An Ongoing Mystery
LPNs are helping push legislation to find cure, treatment for ALS
Sue Coyle








As a disease, ALS progresses at a rapid pace; but as an understood condition, the process of finding a cure, let alone gaining a better understanding of its cause, has been painstakingly slow.

Today, the neuromuscular degenerative disease affects an estimated 30,000 Americans, with approximately 5,600 people in the U.S. diagnosed each year, according to the ALS Association. In the past few years, the condition has gained considerably more attention thanks to advocacy groups which include LPNs.


Finding Clues

What is known is that ALS is a neuromuscular disease that affects motor neurons in the brain and spinal cord. As the disease escalates, all voluntary muscles weaken and become atrophied. Early symptoms, such as tripping while walking, exacerbate to the point where the person can no long ambulate. As his condition continues to deteriorate, simple motions such as sitting up, speaking or breathing cannot be performed without assistance.

Since motor neurons control voluntary movement, sensory nerves and those affecting the mind aren't typically related to ALS. So even as the body stops functioning, the brain continues to work, creating considerable psychological issues for people with ALS.

"All the ALS patients I've seen have been highly intelligent people," said Marva Graham, LPN, a staff nurse with Bayada Nurses, Morristown, NJ. "So frustration easily comes in and, in a lot of cases, they don't want to beckon for help. You have to be very understanding."

Commonly, patients are diagnosed with the disease only after every other possibility such as stroke and Lyme disease is ruled out. A person with ALS typically undergoes blood and urine tests, X-rays, MRIs and even spinal taps before a diagnosis is confirmed.

Graham said the mystery surrounding the disease often affects patient care. She remembers caring for a patient who had been experiencing muscle weakness for several years and underwent a number of tests before he suggested the possibility of ALS to his physician.

"He was showing symptoms before his definite diagnosis, but no one was able to pin down what was going on with him," she said.

This delay significantly shortened the length of time between diagnosis and death. Graham's patient lived for 3 more years, which falls within the average 2-5 years predicted by the ALS Association.

While most patients are between the ages of 55 and 70 when diagnosed, according to the association, some develop symptoms much earlier.

"Ninety percent of ALS is sporadic," said Cynthia Knoche, director of chapter patient services at the ALS Association. "It just shows up and there is no direct link. A mutation of the SOD1 gene has been associated with ALS, but that only occurs with about 2 percent of the people with ALS."


Military Service Link?

One possible connection involves service in the military. Researchers first noted an increased rate of ALS diagnoses in U.S. soldiers returning from the 1991 Gulf War, with nearly one-third of Gulf War veterans reporting chronic health problems, including ALS.

"As time went on, it was discovered that veterans as a whole are twice as likely to be diagnosed with ALS," explained Knoche. Additionally, the risk was proven to exist regardless of whether a person had been deployed overseas or remained in the U.S., she said.

Officials are unsure of the connection between ALS and veterans, but they are exploring theories such as trauma, exposure to toxins, intense physical activity and vaccinations.

On Sept. 23, the VA named ALS a service-connected disease, allowing veterans who have served for more than 90 days and been diagnosed with the disease to receive medical coverage, including disability pay, lifetime healthcare and death benefits.

The VA implemented this ruling without the standard public-comment period, to allow veterans, especially those who are experiencing financial hardship, to receive care earlier. This includes coverage for symptom-alleviating medications as well as speech and physical rehab.


National Data

Another piece of legislation signed into law last month is designed to help all Americans with ALS. The ALS Registry Act will establish a national ALS data registry run by the CDC. Both patients and healthcare professionals will be able to access the database, and information will be collected from state registries as well as healthcare professionals to provide a complete, national documentation of the disease's development and progression.

The Registry Act is a big step forward in raising ALS awareness and gaining a better understanding of its causes, said Joan Argento, LPN, patient services coordinator for the Connecticut chapter of the ALS Association.

"For the past couple of years, the ALS Association has been asking for this registry," she said. "We're so pleased it got through. Now we'll have some place where people can submit their symptoms and the progression of their disease. Hopefully they'll come up with a common denominator."

Currently, the Muscular Dystrophy Association has its own registry, the ALS Connection (www.alsconnection.com) - a collection of data submitted by people with ALS throughout North America. To participate, all parties must sign a HIPAA form and create a user ID before gaining access to the registry. The site is coordinated by a team of doctors in San Francisco who treat ALS patients at the Forbes Norris MDA/ALS Research Center.


LPN Involvement

Argento is one of a few LPNs who are also trying to make a difference at the state level. She became involved with the Connecticut ALS Association 7 years ago after serving as a visiting nurse in Madison, CT. During that time, she cared for a patient with ALS for 7 years.

"After he passed away, I had initially wanted to run as far away from ALS as I could," Argento recalled. "Watching someone in his 40s deteriorate so quickly had been very difficult for me, but when I saw the [ALS Association] chapter was looking for a patient services coordinator I thought this was a great way for me to make a difference."

The chapter offers the community access to a library of ALS resources, loans and medical equipment such as wheelchairs and walkers to people with ALS. It also provides transportation assistance, respite time for caregivers and support groups for patients and caregivers.

In Georgia, Liz Bohling, LPN, is serving in a similar role as Argento. As a patient services provider for the Georgia chapter of the ALS Association, serving middle and southwest Georgia, Bohling works to improve access to ALS services in the Peach State.

"We provide durable equipment such as wheelchairs and closet shower benches to patients through our loan closet, which is comprised of donated equipment," she said. "We also work more as a team with other agencies such as hospice care organizations and ALS clinics, to ensure all services are delivered."

Research being conducted today is typically aimed at understanding the disease and uncovering potential treatments, Knoche said. The association is currently sponsoring 120 projects, but until a cure can be found care plans are designed to help patients live their lives with as much comfort and decorum as possible.

And that's just what Graham and other LPNs caring for patients with ALS are doing.

"You have to listen to them and let them talk," Graham said. "Take the time for patients to express themselves. Make them comfortable and basically be sensitive to their dignity."

Argento agreed, reminding LPNs not to underestimate their patients.

"People with ALS just want to live each day to their best ability. Let them."


Sue Coyle is an editorial assistant at ADVANCE.


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