search for a cure by fundraising and helping bring awareness to the issue.
“It’s heartbreaking, but at the same time, it’s not something that’s on our mind all the time, we don’t talk about it all the time,” Klenowski said. “It’s a touchy subject when it gets into the details of our specific experience, but I like spreading awareness.”
Because of the increased popularity of the ALS Ice Bucket Challenge, fundraising has reached an all time high for both the family and the ALS Association, which among other things, advocates for more research about the disease and potential cures. T he association received $4 million between July 29 and Aug. 12, and donations have come from 70,000 new donors.
“It’s absolutely phenomenal, and it’s one of those things that I don’t think anyone could have ever predicted,” said Carol Lamb, a Hampden resident who is co-chairman of a Bangor-area ALS walk. “It’s brought the name ALS to every household.”
Klenowski said she already has raised $450, though that may have something to do with her promise to douse herself with one bucket of ice water per $10 donated.
This Friday, she will be at Carrier’s Mainely Lobster in Bucksport starting at 4 p.m., and she will subject herself to dump after dump of ice water until the last donor has left. The restaurant also has offered anyone who donates $10 or more 10 percent off their meal. Klenowski said she’s heard from dozens of people interested in supporting the cause.
“I just came up with this idea of doing one bucket for $10, and now I’m going to be soaking wet for six hours straight,” she said with a laugh.
And while she’s excited to have so many people donating through their own ice bucket challenges, Klenowski said she’s mostly happy that people are learning about ALS and shedding light on its impact on families.
“I just want people to be aware that it’s out there and hopefully that with enough research and fundraising we can beat this so that nobody else has to go through this horrible disease,” she said.
For information about the Klenowskis’ fundraising event on Friday, visit their Facebook page at https://www.facebook.com/events/1498541550391738/?ref=2&ref_dashboard_filter=upcoming.