Specialized Treatment Centers Improve Quality of Life for People with ALS

By Lynne P. Taylor, MD, FAAN, FANA

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Imagine being seemingly healthy one day and the next being diagnosed with a progressive, degenerative neurological disorder that will slowly interfere with your ability to move, speak, eat and even breathe. This disease, a tragic reality for thousands of Americans, is called amyotrophic lateral sclerosis, or ALS.

ALS is incurable. For reasons not well understood, nerve cells in the brain and spinal cord that control voluntary muscle movement gradually deteriorate. As a result, muscles atrophy, leading to paralysis and death, often in three to five years.

Messages from motor neurons in the brain and spinal cord are scrambled in ALS. These control functions like limb movement, swallowing and aspects of breathing. Senses and thinking processes remain normal in people living with ALS and, thankfully, pain is rare at any stage.

Background

French neurologist Jean-Martin Charcot discovered the disease in 1869. In the United States, ALS is often called Lou Gehrig’s disease after the beloved professional baseball player whose struggle with the disease and death in 1941 brought it to national attention.

A-myo-trophic comes from the Greek language. “A” means no. “Myo” refers to muscle and “Trophic” means nourishment – thus, “No muscle nourishment.” When a muscle has no nourishment, it “atrophies” or wastes away. “Lateral” identifies areas in a person’s spinal cord where portions of nerve cells that signal and control muscles are located. As this area degenerates, it causes scarring (“sclerosis”) in the region.

The motor nerves affected by ALS provide voluntary movement and muscle control in the arms and legs, which allow people to perform most daily activities.

Types of ALS

There are two kinds of Lou Gehrig’s disease, one is called “sporadic” and the other, “familial.” Sporadic, which makes up 90 to 95 percent of all cases in this country, may affect anyone, anywhere. Familial means the disease is inherited. It accounts for 5 to 10 percent of all cases in the United States. In those cases, there is a 50 percent chance each offspring will inherit the gene mutation and develop the disease.

A relatively rare disease

According to the ALS Association, more than 5,600 Americans are diagnosed with the disease each year, which amounts to 15 new cases every day. It is estimated that as many as 30,000 Americans have the disease at any time. Although most people who develop ALS are between 40 and 70, with an average age of 55 when diagnosed, cases do occur among people in their 20s and 30s. However, ALS generally occurs in higher percentages among older men and women. Despite it being 20 percent more common among men, the incidence of ALS is more equal between men and women with increasing age. For unknown reasons, military veterans are about twice as likely to develop ALS. Half of all people affected with ALS live at least three or more years after diagnosis. Twenty percent live five years or more, and up to 10 percent live more than 10 years.

ALS symptoms

Symptoms usually develop after age 50. In its early stages, ALS symptoms include:

  • Increasing weakness in one limb, especially in a hand
  • Clumsiness of the hands
  • Difficulty walking
  • Subtle, light twitches under the skin
  • Impaired speech
  • Difficulty swallowing

Modern treatments have allowed people living with Lou Gehrig’s disease to continue communicating and moving. An important aspect of the disease is that involuntary muscles, such as those that control heartbeat, digestion, bladder and sexual function are not directly affected by ALS.

Fortunately, intellect, hearing, vision and touch generally function normally in people living with ALS. One behavioral effect, called involuntary emotional expression disorder, causes some people living with ALS to lose control over expressions, like laughing and crying. Some experts believe this is caused by lost neurons that control muscles involved in physical expressions of emotion.

Diagnosing ALS

ALS can be challenging to diagnose since symptoms can resemble other neurological disorders. A definitive diagnosis is based on a careful medical history, physical exam of the nervous system along with muscle and nerve function tests.

Medications

Currently, there is only one FDA-approved drug, riluzole, which can slow the progression of ALS in some people. Although there is not yet a cure or treatment that halts or reverses the disease, scientists have made significant progress in learning more about it over time.

Research

There are numerous research studies – past and present – investigating possible risk factors that may be associated with ALS. However, more work is needed to determine what genetic and environmental factors contribute to its development.

Improving quality of life

Research has shown that people living with ALS benefit from a better quality of life by participating in support groups and receiving care at an ALS Association-Certified Treatment Center.

These centers, like the ALS Clinic at Virginia Mason, provide a national standard of best-practice, multidisciplinary care. People living with ALS and their families work with a team of providers who specialize in neurology, physical medicine and rehabilitation, speech therapy, physical and occupational therapy, neuropsychology, respiratory therapy, nutrition and patient advocacy. The care team helps manage disease symptoms and assists people living with ALS to maintain as much independence as possible for as long as possible.

ALS Association-Certified Treatment Centers often provide services that are not readily available in some areas.

A local example is voice banking, which is offered through the ALS Clinic at Virginia Mason. It is a way to preserve the sound of a patient’s own voice to use after real speech is lost. The technique involves a patient recording a variety of phrases and sentences in their own voice. The recordings are then uploaded to a speech-generating device so they can be played back in the voice familiar to family and friends.

Services like voice banking support study results published in the Journal of Neurology Neurosurgery and Psychiatry that showed participation in a multidisciplinary ALS clinic prolongs survival and improves quality of life.

To find an ALS Association-Certified Treatment Center near you, visit ALSA.org/community/centers-clinics.


Taylor_Lynne_2014Lynne P. Taylor, MD, FAAN, FANA, is board certified in Neurology and Neuro-Oncology with subspecialties in Hospice Care and Palliative Medicine. She practices at Virginia Mason Hospital and Seattle Medical Center and is medical director of the health system’s ALS Clinic.

What is Voice Banking?

177425_thumbnailFor people with amyotrophic lateral sclerosis (ALS), also called Lou Gehrig’s disease, losing the ability to speak is only one of the devastating effects. But at the Virginia Mason ALS Clinic, there’s a way to preserve the sound of a patient’s own voice to use later, after real speech is no longer possible.

The technique is called voice banking: the patient records a variety of phrases and sentences in their own voice, then uploads the recordings to a speech generating device — most look like tablet computers — to be played back in the future. Instead of relying solely on the generic synthesized voice supplied with the device, voice bankers can choose to play recordings they made in the voice friends and family are familiar with.

The goal, says Virginia Mason speech pathologist Roberta Kelley, MS, CCC, COM, is to convince ALS patients to make the recordings before it’s too late.

“Of the patients I see who still speak well enough to do it, many won’t want to because they’re not ready to face it,” says Roberta. “I try to tell patients that talking to their spouse, children or even the family pet in their own voice makes a big difference.”

Given that more than 95 percent of ALS patients will eventually use a speech generating device, electing to voice bank could mean a big payoff for many. Newer devices are able to support patients even if they’ve lost the use of their hands, with commands controlled by eye movements. While the ALS Clinic doesn’t supply the devices directly, clinicians like Roberta coach patients on how to make the recordings and work with insurance companies to get the devices people need.

“If we see them early enough and they’re willing, we try to get patients recording right away,” says Roberta.

In the face of a relentless disease, voice banking is one way ALS patients can regain a sense of control. To hear more about how voice banking is helping patients, listen to this recent National Public Radio interview of a Virginia Mason patient and his wife.