- COMMUNITY LINKS
For many folks, it seems to be the simplest of tasks: "I can get into the grocery store and remember things without even writing it down," says Jim Bodien of Ridgway, "even if it is just two or three things."
Jim suffers from Huntington's disease, a genetic defect on chromosome No. 3 that has caused loss of memory, judgment, speech changes, personality changes, and has brought on confusion in his life. To simply remember those two or three grocery items is in itself a noteworthy achievement.
"It seems like it goes in three-month increments where it's a little bit worse here and there," said Nancy Bodien, Jim's wife. "He has to go for his regular therapy and the doctor's appointments go back and forth between DuBois and back and forth to Pittsburgh on a regular basis.
"He did get approved for his disability and that happened in November of 2010."
However, with disability benefits based on 30 years worth of work, multiple zeroes have been factored in and do not work in Jim's favor. He is just 44 years old.
"The amount is unbelievable that we have to live off of, it's considerably less because it's based off 30 years of earnings and he hasn't worked 30 years, he's only 44," Nancy said. "He hasn't worked that long so there are a lot of zeroes factored in-- we're making the house payment and some other payments are sometimes let go for a while, basically robbing Peter to pay Paul."
To counter the effects of the debilitating disease, Jim has been prescribed tetrabenazine, a drug that is supposed to slow the symptoms.
"We're trying to keep him from having to get on much more of it because it's so potent, and the effects of it make him tired," Nancy said, noting that Jim was taking 25 milligrams last year but his dose has since doubled. "The mood swings are also difficult to deal with from the medication as well-- everyone sees 'mellow' Jim, but the kids and I don't always see that-- but it's not his fault.
"Nice, mellow Jim is who I married but he can be mean, he can yell, and that's all from the medication and the disease itself, that's just another way that it affects you, affects your brain and your attitude."
Huntington's disease is known to cause antisocial behaviors, hallucinations, irritability and moodiness-- but perhaps the most noticeable problem involves simple everyday tasks: chewing of food, swallowing, and even walking.
"He has to physically think when he walks and tell himself, 'I have to take a step in front of the other step to walk or I'll fall on my face,'" Nancy said. "That's what he'll do when he goes to therapy, just retraining the brain every time, and he forgets it within three months, and that's just the progression of the disease chipping away at the brain cells."
Jim is currently immersed in a five-year study in Pittsburgh for Coenzyme Q10 (CoQ10), a component of the electron transport chain that participates in aerobic cellular respiration.
"We wanted to get him into a different study but the last drug ended up failing anyway," Nancy said. "This one is for CoQ10, it's for something that your body already produces anyway and it helps people remember, it helps your heart, it helps your brain-- but they're studying it right now because they're thinking there is a probability that it would stop the progression of the disease and help your short-term memory.
"It's a double-blind study so we don't know what he has, but his short-term memory seems to have gotten quite a bit better since he started on the study. It was just simple things we'd do last year, like tell him something and walk away, and then later ask what it was and he would have forgotten."
Another frustrating part of the disease is the patience involved, not just with everyday life, but with pharmaceutical advancements as well.
"There are other things that have been looking really good as far as possible medications, but you just never know how long this stuff is going to take," Nancy said. "I guess first the scientists had to figure out what part of the brain it's targeting and really how the disease is targeting it before anything could ever be developed to counter the effects, and I know that has taken a while.
"They're trying to work on something now to make it so that this hits you when you're maybe 100 years old, but not in your 40s."
The defect causes a part of DNA, called a CAG repeat, to occur many more times than it is supposed to, according to the National Center for Biotechnology Information. But in persons with Huntington's disease, it is repeated 36 to 120 times. As the gene is passed on from one generation to the next, the number of repeats-- called a CAG repeat expansion-- tends to get larger. The larger the number of repeats, the greater your chance of developing symptoms at an earlier age. Therefore, as the disease is passed along in families, it becomes evident at younger and younger ages.
"I wish we had started looking for these studies years earlier, but we weren't really able to pinpoint when his symptoms started," Nancy said. "We've been lucky enough to be able to rely on some people who tell us some things that they remember, maybe acting nervous or whatever, and that helps us to pinpoint the start of his progression.
"I certainly appreciate that people tell me if they see something not right with him-- I'm with him all the time so I don't really see everything, it just starts to become normal. We're lucky to live in a town where people know you well enough that they're able to say these sort of things to you because it really helps.
"That way I'll know what to watch for and then we can go to therapy and get the problem fixed. We certainly have people who care enough to tell us when they notice something and we greatly appreciate that, it means a lot to us."
A bit more than a year ago, the Bodien family shared Jim's struggles with the local population through newspaper articles and since then, several businesses and organizations stepped forward and offered to help through various fundraising efforts.
"We were able to do some amazing fundraisers and the response was unbelievable, and we appreciate everything that was done for us," Nancy said. "That, along with the disability, has carried us up until now and now we had to use loose change to take our youngest child to the doctor's office. It seems like everything bad that could happen has happened.
"The last few years has been one thing after another and now I'm under doctor's care right now-- they're trying to figure out what's wrong with me and it's probably stress-- our youngest one also started get bald spots on his head, so we had to go to the doctor about that too-- we also had one that put his shoulder out of place.
"Dealing with all that probably raises the stress and it causes us to do a lot of extra traveling back and forth as well."
But as things started to look more and more grim, a new opportunity arose-- a fundraiser with the Championship Wrestling Federation as the Ridgway Rumble hits town Aug. 6 at Central Fire Station along North Broad Street in downtown Ridgway.
"We've been friends with one of the promoters for CWF wrestling, Bill 'Powerhouse' Hughes, and they've been to Johnsonburg a few times to see the shows, and I had met him from working at the radio station," Nancy said. "He had mentioned about really wanting to come back to the area and they hadn't been contracted out here recently, and he suggested that it would be a good fundraiser for the family and that they'd love to help us out."
Hughes and a band of wrestlers are waiting in the wings and more than happy to be back in the area.
"With going to the area about once a year to do a show, I met Jim and Nancy," Hughes said. "We really consider the area our home away from home, and Jim and Nancy were at the first show that we put on in Johnsonburg back in 2008.
"I got to know them and I've made a lot of other friends in the area as well, and kept in touch with a lot of people. I've been kept in the loop about their situation and we keep in touch through Facebook to see how they're doing."
Last year during Johnsonburg's Bicentennial celebration, Powerhouse was able to make an appearance at a breakfast fundraiser to help raise money for the family in need.
"It just the type of thing where I was always raised to help others and with this situation, I thought it was the least we could do," Hughes said. "We hope that this show will help raise money for them and more awareness as well.
"It gets us back in the area and we're really excited about that. We literally had to turn some wrestlers away."
The venue will hold roughly 375 spectators with the ring and organizers hope to sell 300 tickets, and could also sell standing-room-only tickets at the end if regular passes are exhausted.
$50 will sponsor a wrestler and $100 will sponsor a match. Tickets are $15 ringside, $12 for adult general admission and $10 for child (under 12) general admission. The doors will open at 6 p.m. and the first bell will ring at 7 p.m. Tickets are available at Ridgway BiLo or by calling (814) 335-0473. Patrons can also meet and dine with several of the wrestlers from 2-4 p.m. at Joey's Bakery along Main Street in downtown Ridgway on the day of the event.
Pick up a copy of the Saturday, July 23. 2011 edition of The Ridgway Record for more.