The rehabilitation field is filled with success stories. "Work hard, do your exercises and you'll get better" is a formula that pays off for countless patient populations. But amidst a plethora of positive outcomes, some conditions can render even model patients immune to recovery.

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is one such adversary. This rapidly progressive, fatal neurological disorder ravages the nerve cells responsible for controlling voluntary muscles. As the motor neurons degenerate and die, muscles weaken, atrophy and twitch. Eventually patients lose their strength and paralysis ensues, all while the mind remains alert. Once muscles in the diaphragm and chest wall fail, patients need ventilator support to breathe. Despite interventions, the disease steals most patients' last breath within 3 to 5 years from the onset of symptoms.

When beating the odds and happy endings aren't realistic, clinicians must take exhaustive strides to manage patients' symptoms and preserve their livelihood for as long as possible.

Patients and clinicians must remember that life doesn't stop at the moment of diagnosis, says Sara Feldman, MA, PT, ATP, a 15-year veteran with the MDA/ALS Center of Hope at Drexel University College of Medicine in Philadelphia.

A family's world may be turned upside-down, but daily responsibilities and events persist. "Our job is to provide the person with the tools to continue doing the activities that are important to them as independently and safely as possible," says Feldman. Mandatory in this pursuit is an interdisciplinary team of specialists.

COPING WITH CHANGE

Shock, fear, anger and denial only begin to describe the range of emotions a person faces after being diagnosed with a terminal illness. In addition to experiencing the physical symptoms of the disease, patients often battle depression and anxiety, which can become major obstacles in patient care, says Kevin J. Felice, DO, director of the Neuromuscular/ALS Clinic at Hospital for Special Care in New Britain, Conn.

Taming one's survival instinct to accept the course of a disease with no known cure is unfathomable. With counseling and therapy, people learn to cope with harsh realities that lie ahead.

Clinical jargon can diminish the deep impact that ALS has on its victims; patients' words do not. Although each person's experience is different, several interventions usually play some role in management.

"I sit on the examination table and my doctor and I watch as my body twitches uncontrollably. It feels like something is crawling under my skin; it's unbearable. I try to press my skin to make it stop, but I can't...."?¹

Pharmacologic treatments for ALS serve one of two goals-slow disease progression or improve symptoms. Riluzole is the sole FDA-approved medication that can improve ALS survival duration. But it only buys patients 2 to 3 months, says Dr. Felice, and that moderate reprieve can be discouraging.

The majority of medicinal options cater to symptom relief. Seventy-five percent of patients initially present with weakness and atrophy of hand or leg muscles. Cramps, spasticity, fasciculations (muscle twitching) and increased tone soon follow. Quinine or gabapentin help alleviate cramps, while baclofen and tizanidine can control spasticity.

About 25 percent of patients experience bulbar onset ALS, which is marked by speech or swallowing problems, followed by pharyngeal muscle weakness. "Excess saliva in the face of swallowing difficulties is a major problem in ALS," cautions Michael Rivner, MD, Charbonnier professor of neurology at the Medical College of Georgia (MCG) and medical director of the MCGHealth ALS Clinic in Augusta. Therefore, saliva management is a top priority. To dry secretions, Dr. Rivner suggests amitriptyline or glycopyrrolate. Employing guaifenesin can reduce secretion viscosity to facilitate clearing. If these treatments aren't successful, botulinum toxin can be injected into salivary glands or radiation therapy may be necessary.

"Each meal takes me a good 90 minutes to get down. The danger here is you get so bored reheating your food and sticking with it, you want to give up." ²

Patients often turn to various herbs, antioxidants, detoxifying agents and supplements in hopes of stalling disease progression. Although none of these alternatives have been scientifically substantiated through research, proper nutritional management is critical.

The most important dietary factor is the consumption of adequate calories, says Marc DePerno, OTR/L, director of occupational therapy at the Charles T. Sitrin Health Care Center in New Hartford, N.Y. This prevents deterioration due to poor nutrition, he explains. Causes of weight loss among this population are plentiful. Difficulty chewing and swallowing causes choking, and upper limb weakness limits self-feeding. Other factors include decreased appetite, constipation, shortness of breath, nausea and fatigue.

A dietician or nutritionist can suggest foods that meet nutritional and caloric needs and are still easy to eat. Consuming several small meals throughout the day can reduce fatigue and boost calorie intake, while decreasing caffeine and nicotine intake may reduce fasciculations, advises DePerno.

Eventually, a percutaneous endoscopic gastrostomy (PEG) tube placement may become necessary to prevent malnutrition, improve patient comfort and prolong survival. The rehabilitation services team at MCGHealth in Augusta addresses the topic of a feeding tube promptly, says rehabilitation services director Jane Willson, SLPD, so patients have time to think about this option.

"When I go through a period of inactivity, that is when ALS gets a better foothold. That being the case, I try to stay as active as I possibly can." ³

Contention over the possible advantages and disadvantages of exercise for people with ALS has persisted for years. But according to a recent study, moderate strengthening exercises may help people with early stage ALS maintain function and quality of life longer.⁴

"The controversy may come from the perceived risk of damaging the patient or making the disease worse with exercise," says Charlotte Chatto, PT, PhD, assistant professor of physical therapy at MCG. When symptoms of weakness first start, patients sometimes increase their exercise level, attributing their weakness to a lack of exercise, she notes. Although there's no evidence that increasing exercise causes the disease to progress faster, the patient may experience heightened fatigue. Determining if a patient's weakness is resulting from disease progression or from disuse is a dilemma that therapists face when forming a plan of care.