“I am quite often asked: How do you feel about having ALS? The answer is, not a lot. I try to lead as normal a life as possible, and not think about my condition, or regret the things it prevents me from doing, which are not that many.” – Stephen Hawking
Amyotrophic Lateral Sclerosis (ALS) – also referred to as ‘Motor Neuron Disease’ (MND) – is a progressive neurodegenerative disorder resulting from the death of nerves that control the voluntary movements of the trunk and limbs, respiratory system, as well as the motor control centres involved in speech and swallowing.
The death of nerve cells can cause muscle weakness and deterioration.This gradual degenerative process can lead to difficulty with talking and swallowing, muscle paralysis, and respiratory depression. Typically, the onset of ALS occurs later in life. It is ideopathic in nature, meaning there is no known cause.
About Amyotrophic Lateral Sclerosis – ALS
Early symptoms of ALS includes muscle weakness or atrophy (decrease in muscle mass). Further symptoms include muscle stiffness of the trunk and limbs, frequent cramps, muscle fasciculation (involuntary twitching of the muscles) and/or slurred and nasal sounding speech. Patients may also experience difficulty swallowing foods and/or fluids, a disorder known as Dysphagia. These signs of weakness/slowness of movements may essentially manifest in common day to day tasks. Gross motor movements may be affected, such as walking and/or running; patients will often stumble/trip during these motor tasks. Changes with everyday fine motor movements may also become apparent in the early stages of disease progression, with patients having difficulty with buttoning up a shirt, writing or operating a remote control. Most patients, however, experience limited changes with regards to cognitive function. Disease progression varies from person to person.
Patients with ALS will often experience dysphagia when swallowing foods and/or fluids. This arises due to death of the nerve cells involved in the motor coordination when swallowing. In the later stages, patients may experience difficulty with swallowing and breathing. The process of swallowing requires precise coordination of the respiratory system (breathing) in order protect the airway from the risk of aspiration. Aspiration is a term used to define foreign particles (food/fluid) entering through the trachea (windpipe) down into the lungs, instead of passing through oesophagus down to the stomach. Aspiration may cause changes in temperature (due to aspirants/foreign particles entering the lungs) leading to possible risk of aspiration pneumonia. A patient’s diet may have to be modified so that the patient is not at risk of aspiration.
Initially, patients will notice difficulties with motor speech control. Their speech, for example, may sound slurred. Often patients with ALS feel that the need to repeat themselves constantly in order to be understood by the listener. As the disease progresses, patients may lose the ability to communicate verbally altogether. Advances in technology allow patients to use alternate means of communication, with patients opting for Alternative and Augmentative Communication (AAC) devices. Essentially, these devices can be in the form of low tech (e.g. signing, pictures etc.) or high tech (iPad, touchscreen or Speech Generating Devices [SGD] etc.).
How can Speech Pathology assist people who have ALS?
A Speech Pathologist is a trained professional that works with patients with ALS to optimise their communication and enhance their participation in everyday activities. Speech Pathologists assess, diagnose and provide treatment plans for patients with communication and swallowing disorders.
Patients with ALS will require ongoing intervention from disease onset through to the latter stages of disease progression, in order to ensure patients receive optimal care. Speech Pathologists work with motor speech difficulties, whether it be providing exercises to enhance speech intelligibility or recommending AAC devices to assist with communication. A Speech Pathologist also assesses and modifies a patient’s diet in order to ensure airway protection and safe swallowing when eating/drinking.
Contact us for results focused speech therapy in Sydney
This article was written by our Speech Pathologist Eugene Pillay who is a Speech Pathology Australia member.
If you have questions about speech therapy in relation to amyotrophic lateral sclerosis, contact your local doctor, who will arrange for you to see a speech pathologist, Contact us today!
Paris, G., Martinaud, O., Petit, A., Cuvelier, A., Hannequin, D., Roppeneck, P. & Verin, E. (2012). Oropharyngeal Dysphagia in amyotrophic lateral sclerosis. Journal of Oral Rehabilitation, 40(3), 199-204.
Robarge, K.M. (2009). Communication Management in Amyotrophic Lateral Sclerosis: The Role of the Speech-Language Pathologist during Disease Progression. The Internet Journal of Allied Health Sciences and Practice, 7(2), 1-6.