The 'good guy's' disease: ALS afflicts about 300 South Texans
Oct. 30, 2008 at 5:30 a.m.
Carolyn Shimek has been in a wheelchair for only two years, though her husband, Michael, said it feels like it's been longer.
"It's hard to remember when things were normal," he said.
Doctors told Carolyn she had ALS in July 2005. Since then, she has lost the ability to walk and feed herself. Every time she talks, she gasps for breath.
"It just feels like you can't do anything," she said. "You have no control over the way you can use your hands and legs."
Each week, a team of family, friends and nurses help care for Carolyn. Her husband, who is a theology teacher and coach at St. Joseph High School in Victoria, travels back and forth from work to home to assist them to move her off the wheelchair.
"The school has really allowed some flexibility in my schedule so that when I need to go, I can go," he said.
"When we first came out with the disease, the St. Joe family was awesome," Carolyn added.
The school held a fundraiser to buy a ramp van. Others put on a golf tournament and benefit in Hallettsville.
"Friends have been really supportive all the way round," she said.
When the Rev. Bill Hassel dropped the Cross on Good Friday in 2006, he realized something was wrong.
"Your mind says, 'Hold this up,'" the pastor of the First English Lutheran Church in Victoria recalled. "But the muscle wasn't there and it dropped."
Within a matter of days, doctors launched a two-year effort to discover the cause. They ruled out a range of illnesses, from Carpal Tunnel Syndrome to Lyme Disease, before they determined it was probably Amyotrophic Lateral Sclerosis, or Lou Gehrig's Disease.
ALS afflicts about 300 South Texans, said Patrick Callihan, executive director of the ALS Association of South Texas. Anyone can get the neurodegenerative disease, but it is far more common among adults.
Although some people, most notably the British physicist Stephen Hawkins, can live with the disease for a long time, most die within two to five years. ALS patients gradually lose the ability to control their voluntary muscles and eventually become "locked in," Callihan said. Their body does not respond to brain function and they can no longer move.
"There are some circumstances of dementia," Callihan said. "But generally speaking, their mind continues to be just as sharp."
Most eventually suffocate to death because the diaphragm, which is a voluntary muscle, stops accepting the brain's communications.
Doctors understand what happens in the body, but they do not understand why. ALS remains a mysterious disease decades after Yankee slugger Lou Gehrig made it famous. This is the reason other diseases had to be ruled out before doctors could conclude Hassel likely suffered from ALS.
"It's not really diagnosed," Callihan said. "Because we don't know what causes it, that's what goes into our ability to test it."
On Sept. 16, Hassel announced in a letter to his congregation that he would not continue as its full-time pastor. The neurodegenerative disease sapped his strength and limited how much he could do on the job.
"By the end of the summer, I knew I wasn't giving 100 percent," he said.
Many in the congregation were taken by surprise. Others already suspected he wasn't well.
"Some said, 'Oh, that's it,'" he recalled.
Hassel's speech slurs if he talks for too long and he often walks with a cane for balance.
"That Dr. House does it wrong," he said of a television character who also has a cane. "You should use the arm opposite the leg affected."
He feels other indicators of the disease - strain in his forearm and grip, sudden, uncontrollable twitching and leg cramps.
"These muscles are weakening," he said. "They got to do extra work."
Now on a two-month disability leave, Hassel is preparing for life with the debilitating disease. A remote-controlled door and bathroom handrails have been installed in his house.
And every day, he records in his computer some of the 1,640 words and phrases necessary to create a replica of his voice. The program will enable him to communicate when he loses his ability to talk.
Despite his prognosis, Hassel has an optimistic outlook.
"In my mind, I'm convinced I'm beating it," he said.
He's focused on living life completely. After a European vacation with his wife, Renee, he plans to return to the church and help out on a part-time basis until a new senior pastor is hired.
"When the senior pastor walks in the door, I walk out the door," he said.
He is also participating in a study at the Methodist Neurological Institute in Houston. The study analyzes the effect of the drug Lipitor on the inflamed immune systems of ALS patients.
"It actually possibly had a way of transforming the immune system from an inflammatory state to a non-inflammatory state," Dr. Ericka P. Simpson said.
Bringing out the protective and reparatory roles of the immune system could slow the progression of the disease, Simpson said.
Simpson heads the study as co-director of the MDA/ALS Clinic at the Hospital. It includes 17 ALS patients, including Hassel and another Victorian, Carolyn Shimek.
"ALS is called the good guy's disease. These are the people their communities looked up to," Simpson said, adding. "I can't say I found one ALS patient who wasn't willing to participate in some manner. They say, 'If it doesn't help me, it will help someone else.'"
Other neurological diseases are often associated with personality types, Simpson said. For example, multiple sclerosis patients tend to ask many questions and give each question the same weight.
A "good guy" ALS patient was the reason Simpson decided to devote her career to the disease. As a medical student, she was impressed with the response of a 36-year-old man who had just been told he had ALS.
"The patient put his arm around the doctor and says, "Let's start fighting. What are we going to do about it?'" she recalled. "It's just so typical for ALS patients."