ALS slowly steals strength needed for life
Those with ALS, or lou gehrig's disease, face not only the loss of muscle control but also the impending loss of their life
By IN-SUNG YOO, The News Journal


Sally McLaughlin maintains a positive outlook and her reputation for snappy repartee despite the physical toll of amyotrophic lateral sclerosis. (Buy photo)

The News Journal/ROBERT CRAIG


McLaughlin can no longer climb stairs alone. Her service dog, Decker, helps by providing stability.
(Buy photo)

The News Journal/ROBERT CRAIG
Behind the sluggish movements, the silenced voice and the slightly slack jaw, there is the smile.

Since being diagnosed with amyotrophic lateral sclerosis in 2003, Sally McLaughlin, 53, of Wilmington has seen her hands stiffen and grow weak. She needs a walker or wheelchair to get around. Within 18 months, she lost the ability to speak completely. But still, the bright-eyed grin -- and the laugh, raspy and stuttered as it is -- persists.

ALS, or Lou Gehrig's disease, afflicts about 30,000 Americans. The disease has no effect on cognitive abilities, but leads to the death of motor neurons that communicate with the voluntary muscles in our body. When these nerve cells die, there is no way to send commands to the muscles, which weaken and eventually atrophy. Everything from walking to speaking, chewing, grasping, and even breathing is eventually affected.

Death usually occurs within three to five years of diagnosis when the chest wall muscles become too weak to inhale and exhale. Since being discovered in 1869, there is still little known about the disease's causes or risk factors. About 10 percent of cases are inherited, but the rest occur without any clear cause.

As is often the case, the initial symptoms were so subtle that even McLaughlin's family couldn't tell the difference. But one night out at a steak dinner with friends, she knew something was wrong.

"I had two glasses of wine and I thought it was odd to be slurring my words," she said.

The slurring was the first sign of bulbar ALS, which starts in the facial muscles, rather than in the extremities like other forms of ALS. Diagnosing the cause of her problems was a difficult, drawn-out process. There is no single test to diagnose ALS. Instead other conditions like multiple sclerosis must be ruled out. Then, she had to wait five months so her doctor could observe how symptoms progressed before her condition was officially diagnosed.

The muscle weakness in her jaw and throat has made it hard for McLaughlin to control her saliva, frequently causing her to choke on it, as well as food and drink. Before they knew their mother was sick, McLaughlin's three children ribbed her for "always choking on air." After they were aware of the reason, the choking episodes became more severe -- though not life-threatening -- and her children would leave the room, too upset to watch their mother suffer. Hard as it is, they have now learned to ignore it, they said.

"It's hard to watch, but you can't be upset forever," said daughter Eileen, 17.

Around the house, McLaughlin uses a walker. Since she can no longer speak, her primary form of communication is a laptop computer that has been outfitted with a speaker. She types in what she wants to say, and the computer reads it out loud in a synthesized voice. McLaughlin also uses sign language and a lot of nonverbal cues to get across quick thoughts. She can even use her cell phone by replying to calls with text messages or by using the keypad -- pressing 1 for "yes" or 2 for "no" -- to answer simple questions.

Having to wait for her mother to type out long responses on her keyboard or working their way through cryptic hand signals can be frustrating, especially considering McLaughlin always has been known for her witty repartee, Eileen said.

"She was always really quick with a joke or a comeback," she said. Now, Sally's computer is set up with shortcut commands to yell out a biting remark or reprimand when the need arises.

In June, Sally received a black Labrador service dog named Decker to help her around the house. She's not blind, but Decker can help her open the refrigerator by pulling on a rope attached to the handle, pick up kitchen utensils off the ground and give her added stability as she climbs stairs to the bedroom. He's a bit of a goofball, but then again, "they said they try to match the dog's personality with the owner, so I'm not sure what to think about that," she jokes.

Because there is no cure for ALS, there are no "better" days -- just worse ones. And though there is a drug to treat the disease, at best, it can only extend life by about three months. Still, if McLaughlin's initial prognosis had panned out, she'd be dead by now, she says.

"She's just stubborn that way," said son Kevin, 23 as lighthearted and irrepressible as the rest of his family.

For most patients, ALS progresses swiftly, said Dr. Alan Fink, a neurologist at Christiana Care Health System and clinical assistant professor of neurology at Jefferson Medical College of Thomas Jefferson University. However, in some patients, it does not. In fact, 20 percent to 30 percent of patients are alive without significant change to their abilities after five years, he said. And there are other, less-serious neuromuscular diseases that present similarly to ALS in early stages. Careful monitoring is needed to be certain of a diagnosis.

"It is a serious disorder and most of the cases that do have the pure form do carry a serious diagnosis," Fink said, "but not always, and in my mind that's a very important thing to stress."

The neurologist also said that although it must be done carefully, maintaining physical activity can be beneficial for patients. Not only does it fill their days and keep their minds off the disease, there is some indication that it can stall the disease's progression. So can the patient's attitude.

As the disease progresses and breathing becomes difficult, McLaughlin will be faced with the decision of whether she wants to go on a respirator that will breathe for her. It's something she tries not to think about. Instead, she has become involved with the activities offered by the Greater Philadelphia chapter of the ALS Association. Since being diagnosed, she and her family have gone to trips to Hershey Park, the Philadelphia Zoo, Cancún and Aruba. She and Eileen have also given talks about ALS to groups of students.

McLaughlin and her family have no illusions about the future. Instead, they look to short-term survival goals, savoring each opportunity that they are afforded. They cried plenty. Now, they try to laugh as much as possible -- just like always.

McLaughlin is glad to have seen Kevin graduate from the University of Delaware this past spring and hopes she can see Eileen graduate from Brandywine High School next year. Statistically speaking, it's not likely. But that's out of her hands. McLaughlin knows that and embraces it.

"I think about it every day," she admits. "But what can you do?"

She turns her palms up to the sky, cocks her head and shrugs her shoulders. And, as always, she smiles.

Contact In-Sung Yoo at 324-2909 or iyoo@delawareonline.com.
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