Benefit to be held for former Jim Thorpe man with Lou Gehrig’s disease

On Aug. 23, 2017, Mark and Stephanie Bennyhoff, formerly of Jim Thorpe, received confirmation of their worst fear. Mark was diagnosed with amyotrophic lateral sclerosis, commonly known as Lou Gehrig’s disease.

According to the ALS Association, the condition is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord.

ALS affects the motor neurons that reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their demise.

With voluntary muscle action progressively affected, people may lose the ability to speak, eat, move and breathe.

There are two different types of ALS, sporadic and familial.

Sporadic, which is the most common form of the disease in the U.S., accounts for 90 to 95 percent of all cases. It may affect anyone, anywhere.

Inherited familial ALS accounts for 5 to 10 percent of all cases in the U.S.

In those families, there is a 50% chance each offspring will inherit the gene mutation and may develop the disease.

In Mark’s case, there is no known family history of ALS.

According to the ALS Association, the average life expectancy of a person with ALS is two to five years from the time of diagnosis; however, it varies greatly.

A normal life suddenly turns abnormal

Mark, who is now 51 years old, graduated from LCCC with a degree in Industrial Automated Robotics Technology, and for six years, he worked for Simplex as a service technician.

On one particular afternoon, he could not screw a nut onto a bolt with his left hand. Visits to doctors determined he had discs protruding on his spinal column in his neck. Surgery was recommended.

A car accident in 2006 left him legally disabled, and another after a return trip from Disneyworld “flipped a switch,” according to Stephanie, his wife of 18 years.

“Mark was randomly falling, and we wondered if the trauma of hitting his head on the steering wheel from the accident was a cause,” she said.

Local neurologists could only guess that Mark had ALS, but four hours of testing at Thomas Jefferson University Hospital in Philadelphia resulted in a neuromuscular disorder diagnosis.

“When I asked if he had ALS, the doctors nodded their heads,” said Stephanie.

The tasks of the daily regimen

She explained that Mark’s body uses all its energy to breathe and to do normal tasks that a healthy person does not have to think about.

“His energy level is like a gas in a tank,” she said. “When he’s running close to empty, he has to sleep, sometimes 15 hours a day.”

When asked about his appetite, Mark said he gets hungry enough, but he eats child-sized portions.

Stephanie said he has to control his weight because losing pounds also means losing muscle.

Mark, who also has diabetes, has 24-hour care to help him get to the bathroom and move him from one place to another.

“We have a chairlift in our house to move him up and down the stairs,” but he needs to be helped through the few steps to the bathroom.”

Mark also cannot sleep on his back or breathe oxygen, so he has a special machine to remove the carbon dioxide from his lungs.

He operates a custom built wheelchair and they recently purchased a handicap accessible van for transportation to doctors; offices, hospitals and wherever they need to go.

Because his immune system is compromised, Mark has to be careful. Catching a cold could become pneumonia.

A dose of hope and a cause for laughter

When asked if he still has hope for a cure, Mark’s eyes lit up.

“Absolutely,” he answered.

He said that there are medical treatment trials in Massachusetts, and since Pennsylvania is a “Right to Try” state, he will not need to wait for FDA approval to take experimental treatment.

In addition, with social media contacts, new information about potential cures for ALS is continuously available.

“Mark is not dying from ALS,” explained Stephanie. “He’s living with ALS.”

They have grown closer together, not just out of medical necessity, but now husband and wife, along with Mark’s sister, Bonnie and their children, Lauren, Nicholas, and Christian, appreciate and enjoy every moment they spend with each other.

“We cry together and we laugh together,” she said. “It’s a struggle each day. We don’t know what to expect, what might get worse, but we’ll do the very best we can for Mark.”

Mark keeps his sense of humor. He is an observer of human behavior.

“I’ll sit in the van and watch people and laugh about the stupid things they do,” said Mark, noting that there’s plenty of that to see every day.

A dance for donation

On Feb. 29, the “Leap Year Fancy Dance of Hope” will be held at Dean Anthony’s Banquet Hall in Jim Thorpe.

Proceeds from the benefit tickets will help the Bennyhoffs pay down some of their debt, which averages $250,000 a year for a person with ALS.

Last year’s benefit raised $7,000. To purchase tickets and for further banquet information, call Bonnie Hoffman at 610-554-3528.

Stephanie Bennyhoff used an old but still valued expression to describe how she and Mark have been coping with their daily burdens.

“Life is 10 percent what happens to you and 90 percent how you react to it.”

The Bennyhoff family has made a reaction to fight his disease. They live each day by a slogan they wear on their T-shirts.

“Never give up!”