What is amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis (ALS) is a chronic disease of the nervous system in which motor nerve cells are destroyed. This leads to progressive muscle paralysis and muscle atrophy. A characteristic feature of ALS is that both the nerve cells that transmit signals from the brain to the spinal cord (1st motoneuron) and those that pull from the spinal cord to the muscles (2nd motoneuron) are affected. This results in both spastic paralysis with increased reflexes and flaccid paralysis with muscle atrophy and weakened reflexes. The exact causes are still unknown. Mostly ALS occurs sporadically, but hereditary forms are also known. The onset of the disease is typically between the ages of 50 and 70; men are affected more frequently than women.


What are the symptoms of ALS?

At the beginning, there is often muscle atrophy in the hand or calf muscles, which is noticeable to those affected by clumsiness or increased stumbling. In addition, involuntary twitching of individual muscle fibers, especially on the tongue, or fiber bundles may be observed. Some sufferers experience speech and swallowing disorders as well as painful muscle cramps at night.
As the disease progresses, the paralysis spreads to the entire musculature. ALS is not curable, but symptoms and speed of progression of the disease are individual. As a rule, there is a rapid progression with severe weight loss up to paralysis of the respiratory muscles. Most patients die within 2-5 years, but long-term courses are also known.

How is ALS treated?

A causal treatment for ALS does not exist. Various medications can delay the progression of the disease by a few months and alleviate symptoms such as muscle spasms, increased salivation due to swallowing disorders and spasticity. Due to the rapid progression of ALS, it is important that affected individuals consider treatment options early on, such as artificial feeding through a feeding tube or the desire for invasive ventilation. Interdisciplinary care consisting of physiotherapy, occupational therapy, speech therapy, psychological and palliative therapy should be started in good time.
As the disease progresses, various aids can make everyday life easier by maintaining a degree of mobility for those affected or enabling communication in the case of impaired speech function. These include, for example, communication boards, commode chairs, stair lifts and shower chairs.
The focus of ALS treatment is primarily on rehabilitation, i.e. maintaining 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 increase their quality of life.

What therapeutic tools are available for people with ALS?

Affected persons initially suffer particularly from the loss of the ability to walk. In the earlier stages of the disease, so-called peroneal splints, i.e. orthoses that facilitate lifting of the paralyzed foot, can be used. An individually adapted orthosis improves the feeling of safety for the patient and provides better stability when walking and standing. Furthermore, canes, crutches and other walking aids are used. The choice of walking aid depends on the individual weaknesses, or the still functional upper and lower body muscles. If ALS progresses, there is often a pronounced weakness of the neck and neck muscles, which makes it difficult to keep the head upright independently. In this case, it is also possible to professionally fit orthoses that prevent the head from falling down. In the later stages, the majority of sufferers are ultimately dependent on a wheelchair. Often, symptoms rapidly worsen, requiring regular qualified assessment and specific fitting of assistive devices.

Which wheelchair is most suitable for people with ALS?

In the advanced stages of ALS, walking aids are no longer sufficient, and patients need a wheelchair to maintain their mobility. This is often a great emotional and organizational challenge for those affected. Detailed counseling sessions are necessary here to discuss possible functions of the wheelchair and to adapt it to individual needs. As the symptoms of ALS change in the course and the degree of disability increases, forward-looking decisions should be made. Here, an experienced team of orthotists and physical therapists can help look at important aspects of financing, home environment and specific symptoms and find solutions together.

Basically, a distinction must be made between manually and electrically operated wheelchairs. Both types offer different advantages, which are briefly presented below.

Manual wheelchairs are increasingly used in the early to middle stages of ALS. A certain amount of strength of the trunk and arms is needed to steer them independently. This type of wheelchair weighs less than the electric version and is therefore easier to transport. Therefore, they are popular for participating in activities outside the home and traveling longer distances due to the energy savings. In advanced stages, affected persons are permanently dependent on a wheelchair, can no longer propel it under their own power and therefore usually switch to an electric wheelchair. For this reason, we recommend, depending on the financial situation, to rely on an electric wheelchair from the very beginning and, if necessary, to use a manual one in addition.

The electric wheelchair

Electric wheelchairs can be controlled independently, even with diminishing strength, giving sufferers more autonomy. When choosing a wheelchair, care should be taken to ensure that it is equipped with basic electronics that can be gradually expanded. Various joysticks exist for control, which can be operated with hands, chin or lips. Special sensors enable handling even when force is no longer possible. Additional elements can be installed to enable the operation of everyday objects such as doors, televisions, smartphones and computers.
Likewise, the seat, backrest and legrest angle should be adjustable to ensure maximum comfort and prevent pain or pressure damage to the skin.
If the wheelchair has the function to assume a lying position, this can be used to rest in between and reduce the number of transfers between the wheelchair and the bed, which are a heavy physical burden for both the patient and the caregiver. However, due to the progressive weakness of the respiratory muscles, it can happen that breathing is impaired when lying down. This is where wheelchairs with a standing function, which allow the affected person to change position while standing, can help.
The type of drive also plays a role. Front-wheel and center-wheel drives make it difficult for attendants to steer the wheelchair. However, this may become necessary as strength decreases, so rear-wheel drive is generally recommended.

A power wheelchair with standing function for breathing

Some patients cannot tolerate a lying position, as this impairs breathing. We have therefore experienced that a standing function in the wheelchair can provide relief here. This function relieves breathing and shifts more weight to the legs and feet. However, affected persons should check the possible standing function at an early stage to determine whether it is really an option.

Elk wheelchair-ottobock-with standing function

The operation of the wheelchair with decreasing strength

In such cases, it is particularly common to control the wheelchair with a joystick. For this control to be feasible, the wheelchair should have electronics that can be removed. This allows the right option to be selected from the wide range of joysticks. If control with arms and hands is no longer possible at some point, various models can be controlled with the chin or lips.

Here, professional consideration must be given to which option is most suitable. This allows those affected to retain a certain degree of mobility and independence for as long as possible. With the use of sensors, the wheelchair can still be steered even when power is no longer possible.

In addition, further devices can be controlled by means of add-on modules on the wheelchair. These include doors, the TV, the smartphone or the computer, to name just a few examples. These solutions should be requested at an early stage, as a certain amount of familiarization is usually required.

Conclusion: More quality of life through aids for people with ALS

Amyotrophic lateral sclerosis is a rapidly progressing disease in which severe muscle paralysis occurs due to the destruction of motor neurons. It is not curable, but can be well supported by suitable aids. Individually adapted orthoses and wheelchairs enable people with ALS to remain independent for longer and maintain their quality of life.

[1] Grad, L.I., Rouleau, G.A., Ravits, J., Cashman, N.R., 2017. clinical spectrum of amyotrophic lateral sclerosis (ALS). Cold Spring Harb Perspect Med 7, a024117. https://doi.org/10.1101/cshperspect.a024117

[2] MAJMUDAR, S., WU, J., PAGANONI, S., 2014. REHABILITATION IN AMYOTROPHIC LATERAL SCLEROSIS: WHY IT MATTERS. Muscle Nerve 50, 4-13. https://doi.org/10.1002/mus.24202

[3] Oskarsson, B., Gendron, T.F., Staff, N.P., 2018. amyotrophic lateral sclerosis: An Update for 2018. Mayo Clin Proc 93, 1617-1628. https://doi.org/10.1016/j.mayocp.2018.04.007

[4] Soriani, M.-H., Desnuelle, C., 2017. care management in amyotrophic lateral sclerosis. Rev Neurol (Paris) 173, 288-299. https://doi.org/10.1016/j.neurol.2017.03.031

[5] Talbott, E.O., Malek, A.M., Lacomis, D., 2016. the epidemiology of amyotrophic lateral sclerosis. Handb Clin Neurol 138, 225-238. https://doi.org/10.1016/B978-0-12-802973-2.00013-6

[6] Trail, M., Nelson, N., Van, J.N., Appel, S.H., Lai, E.C., 2001. Wheelchair use by patients with amyotrophic lateral sclerosis: a survey of user characteristics and selection preferences. Arch Phys Med Rehabil 82, 98-102. https://doi.org/10.1053/apmr.2001.18062

[7] Anvil - Amyotrophic lateral sclerosis

https://next.amboss.com/de/article/LR0wnf?q=als#Z38867300629f0b87ffa50b1fb90dfbdf (aufgerufen am 18.06.2022)

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