What is Amyotrophic Lateral Sclerosis?

Initially, muscle wasting often appears in the hand or calf muscles, noticeable through clumsiness or increased tripping. Additionally, involuntary twitching of individual muscle fibers, especially on the tongue, or of muscle bundles can be observed. Some patients experience speech and swallowing disorders as well as nocturnal, painful muscle cramps.
As the disease progresses, paralysis spreads to the entire musculature. ALS is incurable, but symptoms and the rate of progression vary individually. Typically, there is rapid progression with significant weight loss leading to paralysis of the respiratory muscles. Most affected individuals die within 2-5 years, though longer courses are also known.

There is no causal treatment for ALS. Various medications can delay the progression of the disease by a few months and alleviate symptoms such as muscle cramps, increased salivation due to swallowing disorders, and spasticity. Due to the rapid progression of ALS, it is important for patients to familiarize themselves with treatment options early on, such as artificial feeding through a gastric tube or the choice of invasive ventilation. Timely interdisciplinary care involving physiotherapy, occupational therapy, speech therapy, psychological, and palliative care should be initiated.

In the later stages, various aids can simplify daily life by preserving some mobility or enabling communication in case of restricted speech function. These include communication boards, toilet chairs, stair lifts, and shower chairs.

The focus of ALS treatment primarily lies in rehabilitation, aiming to preserve physical functions and independence for as long as possible. Orthoses and wheelchairs play a central role here, as they support the autonomy of those affected and enhance their quality of life.

In the advanced stages of ALS, walking aids are no longer sufficient, and patients need a wheelchair to maintain their mobility. This often poses a significant emotional and organizational challenge for those affected. Extensive consultation sessions are necessary to discuss possible features of the wheelchair and adapt it to individual needs. As symptoms and disability levels change over time, proactive decisions should be made. Here, an experienced team of orthopedic technicians and physiotherapists can help to consider important aspects of financing, home environment, and specific symptoms, and to find solutions together.

It is important to distinguish between manual and electric wheelchairs. Both types offer different benefits, which are briefly introduced below.

Manual wheelchairs are often used in the early to middle stages of ALS. To operate them independently, a certain amount of trunk and arm strength is required. This type of wheelchair weighs less than the electric version and is therefore easier to transport. They are often used to participate in activities outside the home and to cover longer distances due to energy conservation. In the advanced stages, patients are permanently reliant on a wheelchair, can no longer propel it on their own, and thus often switch to an electric wheelchair. For this reason, depending on financial situations, it is recommended to opt for an electric wheelchair from the start and possibly use a manual one in addition.

Electric wheelchairs can be operated independently even with decreasing strength, granting more autonomy to the user. When selecting a wheelchair, it should be equipped with electronics that can be progressively upgraded. Various joysticks for control are available, which can be operated with the hands, chin, or lips. Special sensors allow operation even when no force is possible. Additional components can be installed to control everyday items such as doors, televisions, smartphones, and computers.
Additionally, the seat, backrest, and leg support angles should be adjustable to ensure maximum comfort and prevent pain or pressure sores. If the wheelchair has the function to assume a lying position, it can be used to rest intermittently and reduce the number of transfers between the wheelchair and bed, which represent a significant physical strain for patients and caregivers. Due to the progressive weakness of the respiratory muscles, breathing can be impaired while lying down. Here, wheelchairs with a standing function can help, allowing the user to change positions while standing.
Furthermore, the type of drive plays a role. Front-wheel and mid-wheel drives make control by caregivers difficult. This may become necessary as strength decreases, so generally a rear-wheel drive is recommended.

Operating the wheelchair with a joystick is particularly common in such cases. To make this control feasible, the wheelchair should have electronics that can be expanded. This allows for the selection of the appropriate joystick option from a large range available. When control with arms and hands is no longer possible, various models can be operated with the chin or lips.

It is important to professionally assess which option is best suited. This way, patients can maintain their mobility and independence for as long as possible. Using sensors, the wheelchair can still be controlled even when no force is possible.

Additionally, additional modules on the wheelchair can control other devices. These include doors, the television, the smartphone, or the computer, to name just a few examples. These solutions should be applied for early, as some acclimatization is usually necessary.

Amyotrophic Lateral Sclerosis is a rapidly progressing disease that leads to severe muscle paralysis due to the degeneration of motor neurons. It is incurable, but can be well supported with appropriate aids. Through individually adapted orthoses and wheelchairs, people with ALS can remain independent longer and preserve their quality of life.