ALS facts

ALS stands for amyotrophic lateral sclerosis and is often referred to as "Lou Gehrig's Disease" after the famous New York Yankee baseball player who died from ALS in 1941.

ALS is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed. The progressive degeneration of the motor neurons in ALS eventually leads to death.

ALS is not contagious.

ALS is responsible for nearly two deaths per hundred thousand population in the United States every year.

About 5,600 people in the U.S. are diagnosed with ALS each year. The incidence of ALS is two per 100,000 people, and it is estimated that as many as 30,000 Americans may have the disease at any given time.

Although the life expectancy of an ALS patient is typically two to five years from the time of diagnosis, this disease is variable, and many people live with quality for five years and more. More than half of all patients live more than three years after diagnosis.

ALS occurs throughout the world with no racial, ethnic or socioeconomic boundaries.

The onset of ALS is insidious with muscle weakness or stiffness as early symptoms. Progression of weakness, wasting and paralysis of the muscles of the limbs and trunk as well as those that control vital functions such as speech, swallowing and later breathing generally follows.

There can be significant costs for medical care, equipment and home health care needs later in the disease. It is important to be knowledgeable about your health plan coverage and other programs for which your may be eligible, including SSA, Medicare, Medical and Veteran Affairs benefits.

Riluzole, the first treatment to alter the course of ALS, was approved by the Food and Drug Administration in 1995. This antiglutamate drug was shown scientifically to prolong the life of persons with ALS by at least a few months. More recent studies suggest that Riluzole slows the progress of ALS, allowing the patient more time in the higher functioning states when their function is less affected by ALS.

Reports from three patient databases described long-range experience with Riluzole. All three reports suggest a trend of increasing survival with Riluzole over time. More studies that are double-blind and controlled are needed to confirm these database observations.

The ALS Association.

At Christmas time in 2009, Jennifer and Todd Keevern took their daughter, Sophia, to visit Santa Claus at Northwoods Mall, and they instantly "fell in love" with the rotund guy in the red suit.

Like Santa on Facebook

The friends and family of Joe Darnell have set up a Facebook page for him and invite people to post photographs. Go to www.facebook.com/SantaJoe.

"He was the perfect Santa," said Jennifer Keevern, of his natural white beard, rosy cheeks and blue eyes matching his jovial disposition. "I immediately knew, 'This is OUR Santa.'"

In 2010, the family returned to Northwoods with a new addition, Owen, then again in 2011.

In 2012, their Santa wasn't at Northwoods. A different man was sitting in Santa's chair.

"I walked in the doors and looked for him and I was upset," recalled Keevern, adding that the family opted to go to another Santa Claus for a family picture.

It still wasn't the same.

Last February, Keevern, a physical therapist at the Medical University of South Carolina, met with a couple, a man with ALS, or "Lou Gehrig's Disease," and his wife, who live in Goose Creek.

"I looked at him and he looked so familiar. I started talking to his wife and she mentioned that he used to be Santa Claus. My heart sank. I said to him, 'I know you! You're my Santa!'?"

Following the therapy session, she admitted, "I wept."

Joe's calling

Joe Darnell worked at Cummins Engine for 32 years before retiring a few years ago. His longevity and loyalty at work was trumped only by his commitment to the love of his life, Debbie. The couple began dating when they were 15 years old. They were married in 1970.

In 2008, a year after Debbie retired from BellSouth, she decided to go back to work, and urged Joe to do so too. When a sales clerk at Dillard's mentioned that Joe would make a good Santa Claus, Debbie decided that was going to be Joe's new job, even though he was more than OK being retired.

"I marched him down to that (mall) office and it was like I was taking a misbehaving kid to the principal's office," said Debbie. "He was walking about 30 or 40 yards behind me. He's like, 'I'm happy doing what I'm doing, so why are you doing this to me?'?"

But Joe, who was 6-foot-1 and 285 pounds at the time, turned out to be a natural.

Cherry Hill Photo, which specializes in photos with Santa in regional malls, immediately found him a fill-in job in Columbia and gave him a Santa training manual.

"He was an absolute nervous wreck on the way up there," recalls Debbie. "He was reading that book and practicing while I was driving. But the minute he put on the Santa suit, it was the most natural thing for him. Joe has always been basically very reserved, but he put on that Santa suit and was a whole different person."

Afterward, while visiting relatives in Greenville, he got the call that a mall in Durham, N.C., needed a Santa. He drove back to Charleston, packed his Santa gear, and immediately headed to Durham, getting there at 1 a.m.

In 2009 Joe got the Santa gig at Northwoods and held it for the following two years, working often nonstop from 10 a.m. to 9 p.m. during the season.

And while many might want a break from that seemingly exhausting role, once Joe got the Santa bug, he kept the long white beard and often dressed in red year-round. On a cruise in October 2010, people stopped Joe dozens of times to get a photo with Santa on vacation.

"Being Santa was his true calling in life," said Debbie. "It was an honor for him to brighten the day of others. ... Children would come up to him year-round and he would remind them to be nice because he's watching."

Debbie herself reveled in Joe's experiences. Sometimes she would go to the mall and just watch him with the kids.

"The one thing that I admired so much about him is that he just had a calmness and sweet demeanor and knew exactly what to say to the children," she said.

"Even though he worked for a company that wanted to make money with the photos, that didn't matter to him. He took his time with each child, regardless. And if there was a line, then so be it, he wasn't rushing any child off his lap."

Joe would come home and share stories that would make both of them laugh and cry, such as the little girl who told him that what she wanted for Christmas was to have Santa "bring my mother back from heaven."

Debbie added, "There were a lot of funny ones too, like the child who warned Santa "not to give the reindeer chocolates because it would make them 'f-a-r-t.'?"

Trouble surfaces

Joe Darnell had planned to be Santa for the rest of his life. It was something he loved doing and was just starting to do when one of the most insidious diseases to strike human beings showed its first signs in April 2011.

Debbie noticed that Joe's legs, which has been rock-solid since his days of playing high school football, were starting to get smaller. Joe himself noticed weakness in his upper body.

Debbie, whose stepfather died of ALS 22 years ago, secretly feared the worst.

There is no test to diagnose ALS, or amyotrophic lateral sclerosis, a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord.

According to the ALS Association, as motor neurons degenerate, they no longer can send impulses to the muscle fibers that normally result in muscle movement.

Early symptoms of ALS often include increasing muscle weakness, especially involving the arms and legs, speech, swallowing or breathing. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy (become smaller). Limbs begin to look thinner as muscle tissue atrophies.

When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.

The progressive degeneration of the motor neurons in ALS eventually leads to death.

Diagnosis usually takes place when all other diseases are ruled out.

"When (medical experts) say all your tests are negative, that's not good because it means it's ALS. Negative tests defaults to ALS," said Debbie, who noted that early tests on Joe ruled out problems such as poor bone density.

"I prayed and prayed and prayed to please not be ALS, and one day I was in the shower and I just knew. I started praying, 'Please help me get through it.'?"

While not officially being diagnosed with ALS during Christmas of 2011, Joe endured symptoms, including losing nearly 70 pounds, to be Santa for one season.

"He managed to be Santa in 2011 only through sheer determination. I could tell he was not well. Before he could come home at night, Joe had to sit in the car for 15 minutes because he was so worn out."

February 2012

The official diagnosis of ALS came two months later.

While his decline continued, Joe was still able to walk with the aid of a wheeled walker the rest of the year, but the first Christmas after ALS was a challenge for him and Debbie.

"It was terrible for everyone. And it still is hard," said Debbie, adding that she was tempted not to put up Christmas decorations. "If it were not for him and his love for Christmas, I wouldn't have done it. I asked him and he said bring them out."

A year after the diagnosis, Joe started having problems breathing. They had to decide whether to put him on a ventilator or let nature run its course. Debbie recalled Joe saying he just wanted a couple more years.

She added that the trip to the hospital last February was the last time Joe walked. Today, Joe, who just turned 62, can barely talk.

"It's so sad, so incredibly sad. 'Heartbreaking' is the word I use because it's the only word that can describe my feelings. To see this man I love and see just a little bit of him go every day, it's hard," said Debbie, adding that Joe was a "caring, wonderful husband and father" who didn't deserve such a fate.

"As the caregiver, I pray for strength. I thank God every night that he got me through the day and that he gets me through tomorrow. I used to cry over Lassie. I'm just so emotional, but the strength I've gotten has been remarkable. Still, it's a hard journey."

Because of the difficulty Joe has speaking, he uses a bell to get Debbie's attention when he needs help. Debbie, who sleeps on the sofa next to Joe's bed in the living room, admits that "sometimes that bell drives me nuts," and that she'll even dream about hearing it at night.

"Last night, I had just got settled in and the bell rang. I thought, 'Oh my gosh, what, what, what?' I went over to him and all he wanted was to hug and kiss me. And I thought, 'How can I feel so bad about that bell now when that was what he wanted?'?"

The fight continues

As research for a cure continues, those who must endure ALS, like the Darnells, get help from the ALS Association, which offers support groups for "pALS" (people with ALS) and "cALS" (caregivers).

Dorian Jeffcoat, care services coordinator with the Charleston-based South Carolina chapter of the ALS Association, said the Darnells have been an inspiration to the local ALS community.

"Since our first meeting, Joe has always had a smile on his face, and even though ALS may have robbed him of his speech, it did not take away his positive spirit and his infectious smile," said Jeffcoat, underscoring the importance of having a support community.

"This disease can be very isolating and make one feel as if they have been forgotten, but knowing when you pick up the phone there will be someone on the other end to listen, to provide words of encouragement, to provide direction and to provide the resources needed when traveling along this ALS journey brings about a sense of ongoing support and knowing that you are not alone."

Reach David Quick at 937-5516 or dquick@postand courier.com.