Friedreich’s ataxia: Assistive equipment, aids, and adaptations

 

Living with Friedreich’s ataxia (FA) may require specialized equipment to help support daily activities, assist with mobility, and ease health complications such as fatigue.

FA is a genetic condition caused by a mutation in a gene called FXN, which carries instructions for making the frataxin protein. One of the first Friedreich’s ataxia symptoms a person may notice is a loss of balance and trouble walking, which is caused by damage to nerve cells needed to coordinate movement.

Over time, the disease progresses to cause other symptoms such as slurred speech, an abnormal curvature of the spine, hearing and vision problems, and, in many cases, a heart disease. There is no cure for FA, and treatment aims to manage symptoms.

A number of mobility aids and other adaptations are available to make daily life easier at home, school, and work. An occupational therapist can help patients select the assistive devices that will work for them, based on factors such as personal preference, age, symptoms, disease severity, and whether the patient has children, is living alone, or needs full-time caregiving.

As the disease progresses and starts to affect mobility more and more, people living with Friedreich’s ataxia may need to make additional modifications to get the support they need, which often means introducing new types of assistive devices over time. For example, someone with FA may first rely on a cane to get around, but then may need a walker or rollator, and eventually a wheelchair as their mobility declines.

Walkers

A walker is a stable four-legged mobility aid that helps a person get around by supporting their weight. It is a lightweight device that improves mobility and independence, making it a good choice for people with FA who have a slower gait and balance issues.

Some walkers have a pair of wheels on the front of the frame to make it easier to maneuver. Walkers with larger wheels can be used outdoors or on uneven ground.

Walkers can be accessorized with add-ons, such as a cup holder, a basket, pouches, an armrest platform, or an oxygen tank holder.

Medical walker

How it works:	The stable frame helps with balance and is good for those who move at a slower pace.
Best for:	Balance and weight bearing, often for short, indoor trips.
What to know:	Height can be adjusted. Walkers with larger wheels can be used outdoors or on uneven ground.

 

Hemi walker

How it works:	Also known as a side-style walker, a hemi walker is also a four-legged device but is used in one hand like a cane. Non-slip feet and hand grips give it stability.
Best for:	Someone who needs more stability than a cane, or who has limited dexterity on one side of their body.
What to know:	Height is adjustable. Can be used as support when standing up from a sitting position.

 

Other types of walkers include:

• a trolley walker, which has one or two trays for carrying small items.
• a knee walker, also known as a knee scooter, which has three to five wheels for stability, a padded bench for the non-weight bearing knee, and a handlebar for steering. It may be more comfortable and easier to maneuver than crutches.

Rollators

A rollator is similar to a walker but has three or four large wheels. It has lockable hand brakes and a built-in seat for resting.

Unlike a walker, most rollators aren’t intended to support the body’s entire weight when moving. Instead, these devices are designed to help people who can still walk at a normal pace but need some support for balance and stability.

Rollator

How it works:	A rollator steers easily and can be slowed with the hand brakes, which lock in place when stopping.
Best for:	Someone with a steady gait who still needs a balance aid, especially when taking longer walks. Some can double as transport chairs.
What to know:	Rollators fold up and can be put in the trunk of a car. May be outfitted with a basket or a tray. A three-wheel rollator is good for tighter situations, such as narrow hallways or rooms.

 

Wheelchairs

Wheelchairs come in a range of types, including lightweight models that can be pushed by someone else or propelled by the user, power chairs that use rechargeable batteries, and specialized chairs for wheelchair sports. They can be outfitted with either a control pad or joystick for maneuvering.

Wheelchairs often can be customized based on a person’s needs. They can also be sized for children.

Medicare Part B, which helps cover medical expenses like doctors’ services and assistive devices, may cover 80% of the cost of some electric wheelchairs with a prescription from a healthcare professional.

Manual wheelchair

How it works:	A basic wheelchair can be rolled by the user or pushed by someone else while the occupant rides.
Best for:	A person who is struggling with mobility. Can be used full time or in situations where getting around or falling is a concern.
What to know:	Push wheelchairs come with lockable wheels and can be folded for transport. Different models can handle different weights. Some are designed for children and teenagers.

 

Power chairs

How it works:	Can be driven by a joystick, head switches, chin-operated joysticks, sip-and-puff controllers, or voice commands. Some power chairs are foldable for transport in a vehicle or on an airplane.
Best for:	Someone who wants to maintain more independence of mobility. “Smart” wheelchairs are driven by computer and have features such as collision avoidance and route planning.
What to know:	Look for features like anti-tip wheels, knobby tires for traction. power controls for memory angle adjustments, and standing and all-terrain functions. The chair can tip over on a ramp or uneven terrain.

 

What to consider when choosing a power chair:

• how long you expect to be in the chair each day
• whether you want a front-, mid-, or rear-wheel drive system, especially if planning to use the chair outdoors on uneven terrain
• which chair size better fits in your home, considering the turning radius in narrow locations, and the load bearing capacity of the floors, as some models are heavier than others
• seat position, including ability to recline, and cradle the body comfortably
• a controller that works with your dexterity level.

Make sure you practice using the chair in a safe setting first.

Scooters

Mobility scooters come in three- and four-wheel models that can be used indoors and outdoors, along with foldable models for travel. Most have a maximum speed of 5 miles per hour.

Medicare Part B may cover some of the purchase price of a mobility scooter when prescribed by a healthcare provider. Medicare also may cover 80% of the rental cost of a mobility scooter in the U.S.

Many U.S. states have organizations and charities that can loan mobility equipment or cover the cost of a mobility scooter, as will the U.S. Veterans Affairs. Check your state’s Social Security and disability assistance office, Americans with Disabilities Act regional center, or speak with your doctor or physical therapist for more information.

Scooter

How it works:	Mobility scooters have hand controls for speed and brakes.
Best for:	A person with enough mobility to operate the electric device but unable to walk long distances.
What to know:	If you are traveling and don’t want to take your scooter, check if you can rent one in the location you are visiting.

 

When choosing a mobility scooter, consider:

• ease of maneuverability around obstacles
• stability on uneven terrain
• weight of the machine, especially if planning to stow it in a vehicle for road trips or in an airplane
• weight and height of the user.

Handrails

Handrails are usually long pieces of metal or wood that are fixed to posts or walls to provide support and stability when climbing up or down a flight of stairs or walking down a hallway. They can also be installed on bathroom walls.

Handrail

How it works:	Handrails can be installed inside and outside a home near stairs and steps.
Best for:	Someone who needs some help going up and down steps and stairs or moving around.
What to know:	Handrails should be at the correct height for the user and securely anchored. Consult a carpenter or renovation company experienced in installing handrails.

 

Stairlifts

A stairlift is a chair on a fixed track that helps a person safely go up and down stairs. They should always be used with the built-in safety belt or harness.

The lifts are installed on the stairs, not on the wall, so no structural modifications need to be made. This also allows it to be moved to a new home or sold to another user. Buying a used system can help with stairlift cost.

Stairlifts can be used indoors or outdoors. They can be installed on different types of stairs, including curved stairs. The drive system can be hard-wired into your home or plugged into an existing power outlet, usually with a battery backup in case of a power failure.

Most stairlifts have an emergency braking system and an automatic brake that kicks in if the lift encounters an object in its way.

Stairlift

How it works:	Unfold the chair, armrests and footrest, turn and seat yourself in the chair. Buckle the seatbelt or harness. Press and hold the switch to start the chair, which will automatically stop at the end of its track. Swivel the chair to face the landing, remove the seatbelt and either stand or transfer to a mobility aid.
Best for:	Anyone who needs help getting up or down stairs without fear of falling.
What to know:	Stairlifts offer safety features such as a diagnostic display, backup battery, and a remote control. Outdoor models are made of weather-resistant materials to withstand heat and cold temperatures.

For those who have trouble bending their knees, there are standing lifts, or perch lifts, for sitting with partially bent legs.

Most stairlifts cannot carry the weight of a power wheelchair. People who wish to carry their wheelchair up and down the stairs should instead look into an inclined platform lift. This kind of lift also works on stairs and can be folded up when not in use.

Motorized shopping carts

Motorized chairs with an attached shopping basket are often available in larger grocery stores and shopping malls. These adapted power chairs make it easier to carry items when shopping or riding with a small child.

Some stores have carts that can be attached to a wheelchair with Velcro straps.

Small wheelchair shopping baskets can also be added to a power chair or rollator.

Bathroom adaptations

Bathrooms can be tricky spaces, especially if the room is small. Wet and slippery surfaces are also an issue.

Using a wheelchair in the bathroom may require renovation to widen the doorway and change bathroom features, such as removing a tub and installing a walk-in, level-access shower with nonslip flooring to improve access for those with mobility impairments.

Short of a total retrofit, there are some quick-fix solutions:

• A shower bench equipped with nonskid feet and grab rails can be used to offer stability. Some benches can be built into the wall.
• A nonslip shower mat with suction cups underneath can be placed on the floor of the shower to prevent slipping.
• A caddy attached to the wall can keep shampoo, soap and other items within reach.
• A nonskid mat placed outside the shower can help with footing when entering and exiting the shower.
• changing the faucets on the sink and shower to a lever style can improve handling.
• An upgrade on the lighting, including waterproof lights in the shower, may also be considered.
• Bathroom handrails can be installed by the toilet and in the shower, with height and location according to your needs.
• Depending on your level of mobility, a transfer lift may be needed to go from a wheelchair into the shower or to use the toilet.

Adaptive clothing

Adaptive clothing for wheelchair users or others with limited mobility makes getting dressed easier and offers greater freedom of movement.

More companies are creating lines of adaptive clothing, with fashion brands including Tommy Hilfiger, Seven Jeans, IZ Adaptive, and Billy Footwear offering stylish and comfortable clothing.

Garments may have snap or Velcro fasteners, magnetic buttons, pull-on pants with elastic waistbands, and one-handed zippers. The fabric tends to be breathable, lightweight, and stretchy.

Adaptive clothing can go over the top of braces and other body aids. Pants often have a shorter front rise to avoid fabric bunching when seated in a wheelchair. Seams may be sewn on the outside to keep from irritating skin.

Velcro closures make it easier to tighten shoes.

Technology

Smart home electronics with voice-activated controls or input from a tablet or smartphone can help a person with FA with tasks like operating lights, temperature control, music, and answering the door.

Devices include Amazon Echo, Apple Homekit, Google Nest, and Samsung SmartThings.

Wi-Fi plugs are an alternative for controlling several devices, household appliances, and lamps and can either work with an electronic hub or by using their own smartphone app.

Cellphone clips and lanyards keep phones close, and grip straps that fit around your hand help you hold it.

A Roomba robot vacuum cleaner can help keep floors and carpets clean, including getting rid of pet hair.

Augmented and alternative communication devices also can help with speech issues. They include computer speech generators and eye-gaze devices.

Reaching aids

Reaching aids (or grabber tools) can help pick up small items that have fallen to the floor, get something off a shelf, or pull an item closer. Reaching aids usually have a squeeze or trigger handle that opens and closes a pair or trio of pincers at the far end of the stick.

Other reaching aids include long-handled sponges for washing the body or applying lotion, dressing sticks, long-handled shoe horns, and sock notches for holding a sock open and not needing to bend over.

Eating utensils

Adaptive utensils, cups, plates, and bowls can make eating meals easier. Adaptive eating utensils that may help people with FA include:

• cutlery with grips, or angled and bendable handles that are easier to grasp
• cuff utensil holders, or straps for utensils to hold them in place
• plates and bowls with higher sides to avoid spills, and suction or nonskid bases, or nonslip table mats
• scoop bowls with one high, curved side used to get food onto a utensil
• cups with large handles that make it easier to hold, lids to allow sipping without spills, and weighted bases to direct the flow of liquids
• Spillnot drink carrier for carrying a cup or mug without spilling.

 

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Friedreich’s Ataxia News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.