By Deepa Arora
Illustration by Malcolm Garris
April 5, 1999

Imagine what life would be like if you weren’t able to hug your family, smile, speak, or even move. For patients with amyotrophic lateral sclerosis (ALS) and the nurses who work with them, these thoughts are more than conjecture—they are a reality that must be faced every day.

ALS is a neurological disorder characterized by progressive degeneration of motor cells in the spinal cord and brain. Muscles gradually atrophy, causing increased muscle weakness, especially in the arms and legs. The muscles that control speech, swallowing, and breathing are also affected. The condition, sometimes called Lou Gehrig’s disease, is terminal.

Living with the disease

Every year in the United States nearly 5,000 people are diagnosed with ALS. Most patients have some symptoms for as long as 12 months before they are correctly diagnosed, said Pat O’Connor, RN, nurse case manager at the Forbes Norris MDA/ALS Research Center in San Francisco. "The disease is often confused with multiple sclerosis, but the patients die quicker because the progression is more rapid," she said.

The life expectancy of a patient with ALS averages two to five years from the time of diagnosis. Despite the body’s progressive degeneration, the mind is not impaired and remains sharp. ALS does not affect intellectual functioning, nor does it interfere with the ability to taste, see, smell, hear, or recognize touch. "This is what makes ALS so unique," said Ileane Mindel, RN, home liaison for the Les Turner ALS Foundation in Skokie, Ill.

"The diagnosis is often given to people as a death sentence," O’Connor said. "Patients are told they should get ready to die." But patients need to be told that they can live with the disease, she said. "With the support of family and the healthcare team, we can maintain the quality of their life."

Nurses working with patients with ALS try to provide symptomatic relief, prevent complications, and help maintain patients’ quality of life. In the later stages of the disease this becomes more difficult because even though patients are alert, they are functionally quadriplegic and must prepare themselves to die.

Questions and answers

"I constantly try to instill hope that there will still be good times," said Linda Boynton de Sepulveda, DNSc, FNP, RN, coordinator of the ALS clinic and research center at UCLA. "I tell them that we will try to keep them independent and comfortable for as long as possible."

Some of her patients have mentioned suicide as an option they might consider when the disease progresses past a certain point. But talking about medications and therapeutic alternatives can help them move away from their despair, she said. "Control is a big issue with ALS patients," de Sepulveda said.

Many patients have questions about death and the dying process, O’Connor said. Since most ALS deaths are caused by respiratory failure, patients are afraid they may face excruciating pain or choking at the end. With proper disease management and medication, most patients die very peacefully, often in their homes, O’Connor said. By answering their questions and helping patients confront their fears of dying or becoming a burden on their family, nurses can give patients control over their disease and their life, O’Connor said.

Instilling hope

It’s not enough to just help ALS patients with their physical symptoms, Mindel said. The pain that patients experience from being unable to do such things as putting their arms around their loved ones or kissing them is devastating and much harder to treat.

"Hope is the most important thing you can give an ALS patient," said Kayvan Freeman, RN, ALS coordinator at the Kessler Institute for Rehabilitation in West Orange, N.J. "Hope that their brain is still alive and that they can still love."

Freeman, who runs a support group for patients and families, said fear and depression are openly discussed at the group’s monthly meeting. "Patients use the group to network about medications, insurance, or everyday problems, such as how to get to bed or the bathroom," she said. "They become a large family and that helps them to keep going."

Previous Stories
Getting Noticed
Nurses are experts in all areas of health science

E. R. Workers
face a new world

Crimes and punishment Disciplinary action against nurses.

It takes a community
Increasing access to healthcare

School based clinics keep uninsured kids healthy

Wash Out
Could antibacterial soaps create new bacterial strains?

Alternative Therapy
Working with patients
who are looking for alternatives

Grieving at Work
They leave your heartstrings dangling. Then one day, the bed sheets are neatly straightened and they’re gone.