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FUNCTIONING WHEN MOBILITY
IS AFFECTED BY ALSNanette C. Joyce, DO
University of California, Davis School of Medicine
andCharlotte Chatto, PT, PhD
Augusta University ALS Center

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A note to the reader: The ALS Association has
developed the Living with ALS resource guides
for informational and educational purposes only.
The information contained in these guides is
not intended to replace personalized medical
assessment and management of ALS. Your
doctor and other qualified health care providers
must be consulted before beginning any
treatment.

Living with ALS
Functioning When Mobility is Affected by ALS
Copyright © 2017 by The ALS Association.
All rights reserved.

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TABLE OF CONTENTSINTRODUCTION . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7-4
UPPER MOTOR NEURON (UMN) VS. LOWER MOTOR NEURON (LMN)
PREDOMINANCE . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7-4
BEING PROACTIVE . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7-6
YOUR MOBILITY TEAM: PHYSICAL AND OCCUPATIONAL THERAPISTS . . . 7-7
CONSERVATION OF ENERGY . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7-9
ACTIVITIES AND AIDS FOR DAILY LIVING . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7-10
EXERCISE, FLEXIBILITY, AND STRETCHING . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7-17
DURABLE MEDICAL EQUIPMENT AND ASSISTIVE DEVICES . . . . . . . . . . . . . 7-18
HOME ADAPTATIONS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7-22
SUMMARY STATEMENT . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7-23
BIBLIOGRAPHY . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7-24

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INTRODUCTION
ALS is a disease of the motor neurons: those that make up the chain of nerve
cells starting from the brain, that travel through the spinal cord (Upper Motor
Neurons—UMNs), and ending with nerve cells that project from the spinal
cord to the muscles (Lower Motor Neurons—LMNs). The LMNs provide signals
for muscle contraction and move our bodies through space, as we demand.
Defining this distinction between the types of motor neurons is important when
discussing mobility and ALS because the loss of UMN or LMN will cause uniquecorresponding symptoms and related mobility impairments. Although various
changes in mobility will occur and the challenge of everyday movements will
increase over time, there are many types of equipment and devices that can beused to help maintain independence for as long as possible. For example, one
person with ALS described his experience in the following way:
“ALS is a disease of daily discovery… each day I awaken to discover those things
I can’t do today that I could do yesterday.”But, this individual approached each day creatively, adjusting his technique andtools so that he could continue to participate in the activities that he loved.
He loved to fish. When he lost the function of one of his hands, he fashioned
a fishing pole that did not need both hands to control. When he could nolonger stand, he used his power wheelchair to go to the docks. When he could
no longer hold his pole, he attached it to his wheelchair. His spirit for pursuingwhat he loved could not be taken away from him. He spent his time focused on
maintaining a life filled with meaning and joy.
What we will cover in this resource guide:■ UMN Versus LMN symptoms and impairments■ Taking a proactive approach■ Team members required to address constant decline in mobility■ Energy conservation■ Activities and aids for daily living■ Exercise, flexibility, and stretching■ Durable medical equipment and assistive devices■ Home adaptions

UPPER MOTOR NEURON (UMN) VERSUS LOWER MOTOR NEURON(LMN) PREDOMINANCE
People with UMN predominant ALS develop stiffness in their muscles, also
known as spasticity. Spasticity is defined as an increased tension in the muscle
that depends on how fast the muscle is stretched; the faster a muscle isstretched the greater the involuntary resistance in the muscle becomes. Thisis due to an imbalance in the signals that influence the spinal cord and control
the muscles’ responses to being stretched. In healthy individuals, coordinatedmovements require some muscles to contract while others relax. In spasticity,

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this coordination is impaired. People with spasticity lose fluid, coordinatedmobility of the limbs. Imagine, for instance, driving a car while applying both
the gas pedal and the brakes. As you press faster and harder on the gas you alsopress faster and harder on the brakes. Strangely, you do not know which action,
stopping or going, will prevail. Driving under these circumstances could be jerky,
unpredictable, and unsafe.
When you have a spastic muscle from UMN disease, your response toenvironmental disturbances is impaired by your inability to respond with rapid,
instinctual responses. For example, a person with spastic leg muscles will walkwith a stiff, jerky, and dis-coordinated gait pattern. If during an afternoon stroll
this person catches their toe on a crack in the sidewalk and stumbles, they will
instinctively attempt to right themselves but will instead respond with spastic
stiffening of their legs and likely fall. If their upper limb muscles are also spastic,
they will not be able to coordinate the protective response of reaching their arms
out to slow their contact with the ground and protect their head from injury.
On the other hand, those with LMN predominant ALS have muscle weakness at
the core of their mobility impairment. Lower motor neuron loss causes muscles
to weaken and shrink, becoming smaller and less powerful, until they are nolonger able to signal muscle contraction. Progressive LMN loss in ALS eventually
results in paralysis of all skeletal muscles of the body.
To meet the clinical diagnostic criteria for ALS, a person will necessarily haveevidence of both UMN and LMN abnormalities. ALS, however, is quite varied
from person to person. Each person presents with a unique pattern of signs
and symptoms, and a combination of spasticity, muscle atrophy, and weakness.
Addressing your ongoing mobility needs requires frequent assessments andis best handled in collaboration with your care team, who are willing to beendlessly creative in their approach to problem-solving. Table 1 lists someprimary reasons why mobility might be affected.
Table 1: Primary Reason Why Mobility Might Be Affected
Muscle weakness in limb(s)Paralysis occurs in some muscles, while other muscles may be weak or not affected
Spasticity (involuntary spasms and stiffness in muscles)
Painful muscle cramping
Loss of range of motion and flexibility, especially in the shoulders, hands, and ankles

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BEING PROACTIVE
One of the things that is pressed upon you at ALS support group is to always stay ahead of the curvewhen making decisions. You don’t want to make decisions after you need something; instead, makethese in advance so that you are not caught off guard. This comes from folks who know and who’vebeen there.
Cheryl Timmons, caregiver(Contributed by The ALS Association Greater Sacramento Chapter)
While the details of each person’s story will be unique, there are more similarities
than differences from one person with ALS to another. Experts who focus theirpractices on caring for people with ALS develop a deep understanding of how
ALS symptoms spread and change. In this way, they are able to predict andanticipate the mobility and Activities of Daily Living (ADL) needs prior to majortransitions, thereby avoiding crises. For instance, if your legs become weak, youwill begin to have difficulty standing up from a low-seated position. Instead of
waiting until you are unable to stand from the toilet (which are most often low
in height) and require a visit from the emergency response crew to transfer you,the perceptive practitioner will have anticipated this problem and provided therecommendation in advance to obtain an elevated toilet seat with handrails toenable transfers on and off the toilet.
One of the goals of the ALS rehabilitation team is to help each individual remain
as independent as possible for as long as possible. Just as a construction crew ismore capable of completing a job when they have the right tools, you can remain
more functional and have prolonged independence if you have the right tools,
provided at the right time, to help with declining function.
Ultimately, you have a choice as to whether you will approach your care in aproactive or reactive manner. Taking a proactive approach provides everyone
involved in your care extra time for problem-solving and can help you avoid
the stress of being stuck without options, struggling to get by when you havesuddenly lost an ability. It’s best if you ask your treatment team to provide
recommendations for equipment in advance of your need. Obtaining what
they recommend and putting it away for the day it is needed can help you feelassured you are prepared. Getting the appropriate piece of equipment canrelieve stress. People often delight in their newly found freedom when theyreceive a power wheelchair and realize they are able to resume community
activities that were given up due to intolerable fatigue or difficulty walking withfear of falling. Remember that tools are used in every trade to improve the abilityof the people doing the work. ALS should be no different.

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YOUR MOBILITY TEAM: PHYSICAL AND OCCUPATIONAL
THERAPISTSThe ALS rehabilitation team includes both physical and occupational therapists.
The roles of the physical and occupational therapist are to address mobility
problems including muscle weakness, spasticity, and contractures that cause
impairments affecting a person’s ability to walk, to perform transfers such as
moving from a reclined to a seated position or a seated to a standing position,and to improve abilities to dress, bathe, groom, and feed themselves. These
therapists provide recommendations and treatment to improve function either
by initiating corrective one-on-one therapy or by recommending and teaching
how to use assistive devices. They design and teach focused range of motion
exercises, and ensure that individuals with ALS and their caregivers have the skill
to perform these independently in the home or community setting.

Who to See for What
The ALS Physical Therapist (PT) focuses on gross motor activities such as walking,sitting balance, and lower limb range of motion, strength, and balance. They
provide recommendations for braces, canes, walkers, standers, scooters, andwheelchairs (Figure 1).
A single-point cane or walking stick may
be recommended if help on one side onlyis needed. A quad cane, with four prongs
as the base, provides more stability than acane, but less than a walker.

If you are experiencing falls,
tripping while ambulating,fatigue with walking long
distances your physicaltherapist will check yourbalance and gait and makerecommendations for an
assistive device to makesure you are safe.

A rolling walker (with or without a seat)
may be recommended if help is neededon both sides.

In addition to a walker, powered mobility,
such as power wheelchair, may berecommended to allow for potentialchanges in ability to move.

An Ankle–Foot Orthosis (AFO) is generally
recommended if you have a lot of ankle
fatigue during walking and this leads tofalls, or if you have trouble lifting yourfoot, which may lead to falls.
Figure 1: Physical therapist recommendations for increased mobility.

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The ALS Occupational Therapist (OT) provides assessment and treatment forthe skills needed to perform activities of daily living. They focus on their client’s
ability to transfer, dress, bathe, groom, toilet, and feed themselves. They assess
upper limb range of motion and strength and provide recommendations forupper limb and neck bracing. In addition, they perform home safety evaluations
and also are able to perform wheelchair assessments. Together, the PT and OT
help families negotiate the transition of their loved one from independence
to dependence, providing caregiver training, teaching transfer techniques, andoffering recommendations for durable medical equipment and assistive devices.
In addition to interacting with therapists, you may also need the services of an
orthotist, who fabricates, fits, and maintains braces (Figure 2); and a durable
medical equipment provider, who provides and maintains wheelchairs, transfer
equipment, bath equipment, and hospital beds.

Figure 2: Example of bracing options to stabilize the wrist and head.

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CONSERVATION OF ENERGYAs the disease progresses in ALS, individuals often struggle with the feeling of
fatigue. Using your energy for those activities that matter most and applying
energy conservation techniques for other tasks can be a helpful strategy for
maintaining a schedule that is not too tiring. Both PTs and OTs are trained toprovide recommendations and teach energy conservation techniques.
The following are a few examples of conservation strategies:■ Rearrange your environment:

1. Unclutter rooms and have furniture arranged to improve access.
2. Reduce the need to go up and down stairs by moving a bedroom to themain floor used for daily living.
3. Keep regularly used items in easily accessible places.
4. Replace heavy items with lighter weight versions (e.g., plastic plates andcups as opposed to stoneware and glass).
■ Eliminate unnecessary activities and prioritize:

1. Eliminate activities that are not important for you to do and delegate
those that are important to family and friends.
2. If possible, consider hiring help for completion of chores around thehouse.
3. Sit rather than stand while performing tasks.
4. Use a rolling walker with a seat or a wheelchair with a tray to help carryobjects through the house.
■ Plan ahead:

1. Plan enough time for activities to reduce the extra energy demandrequired when rushing.
2. Schedule rest breaks in the middle of planned activities.
3. Call ahead to make sure that everything you will need is available when
shopping, etc.
4. Have meals made ahead and packaged into easy-to-prepare servings.
There are many other ways to conserve energy. A creative PT or OT can help you
look for ways to modify your home and activities to reduce fatigue. Attending
support groups or participating in online conversations with other people livingwith ALS is another rich resource for solving mobility challenges.
Here are some ways you can plan and prioritize:■ If you know that you are going to have to walk a long distance, plan forplenty of time and rest breaks.

■ If you can sit to take a shower, instead of stand, make sure you have abathtub seat.

■ If you are going to prepare a meal, make sure you get all of the items youneed before you begin.

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■ Think of ways that you can slide, push, or pull items, rather than carrythem.

■ If you have more energy in the morning, do those activities that are mostimportant to you at that time.

ACTIVITIES AND AIDS FOR DAILY LIVING
There is a long list of Durable Medical Equipment (DME) and assistive devices
that are potentially helpful. The goals for using DME and assistive devices are to
improve function, prolong independence, and maximize safe caregiving once a
person has transitioned to dependence. Activities of daily living include dressing,bathing, grooming, toileting, bed mobility, transfers, and feeding oneself. Each
person’s needs differ depending on the extent and location of their weakness.
For example, if your arms are weak and lifting your hand to your face becomeschallenging, elevating the arm above the waist by resting the elbow on a table,tray, or other elevated surface will improve arm function for eating, brushingteeth, or grooming as you eliminate much of the work done against gravity.
Consulting with your OT on a regular basis and exploring online or catalog
resources can provide options for commonly encountered problems.

Devices for Eating, Brushing Teeth, Bathing, Grooming, and Dressing
EatingWhen the hands become weak, it is easier to hold tools that have wide handles
and are lightweight (Figure 3). Using plastic or rubber utensils is lighter weightand easier to do than metal utensils and can extend a person’s ability to eat
independently. As grip strength weakens, holding objects can be made easierby building up the thickness of handles. This can be accomplished simply byusing foam tubing that can be cut to size and slipped over the handle, or you canpurchase ready-made utensils with widened rubber handles. Once a person loses
the ability to grasp but still has the ability to bend their elbow and bring theirhand to their face, a universal cuff that slips over the hand and attaches to theneeded tool is useful. A universal cuff can be attached to grooming tools as well
as utensils.

Brushing Teeth
Electric toothbrushes make brushing teeth easier, as the handles are generally
wider than a standard toothbrush and the user does not need to exert as muchenergy to scrub their teeth. For the person with bulbar dysfunction or those whoare dependent for their care, there are suction toothbrushes available that wickaway water and saliva as the teeth are cleaned (e.g., Plak-Vac® by Trademark
Medical). Electric razors are easier to use to maintain facial hair.

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Figure 3: Utensils and tools to assist with eating.

Showering and Bathing
Aides for showering and bathing include grab bars, long handled scrub brushes,
hand-held shower heads, sliding tub and shower benches, and rolling shower
chairs (Figure 4). Shower chairs and sliding tub benches should be purchased witha backrest high enough to support the head and with a seat belt. These features
are needed in the later stages of the disease, when there is no longer good head
and torso control when sitting.

Dressing
Clasps and closures can become difficult to manage. As fine motor control
worsens, buttonhooks and zipper loops can be used to facilitate buttoning
buttons and zipping zippers. Other dressing strategies include switching fromtailored clothing to loose fitting clothes or clothes made of stretch fabricFunctioning When Mobility is Affected by ALS

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Figure 4: Equipment to assist with bathing.

(e.g., Lycra®) with elastic waistbands and loose pullover shirts. Socks can bedonned more easily using a sock assist tool. Long-handled shoehorns minimize
the work needed to reach down and put on a pair of shoes. Fabric shoe closures(e.g., Velcro®) or magnetic shoe closures (Zubits®), or coiled no-tie shoelaces canmake fastening a pair of shoes much easier than having to tie shoelaces(Figure 5). For dressing, a quick internet search using the term “adaptive clothing”
can provide multiple resources and options for improving the ease of dressing.

Moving Around and Getting in and out of Bed
There are three major aspects of bed mobility that will need to be addressed
during the course of the disease: getting in and out of bed, moving in bed, andbody positioning while in bed.

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Figure 5: Resources for dressing: curly shoelaces, long-handled shoehorn, button assist (top left),sock assist (bottom left) and grab bar (right).

Getting in and out of Bed
To improve the ability to stand from and sit down on the edge of a bed in theearly stage of ALS, risers can be added to the legs of the bed to bring it to aheight that is easier from which to stand. Adding impermanent adjustable bed
rails or a transfer pole that extends from the floor to ceiling next to the bed canprovide a solid purchase for the upper limb to assist with standing. When you
are no longer able to get into and out of bed, a transfer lift system should be
used for safe and easy caregiver supported transfers. Lift systems come in manyvarieties, powered by hydraulic or electrical means, and can be mobile units orpermanent systems that are attached to the ceiling (Figure 6). These lifts use alarge sling that wraps under and around the person being lifted and hooks to
the machine (Figure 7). The system then lifts and supports the individual’s entire
weight, allowing them to be moved easily into a wheelchair, onto a commode, orFunctioning When Mobility is Affected by ALS

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Figure 6: A gait belt is used to transfer people from one position to another.

into and out of bed safely. An OT or PT should be asked to provide your family
and caregivers with training on how to use the lift system when it is ordered.

Moving in Bed
It is important to be able to move in bed, whether by your own efforts or theefforts of others. A person with normal strength can naturally move throughout
the night while sleeping, repetitively repositioning themselves for comfort. As
you weaken, moving in bed can become impossible. For skin health and to avoid
pressure-related wounds, a person who is unable to independently adjust theirposition in bed requires repositioning at least every two hours. Draw sheets
are helpful for repositioning by putting the caregiver at a greater mechanicaladvantage (Figure 8). One option is a two-draw sheet system where a circularpiece of material that is slippery is placed on top of the bottom fitted sheet. This
provides a surface that has less friction for sliding a person back and forth overthe bed. This sheet is then covered with a second unfitted cotton draw sheet.

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Figure 7: Hoyer Lifts allow a person to
be lifted and transferred with minimal
physical effort.

Figure 8: Draw sheet used for repositioning in bed.

The top sheet is used as the pulley that moves the person in the bed. When you
use a draw sheet, the top draw sheet should be placed at the person’s shoulders
and span to below their hips. The draw sheet should be rolled up close to thebody of the person who is being moved, creating a handle to grab. Caregivers
should always roll the person being moved towards them and use their legs
instead of their back for transfers.

Positioning While in Bed
Pillows, pillows, and more pillows should be available to support comfortable
positions. If the person in the bed is on their side they will need to have cushion
support under their ribs so they are not lying directly on their shoulder, which
can become painful. Pillows should be used to support limbs so they are notdangling. Even while lying on one’s back, the immobile person will need pillow
support for under their shoulders and arms.
Hospital beds provide a convenient way for positioning. There are fully electricand semi-electric beds. Medicare typically will cover a semi-electric hospital
bed. These beds have electric hand controls that allow the head of the bed to belowered and lifted and the knees of the bed lowered and elevated, by a push ofa button. Elevating the head of the bed to 30 degrees helps improve respiratory
performance during the night. The semi-electric bed is equipped with a crank toraise and lower the height of the entire bed. The fully electric bed does this with
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the electric hand controls. Changing the height of the bed is helpful for both the
caregiver and person in the bed, enabling better biomechanics for bedside care.
Hospital beds come with standard hospital mattresses, which are not very
comfortable for most people. Alternating air pressure relief overlays or pressure
relief mattresses are usually more comfortable. They reduce the frequency
with which a person with ALS needs to be repositioned. These mattresses arecontinually shifting the pressure applied to the body, thereby reducing the
likelihood of developing pressure-related wounds. It can take time to find amattress that is soft enough for comfort and firm enough to enable bed mobility.

Personal Hygiene Challenges
Do not underestimate the importance of developing a plan to address needs
related to personal hygiene and toileting. This can be one of the most distressing
problems for you. It is a fact of life that what goes in must come out. Between
the ages of two to three years, we take control of this part of life and it remains a
private personal issue until we are challenged by our mobility and can no longer
meet our own toileting needs. While it is a good strategy to perform timed
toileting, scheduling bathroom breaks every four or so hours during the day toprevent accidents, there will always be some randomness to our urges that makeour needs somewhat unpredictable. Of note, it is not a good strategy to avoid
drinking fluids to avoid needing to use the bathroom. This behavior leads todehydration and constipation, both of which cause discomfort and are problemsof their own.
This transition will occur when you have sufficient weakness limiting your ability
to get on and off the toilet, or you do not have the upper extremity mobility
and coordination to wipe yourself clean (medical term: perform perineal care).
Accidents may happen if you cannot make it to the bathroom in time. While
there are adult diapers for protection when accidents occur, sitting in a wet orsoiled diaper for an extended period of time will lead to painful rashes andincrease the risk of developing pressure-related sores if habitual.
So what can you do? Bedside commodes improve the convenience of toiletingby lessening the distance needed to travel through the house to use the toilet. In
addition, they remove the transfers required for toileting out of a small bathroom
into a wide-open space. Their height can be adjusted and they provide handrails,
which improve your ability to stand from a seated position, prolonging the ability
to get on and off the toilet without help. Another option is a rolling shower chair/commode combination that can be rolled into the bathroom and backed in over
the toilet. Again, this equipment removes the dependent transfer out of a small
space.
Actual examples of choices made by two persons with ALS:
Example 1. A woman with ALS declared that she would move into a skilled
nursing facility as soon as she could no longer clean herself after toileting. She
did not want to burden her children with her perineal care. Her weakness began
in her upper limbs. Early into her disease she lost the ability to wipe herself clean;

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her legs, however, were still very strong and she was able to walk and transfer
with ease. She was still considering moving away from her family, the people she
loved the most and who brought her the most joy. The OT recommended she
consider getting a bidet, a tool to help with toileting, which she did. Her distress
was relieved and she remained with her family.
Example 2. Another woman with ALS had psychosocial distress when out inpublic related to toileting challenges. Maintaining an active social life was very
important to her; however, she felt deterred by fears of needing to use therestroom in public. That her husband would have to accompany her into a public
women’s restroom and then transfer her to the toilet in a stall with limited spaceand of questionable cleanliness was overwhelming. She searched for a solution
and decided she wanted to have a suprapubic catheter. A suprapubic catheter
is a permanent tube inserted through the skin of the lower abdomen into theurinary bladder. The catheter tube connects to a bag that is attached to theleg and collects urine as it drains from the bladder. After a lengthy discussion
regarding the cons of having the procedure, she insisted on a referral. She
maintained, without question, that it was the best decision she made.
Men have another option that is less permanent: a condom catheter. There aremany different styles of catheters but all need to be sized to fit well. A physician
will need to provide a prescription for monthly supplies that include the condom,tubing, and a urine collection bag. Baseball season is a popular time of year
for requests for condom catheters. In addition, men can use a urinal and avoid
having to transfer out of a wheelchair or bed during the night to urinate, as longas they have adequate hand function or a caregiver who provides assistance.
For younger women with ALS, menses can be troublesome. If there is no desire
for pregnancy, extended and continuous cycle oral contraceptives that reduce
the number of menstrual cycles may be the best solution. This strategy should be
discussed with an obstetrician/gynecologist and the benefits weighed against
any risks.

EXERCISE, FLEXIBILITY, AND STRETCHING
You may be interested in the role exercise plays in the disease. Can you continue
your pre-diagnosis lifestyle? Does exercise have a reparative role with positiveeffects on endurance and strength?
There is limited scientific evidence regarding the risks and benefits of aerobic
and strength training exercise in ALS. Animal studies in mice with ALS haveshown that moderate-intensity aerobic-type exercise delayed disease onset
and increased survival times in the exercised mice compared to mice that were
not exercised (Carreras et al., 2010; Mahoney et al., 2004). On the other hand,
high-intensity endurance exercise has been detrimental (Carreras et al., 2010;
Kirkinezos et al., 2003; Veldink et al., 2003).
Small clinical studies in people with ALS also support the safety of moderateintensity exercise. The most recently published study on this subject was ahigher quality randomized controlled trial assessing the effects of exercise in ALS
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(Lunetta et al., 2015). The study findings were consistent with results of two priorstudies of exercise in ALS (Drory, et al., 2001 and Bello-Haas et al., 2007).
All three trials showed less functional decline as measured by the ALS Functional
Rating Scale (ALSFRS) in those who participated in a moderate exercise program.
The most recent study showed the greatest benefit in people living with ALS
who performed a moderate intensity strength training and cycling program.
Ongoing benefit was observed up to 12 months after the initiation of the program.
However, none of the three clinical trials demonstrated a prolonged survival
benefit.
Based on the available evidence it appears safe for people living with ALS toparticipate in moderate-intensity exercise. Overexertion, as demonstrated byprolonged fatigue after exercise, muscle pain, or soreness, however, should
be avoided (Petrof, 1998). Restorative gentle exercise can be used as a tool toprevent deconditioning, to improve sleep and mood. Aerobic exercise practiced
in a community setting (e.g., accessible pool, adaptive golf, chair yoga, tai chi)helps provide opportunities for socializing. Stretching and range-of-motion
exercises should be started soon after diagnosis as part of a gentle, wellnessoriented, daily routine. Performing simple stretching exercises to target the major
joints helps prevent painful and function-limiting contractures, especially at
the shoulders. Training by a physical therapist, who will develop an appropriate
exercise routine for you, is recommended. The program can be performed
independently in the early stages of disease and transitioned to caregiverassisted, range-of-motion exercise as needed. Linking the range-of-motion
program to a dressing schedule, when limbs need to be maneuvered into
clothing, is convenient and encourages performance twice daily during morningand night dressing.

DURABLE MEDICAL EQUIPMENT AND ASSISTIVE DEVICES
I could no longer operate my video editing equipment. I was losing my clients one by one. I got very
worried that I was going to lose my video post-production business...At that point, my legs and feetwere still very strong, and the state wanted to keep every able person working as long as possible.
Cheryl contacted the state and after a few days someone from the program called to make an
appointment...he showed up at my office with a bag a few days later. He opened the bag and pulled
out two rectangular wooden boards. One of the boards contained a controller with a ball and theother contained two switch plates. At first, operating the ball and switches with my feet was slow anddifficult, but eventually using the controls got easier and I became faster. I was happy to be working
again!
Person with ALS (Contributed by The ALS Association Golden West Chapter)
Medicare covers Durable Medical Equipment (DME) meeting the following
criteria: “The equipment must be long-lasting, used for medical reasons, andwould not be considered useful to someone who is not sick or injured.” DME

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is for use in your home and has an expected lifetime of at least three years. To
be covered by Medicare, the equipment needs to be prescribed by a physician.
All people with Medicare Part B have DME benefits. If you have an insurance
provider other than Medicare, you will need to review your policy to determine
what is covered, the timing of coverage, and how to optimize benefits.
DME that Medicare covers includes, but isn’t limited to:■ Air-fluidized beds and other support surfaces (these supplies are only

rented)■ Canes (however, white canes for the blind aren’t covered)■ Commode chairs■ Hospital beds
■ Manual wheelchairs and power mobility devices■ Oxygen equipment and accessories■ Patient lifts■ Bilevel Positive Airway Pressure (BiPAP) or Average Volume Assured

Ventilation (AVAP) devices and accessories■ Suction pumps■ Traction equipment■ Walkers

Beds, assisted breathing devices, and other equipment are rented to own. The
cost of the equipment is divided over 13 months and once paid, becomes theproperty of the person living with ALS.
When the loss of independent ambulation occurs, braces, canes, walkers, andwheelchairs should be considered. The transition to and from each of theseassistive devices should be monitored in the clinic (Figure 9). Braces to stabilize
the ankle, called ankle–foot orthoses, are helpful if you have a foot drop. They
prevent catching your toe and tripping.

Single point
cane orwalking stick

Quad cane

Rolling walker
with orwithout a seat

ManualwheelchairPowerwheelchair

Figure 9: Transitions in assisted devices.

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Canes and Walkers. A cane or walker can help prevent falls if prescribed
appropriately, or add to the risk of falls if prescribed inappropriately. For example,
when you have significant spasticity you may be at greater risk of falls andinjuring yourself while using a cane because it does not have adequate stability
to provide reliable support. A walker, however, may provide the support needed
to continue walking safely. People living with ALS most often do well with afour-wheeled walker with a seat. Hand strength is required to be able to lock thebrakes and it should be the type of walker that has enough clearance behind theseat to allow standing within the arms of the wheelchair. Falls are very common
in ALS and every attempt should be made to try to prevent them. In one study,nearly 2% of people with ALS died prematurely from a fall.

This past week I had to give up using my walker and now fully rely on my “Go Chair.” This causes
some mixed feelings since it means giving up a major source of independence, walking. However,using the scooter does provide some freedom that the walker did not. Since I don’t get as tired as I
did with the walker, I am not as hesitant to get up and move, I can travel with ease around my houseor most important, all over the mall; shoppers, watch out here I come. Either with the walker or thescooter, I have to count the cost, preserve my energy, and realize each step in life is precious.
Susan Catlett (Contributed by The ALS Association Greater Sacramento Chapter)
Wheelchairs. As strength and coordination worsen, hand-held devices will
eventually be inadequate to support safe ambulation and a wheelchair will be
required. While a slingback manual wheelchair conveniently folds and is easilytransported in a car, these wheelchairs do not have good cushion support andare not appropriate for prolonged sitting. It is important to work with a specialist
who knows about ALS and can predict what will be needed in a wheelchair for
long-term comfort (Figure 10).

Figure 10: Transport wheelchairand four-wheeled walker for easiertransport.

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The most appropriate wheelchairs when living with ALS have complex features
such as tilt-in-space and recline that enable repositioning for maintaining
skin integrity, comfort, and respiratory health. Many people with ALS spend
most of their time in their wheelchairs, so the chairs must be comfortable andfacilitate good health. There are tilt-in-space manual wheelchairs and power
wheelchairs (Figure 11). The benefit of a power wheelchair is that you cancontinue to be independent and navigate around the home and community, as
well as reposition yourself. However, if there is dementia, a manual wheelchairwith tilt-in-space and recline may be the best option, as it will require caregiver
participation for use. Medicare typically only covers the purchase of one
wheelchair. It is in your best interest to have Medicare help purchase the complex
wheelchair, while you borrow or purchase your own light-weight foldable
transport chair. Also, a good quality supportive seat cushion is a must to avoid
pain and pressure wounds caused by prolonged sitting.

Figure 11: Power wheelchair for easier navigation around the home and community.

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Custom Wheelchairs. At the time this resource guide was published, Medicare
had recently had a competitive bidding process, awarding specific companies
with contracts to provide various DME. The custom wheelchair will be provided
by a Medicare-approved DME vendor and your physician’s office will know whom
you need to contact to obtain the equipment. You will require a face-to-face visit
with a physician. They will write the prescription for the wheelchair and place areferral to an OT or other qualified professional who will provide the specifics
for the wheelchair to the DME provider. It can take months to receive a custom
wheelchair. If ALS progresses faster than anticipated, and a wheelchair is needed
before the custom chair is available, contact your local ALS Association chapter
to inquire about equipment loan programs that may be able to provide shortterm DME solutions.
Transporting a power wheelchair is difficult unless you have access to an adapted
van. The adapted van will need a raised top, side, or rear entry, and either awheelchair lift or ramp for entering and exiting. The van also requires tie downs
that lock the wheelchair safely in place and match those on the wheelchairneeding to be transported.

HOME ADAPTATIONSThe OT and DME provider are able to perform home safety evaluations. During
the evaluation, they will assess the home layout and provide recommendations
for making the home more accessible and safe. These recommendations may
include advice for minor adaptations or major home remodeling as requested.
Common recommendations include placing grabs bars in the shower or bathtub
and ramps for entry to and exit from the home.
Building a ramp requires adequate outside space to meet building code
requirements of no more than a 1-inch downslope for every 12 inches of ramp.
If there is no bedroom space on the main level of the home, a stair lift may
provide a temporary method for moving up and down stairs as long as there is

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adequate upper body strength. Keep in mind this is a significant investment for
only a temporary solution before upper body weakness occurs. Older homes
often have narrow doorways that are not wide enough for wheelchairs; thesecan be widened. If you have the resources, expanding a small bathroom to allow
room enough for a roll-in shower and roll-over toilet is an investment that will
make bathing, grooming, and toileting safer and more convenient. Planninghome adaptations is best done early in the disease, so that any changes may becompleted prior to when they are absolutely needed.
Lastly, environmental controls are available that enable those who are lacking
the independent mobility to turn on and off the lights, televisions, stereos,
computers, etc. on their own. Thankfully, there are more options available now
than ever before to improve independence.

SUMMARY STATEMENT
ALS is a progressive disease that causes growing mobility impairment anddependence on caregivers. The good news is there is an ever-expanding numberof assistive devices and DME to extend independence and mobility. Partneringwith a PT and OT who specialize in ALS will make for a good team as they can
perform serial assessments, make recommendations, and educate you andyour caregivers on the use of assistive equipment and mobility devices. The
goals of treatment are to improve health, safety, independence, and community
access. Moderate intensity exercise and energy conservation techniques can
reduce fatigue and improve the energy needed for the activities that are themost important to you and that provide you with the most meaning and joy. It is
critical to discuss and plan ahead with your ALS rehabilitation team to allow thegoals of treatment and optimal quality of life to be achieved.

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BIBLIOGRAPHY
Bello-Haas V.D., Florence J.M., Kloos A.D., Scheirbecker
J., Lopate G., Hayes S.M., Pioro E.P., Mitsumoto H. (2007).
A randomized controlled trial of resistance exercise inindividuals with ALS. Neurology, 68(23): 2003-2007.
Carreras I., Yuruker S., Aytan N., Hossain L., Choi J.K.,
Jenkins B.G., Kowall N.W., Dedeoglu A. (2010). Moderate
exercise delays the motor performance decline in atransgenic model of ALS. Brain Research, 1313:192-201.
doi:10.1016/j.brainres.2009.11.051. Epub 2009 Dec 5.
Drory V.E., Goltsman E., Reznik J.G., Mosek A., Korczyn
A.D. (2001). The value of muscle exercise in patientswith amyotrophic lateral sclerosis. Journal of the
Neurological Sciences, 191(1-2): 133-137.
Lunetta C., Lizio A.,Sansone V.A., Cellotto N.M., MaestriE., Bettinelli M., Gatti V., Melazzini M.G., Meola G., Corbo
M. (2015). Strictly monitored exercise programs reduce
motor deterioration in ALS: preliminary results of arandomized controlled trial. Journal of Neurology, Oct17, 2015. [Epub ahead of print]
Kirkinezos I.G., Hernandez D., Bradley W.G., Moraes C.T.
(2003). Regular exercise is beneficial to a mouse modelof amyotrophic lateral sclerosis. Annals of Neurology,
53(6): 804-807.
Mahoney D.J., Rodriguez C., Devries M., Yasuda N.,
Tarnopolsky M.A. (2004). Effects of high-intensity
endurance exercise training in the G93A mouse modelof amyotrophic lateral sclerosis. Muscle & Nerve, 29(5):656-662.
Petrof B.J. (1998). The molecular basis of activityinduced muscle injury in Duchenne muscular dystrophy.
Molecular and Cellular Biochemistry, 179 (1-2): 111-123.
Veldink J.H., Bär P.R., Joosten E.A., Otten M., Wokke J.H.,
van den Berg L.H. (2003). Sexual differences in onset of
disease and response to exercise in a transgenic modelof ALS. Neuromuscular Disorders, 13(9): 737-743.

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The following is a list of topics covered in the Living with ALS resource guides:

Resource Guide 1
What is ALS? An Introductory Resource Guide for Living with ALS
This resource guide provides an overview of ALS, what it is, and how it affects your body.
It provides information on what kind of resources are available to help you deal with
ALS more effectively.

Resource Guide 2
After the ALS Diagnosis: Coping with the “New Normal”
This resource guide addresses the psychological, emotional, and social issues that you
must face when your life is affected by ALS. It provides information on how to copewith the many lifestyle changes and adjustments that occur when you live with ALS.

Resource Guide 3
Changes in Thinking and Behavior in ALSThis resource guide addresses how thinking and behavior may be affected by ALS andhow these changes can impact disease course, symptom management, and decision
making.

Resource Guide 4
Living with ALS: Planning and Making Decisions
This resource guide reviews areas where careful planning and decision making will be
required and will provide you with resources to help you and your family plan for the
future.

Resource Guide 5
Understanding Insurance and Benefits When You Have ALSThis resource guide provides strategies and helpful hints to better navigate healthinsurance and benefits. While understanding insurance and benefits may feeloverwhelming, the guidelines outlined here should help simplify the process for you.

Resource Guide 6
Managing Symptoms of ALS
This resource guide discusses a variety of symptoms that may affect you when you have
ALS. As the disease progresses, various functions may become affected and it is helpful
to understand potential changes so that you know what to expect and how to manage
these new changes and symptoms.

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Resource Guide 7
Functioning When Mobility is Affected by ALS
This resource guide covers the range of mobility issues that occur with ALS. It
discusses exercises to maximize your mobility, as well as how to adapt your home andactivities of daily living to help you function more effectively.

Resource Guide 8
Adjusting to Swallowing Changes and Nutritional Management in ALSThis resource guide will help you understand how swallowing is affected by ALS andwhat you can do to maintain nutrition for energy and strength and to keep yourairway open.

Resource Guide 9
Changes in Speech and Communication Solutions
This resource guide covers how speech can be affected by ALS and explores a variety
of techniques, technologies, and devices available for improving communication. By
maintaining communication with others, you continue to make a significant differencein their lives, while retaining control of your own.

Resource Guide 10
Adapting to Changes in Breathing When You Have ALSThis resource guide explains how breathing is affected by ALS. Specifically, it willteach you the basics of how the lungs function, the changes that will occur, and how
to prepare for the decisions that will need to be made when the lungs need maximal
assistance.

Resource Guide 11
Approaching End of Life in ALSThis resource guide examines thoughts and feelings about dying and end of life.
Approaching end of life is difficult and support is critical to help sort out feelings,
expectations, and plans. By talking to friends, family, professionals, and planning andcommunicating your wishes, you can help prepare for the best possible end-of-life
phase.

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1275 K Street NW, Suite 250
Washington, DC 20005
Telephone: 202-407-8580
Fax: 202-464-8869
About The ALS Association
The ALS Association is the only national nonprofit organization fighting Lou Gehrig’s Diseaseon every front. By leading the way in global
research, providing assistance for people with
ALS through a nationwide network of chapters,
coordinating multidisciplinary care throughcertified clinical care centers and fostering
government partnerships, The Associationbuilds hope and enhances quality of life while
aggressively searching for new treatments anda cure.
For more information about
The ALS Association, visit our
website at www.alsa.org.

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