Assistive Technology Strategies and Solutions for

People with Degenerative Neurological Disorders

Unlocking the Mind with the Keys of Understanding! Carolyn P. Phillips & Liz Persaud

Tools for LifeGeorgia Assistive Technology Program

phone: 404-638-0390

email: carolynpphillips@mindspring.comemail: lizpersaud@mindspring.com

www.gatfl.org

° AGENDA

Define & Explore the degenerative neurological disease (DND) process:

Considering AT & matching individuals with AT – Success Stories

“Technology gives me hope & I need a lot of

Hope!" ~ Earnistine

Categories of Acquired Disabilities

Permanent - stable – Spinal Cord Injuries  – Traumatic Brain Injuries (TBI) – Cerebrovascular Accidents (CVA/Stroke)

Permanent - degenerative – Amyotrophic Lateral Sclerosis (ALS)* – Multiple Sclerosis (MS) * – Parkinson’s Disease   – Alzheimer’s Disease  – Huntington's chorea (HD)*  – Acquired Immune Deficiency Syndrome (AIDS)   – Friedreich's Ataxia

Cognition

The act or process of knowing or being aware of thoughts or perceptions; the various thinking skills and processes are considered cognitive skills.

Cognitive Ability - Intellectual ability; thinking and reasoning skills.

Cognitive Limitation - Difficulty with one or more of the basic functions of the brain: perception, memory, expression and processing skills.

What is Huntington’s Disease?

Huntington's Disease (HD) is a devastating, degenerative brain disorder for which there is, at present, no effective treatment or cure. HD slowly diminishes the affected individual's ability to walk, think, talk and reason. Eventually, the person with HD becomes totally dependent upon others for his or her care. Huntington's Disease profoundly affects the lives of entire families: emotionally, socially and economically. This hereditary disorder in 1872, HD is now recognized as one of the more common genetic disorders. More than a quarter of a million Americans have HD (40,000) or are "at risk" of inheriting the disease from an affected parent. HD affects as many people as Hemophilia, Cystic Fibrosis or muscular dystrophy.

What is a Spinal Cord Injury?

Spinal Cord Injury (SCI) is damage to the spinal cord that results in a loss of function such as mobility or feeling. Frequent causes of damage are trauma (car accident, gunshot,  falls, etc.) or disease (polio, spina bifida, Friedreich's Ataxia, etc.). The spinal cord does not have to be severed in order for a loss of functioning to occur. In fact, in most people with SCI, the spinal cord is intact, but the damage to it results in loss of functioning. SCI is very different from back injuries such as ruptured disks, spinal stenosis or pinched nerves.

A person can "break their back or neck" yet not sustain a spinal cord injury if only the bones around the spinal cord (the vertebrae) are damaged, but the spinal cord is not affected. In these situations, the individual may not experience paralysis after the bones are stabilized.

http://www.spinalinjury.net/

Stages & Symptoms for HD Early Stage - Symptoms of Huntington's Disease may affect

cognitive ability or mobility and include depression, mood swings, forgetfulness, clumsiness, involuntary twitching and lack of coordination.

Mid-Stage - As the disease progresses, concentration and short-term memory diminish and involuntary movements of the head, trunk and limbs increase. The most common involuntary movement is chorea*. Walking, speaking and swallowing abilities deteriorate.

Late Stage - Eventually the person is unable to care for him or herself. Death follows from complications such as choking, infection or heart failure.

– info gathered from HDSA

More info about HD & HOPE! HD typically begins in mid-life, between the ages of 30 and

45, though onset may occur as early as the age of 2. Children who develop the juvenile form of the disease rarely live to adulthood. HD affects males and females equally and crosses all ethnic and racial boundaries. Each child of a person with HD has a 50/50 chance of inheriting the fatal gene. Everyone who carries the gene will develop the disease.

In 1993, the HD gene was isolated and a direct genetic test developed which can accurately determine whether a person carries the HD gene. The test cannot predict when symptoms will begin. However, in the absence of a cure, some individuals "at risk" elect not to take the test.

Exciting Research Update! February 15, 2008, News on Therapeutic Compounds (Irvine, CA) According to the University of California at Irvine’s Dr. Leslie Thompson and her

collaborator, Larry Marsh, have tested compounds shown “in fruit flies and mouse models to suppress HD neurodegeneration.” Scientists use “low doses of each single drug that together provide an even greater benefit while minimizing toxic side effects. The use of a Drosophila [fruit fly] model of HD to test possible therapeutic compounds allows us to rapidly assess potential treatment regimens prior to testing in mammalian models with great savings in cost and time. These results also raise the possibility that these particular combinations or other similar types of compounds could prove effective in future human trials.”

For more information, visit the website of National Academy of Sciences at www.nas/edu or consult the Proceedings of the National Academy of Sciences at www.pnas.org.

Exciting Update! January 31, 2009: News on Drugs that may delay the onset or slow down

HD (Dallas, TX and Vancouver, BC) Dr. Michael Hayden, Dr. Ilya Bezprozvanny in collaboration with Dr. Rodolfo

Llinas and Dr. Bruce Kristal have discovered that drugs commonly used to treat psychiatric illnesses and blood disorders in humans may protect the brain cells that die in people with HD, thus delaying the onset and slowing the progression of the disease.

Drs. Bezprozvanny and Hayden found that while using the medium spiny neurons of mice that carry a copy of the mutated human huntingtin gene, treatment of the cells in culture with the drug Enoxaparin prevented inappropriate calcium release and prevented cell death.  Enoxaparin (also sold as Lovenox) is an anti-coagulant that is FDA-approved in humans for use in treating blood clots. Dr. Llinas made the initial discovery that Enoxaparin acts as a calcium blocker and provided the team with Enoxaparin samples for the studies.

 

GINA

What is Muscular Dystrophy (MD)?

Muscular Dystrophy (MD) is defined as a group of genetic (inherited) degenerative disorders that affect the muscles. MD causes the muscles in the body to atrophy and progressively weaken. The muscles break down and are replaced with fatty deposits over time. In some forms of muscular dystrophy, cardiac and smooth muscles are affected. The muscular dystrophies are the most known hereditary diseases.

Info from MDA

Types of Neuromuscular Diseases recognized by the Muscular Dystrophy Association (MDA)

There are currently 8 different categories of neuromuscular diseases recognized by MDA.– MUSCULAR DYSTROPHIES – MOTOR NEURON DISEASES – INFLAMMATORY MYOPATHIES – DISEASES OF THE NEUROMUSCULAR JUNCTION – MYOPATHIES DUE TO ENDOCRINE ABNORMALITIES – DISEASES OF PERIPHERAL NERVE – MYOPATHIES – METABOLIC DISEASES OF MUSCLE

There are 43 TOTAL different types of neuromuscular diseases recognized worldwide.

Info from MDA

More about MD & HOPE! Onset, symptoms, stages of muscle atrophy,

progressive weakness and life expectancy are all determined by what form of neuromuscular disease is diagnosed.

Disease onset can range from 2 months old to well into adulthood.

Life expectancy depends on severity of neuromuscular disease and the how severe the respiratory and cardiac muscles are effected.– Can range from birth to adulthood

Doctors are no longer making predictions about life expectancy due to earlier diagnoses, life long therapy and the increased use of AT.

Info from MDA

Research & Exciting News for MD!

MDA is the world's largest non-governmental sponsor of research seeking the causes of and effective treatments for neuromuscular diseases, sponsoring some 400 research projects annually.

In 2005 & 2006, there have been successful breakthroughs in research for Duchenne MD, Spinal Muscular Atrophies, Friedreich’s Ataxia, Myotonic MD and much more!

Info from MDA

Exciting Research Updates! ‘Read-Through’ Drug in Development for

Duchenne Muscular Dystrophy – A drug that causes cells to “read through” genetic

errors thought to affect some 15 percent of children with Duchenne muscular dystrophy is shown safe in healthy volunteers.

Aminoglycosides Coax Cells With SMA to make Needed Protein– Drugs known as aminoglycosides boost levels of

the needed protein SMN in cells carrying mutations that cause spinal muscular atrophy. SMN, missing in SMA, is necessary to allow nerve cells to survive and function.

Info from MDA

What is Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS) is a fatal, neurodegenerative disease that attacks nerve cells and pathways in the brain and spinal cord. When these cells die, voluntary muscle control and movement dies with them. People with ALS in the later stages of the disease are totally paralyzed, yet in most cases, their minds remain sharp and alert.

ALS struck Yankee Hall of Famer Lou Gehrig in 1939. He died two years later, but ALS is still commonly known as Lou Gehrig's disease.

More Info about ALS & HOPE The facts about ALS are sobering: Every day, an average of 15 people

are newly diagnosed with ALS - more than 5,600 people per year. As many as 30,000 Americans may currently be affected by ALS. The average life expectancy of a person with ALS is two to five years from time of diagnosis.

There is no known cause or cure, but with recent advances in research and improved medical care, many patients are living longer, more productive lives.

In keeping true to the spirit of the game Gehrig loved best, ALSA seeks to covers all the bases - research, patient and community services, public education, and advocacy - in providing help and hope to those facing the disease.

This & more info can be found at www.alsa.org

What is Multiple Sclerosis (MS)?

Multiple sclerosis (MS) is a life-long chronic disease diagnosed primarily in young adults. During an MS attack, inflammation occurs in areas of the white matter of the central nervous system (nerve fibers that are the site of MS lesions) in random patches called plaques.

Physicians use a neurological examination and take a medical history when they suspect MS. Imaging technologies such as MRI, which provides an anatomical picture of lesions, and MRS (magnetic resonance spectroscopy), which yields information about the biochemistry of the brain. No single test unequivocally detects MS. A number of other diseases produce symptoms similar to those seen in MS.

This and More info found at the MS Society web site.

Symptoms of MS Symptoms of MS may be mild or severe and of long duration or

short and appear in various combinations. The initial symptom of MS is often blurred or double vision, red-green color distortion, or even blindness in one eye. Most MS patients experience muscle weakness in their extremities and difficulty with coordination and balance. About half of people with MS experience cognitive impairments such as difficulties with concentration, attention, memory, and judgment.

The cause of MS remains elusive, but most people with MS have a normal life expectancy. The vast majority of MS patients are mildly affected, but in the worst cases, MS can render a person unable to write, speak, or walk.

More Info about MS & HOPE There is as yet no cure for MS. Until recently, steroids were the

principal medications for MS. While steroids cannot affect the course of MS over time, they can reduce the duration and severity of attacks in some patients. The FDA has recently approved new drugs to treat MS. The goals of therapy are threefold: to improve recovery from attacks, to prevent or lessen the number of relapses, and to halt disease progression.

What is a Direct Brain-Computer Interface?

… a system that captures signals directly from the human brain, providing a channel to control computers and other devices.

The GSU BrainLab Missionis to pioneer real-world applications research for biometric technologies to improve the quality of life for people with severe disabilities, and to explore mainstream applications.

Brain Signal Detection Techniques

Invasive: implanted electrodes (single neuron)

Noninvasive: scalp electrodes (EEG)

Restoring Motion - Neural ProstheticsBrain “re-learns” how to move limbs via an artificial nervous system

Simulation

• Virtual reality hand

Restoring Physical Motion

• Robotic arm

The “Aware ‘Chair”

Integrated communication and environmental control

• Intelligent, neurally controlled wheelchair

• Conversation and environmental control prediction

• Learns users habits and context

• Provides emotional expression

Considering Assistive Technology: What Works for the Individual

Why Assistive Technology?

For a person without a disability, technology makes things easier….

For a person with a disability, technology makes things possible.

Assistive Technology Matching: A TEAM APPRAOCH

² The Person with the disAbility

² Circle of Support – Family of Choice

² Case Managers

² Technologist

² Occupational Therapist

² Speech & Language Pathologist

² Physical Therapist

² Engineer

Human Activity Technology (HAAT) Model

Human: represents the skills and abilities of the person with a disability

Activity: a set of tasks to be performed by the person with a disability

Context: the setting or social, cultural and physical contexts that surround the environment in which the activity must be completed

Assistive Technology: devices or strategies used to bridge the gap between the person’s abilities and the demands of the environment

Developed by Cook & Hussey

Promoting Strengths & Managing Weaknesses

A Holistic Approach

Selection of an AT Device

Depends on an analysis of the following conditions:

² Prior experience or knowledge, and interests;

² The individual’s specific strengths, weaknesses;

² The specific device (reliability, ease of operation, technical support, cost)

² Usefulness across settings

² Usefulness over time as symptoms manifest!

Most Effective Technological Products Able to level out the playing field Work in cross-settings Portable Easy to maintain Affordable to replace and/or maintain Good and reliable technological support Accessible training format

Staying off the FAST Track! ² Frustration ² Anxiety ² Stress ² Tension

Success Stories of AT Solutions

A Closer Look at: HD ALS MS

Meet Earnistine!

Meet Earnistine! Age: 34 Diagnosis: HD affecting Motor Skills and some Cognitive - primarily affecting

reading, writing and memory. Speech is affected also. Earnistine is the third child from a large African-American family. She was born and raised

in a small southern town. Her father & grandmother both died from HD. One sister had HD & took her own life. The other is symptom free.

Earnistine graduated from college & was gainfully employed with the Decatur Police Department. Earnistine was diagnosed with HD 8 years ago and was “released” from DPD 5 years ago. Earnistine lived independently in her own home until 3 years ago – now lives with her mother.

At this point, Earnistine wants to use her computer to “explore the world” & “talk with her friends” via e-mail. She also wants to go to the local grocery store and movies & communicate independently. She also has a difficult time remembering the day and time.

Earnistine’s reasoning and logic skills seem to be strong. She has a great zest for living & wonderful sense of humor.

How would you work with Earnistine to help her?

AT Success Story I

AT Solutions for Earnistine!

What Worked Communication Device

³ Hip Talker Memory of Day & Time

• Talking Watch• Talking Clock

Reading³ WYNN

Writing³ Writing

Guides

³ Big Check Book & Address Book

³ WYNN

What Did NOT Work

Communication Device

Low Tech BooksHeavier more complicated devices

Reading

Audio Books

Plan for the Future…

Talking Watch

Talking Watch helps students tell accurate time

Look for easy to read LCD display

Alarm Come in many

different styles Low in cost

WATCH MINDERLD OnLine or 800-961-0023

Assist with A.D.D and LD

Program as medication reminder

Large Screen Easy to use $99.50

Hearit

Increased phonological awareness for all auditory input-results in better focus and comprehension

Noticeably reduces distractions - task!

“Only as high as I reach can I grow, only as far as I seek can I go, only as deep as I look can I see, only as much as I dream can I be.”

~ Karen Ravn

Meet Miss V! Age: 45- ish Diagnosis: ALS affecting Motor Skills and some Cognitive - primarily

affecting writing. Speech is affected also. Fatigue is a factor. Miss V is an only child. She was born and raised in a small mid-western town. Miss V graduated from college & is gainfully employed with the DOL. Miss V

was diagnosed with ALS 12 years ago. Miss V drives her own modified van & lives independently in her own home.

At this point, Miss V wants to use her computer for a longer period of time & with less effort. She also wants to be able to be heard in meetings & communicate independently.

She is a strong advocate. She loves life & has a sassy of humor. How would you work with Miss V to help her?

AT Success Story II

AT Solutions for Miss V! What Worked Voice Amplification Device Hear IT

Reading³ WYNN

Writing³ Reach

What Did NOT Work

Communication Device

Voice Input

REACH Interface Author Applied Human Factor, Inc.888-243-0098

On-screen keyboard Seven years of R&D Word/sentence

completion Speech augmentation Scanning keyboard

options Automatic window

management 100+ built-in keyboards 10 built-in assessment

tools

“Two prisoners look out through the bars of their cell – one sees mud, the other sees Stars!”

~ Abe Lincoln

Meet Mark! Age: 29 Diagnosis: MS affecting Motor Skills and some Cognitive - primarily

affecting reading & writing. Speech is now affected also. He also has extreme fatigue.

Mark is the oldest child of four children. He was born and raised in Atlanta. He was diagnosed while in college at the age of 20. His initial episode with MS left him paralyzed for 3 months. He has lost his vision & his ability to speak – has regained both. He had a stroke last March & has been struggling to regain his strength.

Mark is a very talented computer technician. He is not able to work at this time. He now lives with his mother.

Mark wants to use his computer for a longer period of time. He also wants to go work on computers independently.

How would you work with Mark to help him?

AT Success Story IV

AT Solutions for Mark!

What Worked ZoomText TextHelp Modified & Adjustable

Workstation Arm Rest Head Mouse

What Did NOT Work

Voice Input

Plan for the Future…

Speech Recognition Software Turn spoken words into

text Connect with the timing

of your thoughts Dictate eyes-free, with

out watching for errors or stopping to make corrections

Dictation speed 70 to 100 words per minute

Getting to Work

Handheld AT

T-Mobile SidekickFeatures: Full Color Screen Mobile Snapshots - camera, snap a shot and send it via e-mail. AOL Instant Messenger ™ Service - Buddy List E-Mail - set up as many as three POP3 email accounts Captures images up to 240 x 160 pixels Includes Carrying Case and hands-free headset Full-featured mobile phone Game arcade - An organizer! Synchronize wirelessly to Desktop Interface. Web browser $104 

Job Accommodation Network

US Department of Labor’s Office of Disability Employment Policy

www.jan.wvu.edu

Resources

HDSA www.hdsa.org ALS www.alsa.org MS www.mss.org Job Accommodation Network www.janweb.icdi.wve.edu Technology Act Projects www.resna.org Ga Assistive Tech: Tools for Life www.gatfl.org Ga Project for Assistive Technology www.gpat.org Center AT & Environmental Access www.catea.org

The Starfish There was a young man walking down a deserted beach just before dawn.

In the distance he saw a older woman appear to be dancing.

As he approached the woman, he saw her picking up stranded

starfish and throwing them back into the sea. The young man gazed in wonder as the woman again and again

threw the small starfish from the sand to the water. He asked, “Why do you spend so much energy doing what seems to be a waste of time.”

The woman explained that the stranded starfish would die

if left in the morning sun. “But there must be thousands of beaches and millions of starfish!”

exclaimed the young man. “How can you make any difference?”

The woman looked at the small starfish in her hand and as she threw

it to the safety of the sea, she said,

“It makes a difference to this one!”.

For Your Time & Interest!

We Want to Hear from You!