Men in the mid to late 40’s are usually celebrating their accomplishments in life with friends and family.

Fishing, hunting, hanging out with the guys on lazy days enjoying the fruits of life’s labor
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For one local first responder, that and more came to a halt this past December when it was discovered ย Russell Butch Helsley, 47, has ย Alpha-1 Antitrypsin Deficiency (AAT).ย
The disease, according to Medscape, is one of the most commonly inherited disorders affecting the Caucasian population. There are two variations of the illness, Helsley’s has manifested in the breakdown of his liver, he said.
Helsley, who is still part of the Tullytown Fire Company, Station 33 ย Fire Police, stopped working and volunteering in December due to the illness’ impact on him.
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He is now in need of a liver transplant.
Medical costs have piled up to the point where there will be a beef and beer held in his honor Saturday night at Westabay Hall in Fairless Hills from 7 p.m. to 11 p.m.
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Despite feeling tired all most of the time or being in pain, Helsley’s outlook is refreshing.
“I spent most of December, January and February in the hospital”, he said. “My friends said I just like the food there better,” he said as we both laughed at the self-deprecating comment.
“I’m not ready to die,” he said during a pause in the exchange Friday afternoon.

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Helsley shared some of the adjustments he’s had to make in his life as a result of his illness.
“I still go down to the firehouse to hang out with the guys,” sensing he misses the everyday in the moment camaraderie local first responders are known to share among “the brotherhood” as one local firefighter, George Redner, often describes it as.
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“I take lots of naps these days, ” he said, as we both agreed that’s not exactly a bad thing.
Once diagnosed, Helsley quit smoking , which is a exacerbating factor for the manifestation of the deficiency and contributes to current symptoms for many that have the COPD version ย of the illness.
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His lungs and heart are fine, he said. “It’s just this liver that keeping me down,” he offered.
Organizers of the fundraiser hope the beef and beer will defray costs of a liver transplant procedure, which Helsley is now in need of.
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Helsley said he has COBRA insurance at the moment but the costs are prohibitive.”But with the friends I have – I’m sure I’ll make it through,” he said.
Helsley had one request of the public before the conversation ended.
I just want people to sign the donor part of their licenses, ย he said, donors are needed and there aren’t many these days.
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AAT is a relatively new illness discovered by research scientists in Sweden during the the early 1960s according to Medline.
The liver variant of the illness was reported in the late 60’s
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If you can’t make the Beefย and Beer being held Saturday night, Tina Marie Grady said donations can be made in Helsley’s name at Well Fargo Bank.
Or you can contact Grady atย mommyoo6@yahoo.com ย to find out other ways to help.
Tickets are still available for tonight’s fund drive for $30 at the door.
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The brotherhood of first responders from the area will be there in numbers as is always the case when one of their own suffers.


