Amyotrophic lateral sclerosis (ALS) is a progressive neurogenerative condition which leaves the body completely paralysed. Celebrated physicist Stephen Hawking was perhaps the most renowned figure afflicted by this condition.
ALS is fatal, with prevalence rates ranging from 2.7 to 7.4 per 100,000 people. Science is still uncovering the mysteries of this condition, and from what is known, it is caused by the degeneration of upper and lower motor neurons.
Motor neurons send signals to the muscles from the brain and nervous system, enabling movement. When this communication fails, the muscles progressively weaken and waste away. Along with mobility challenges, ALS adversely affects cognitive functions, speech and swallowing.
When words fail
This June, the 2nd International Conference on Amyotrophic Lateral Sclerosis and Motor Neuron Disease, held at the National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, raised awareness about ALS.
Dr BK Yamini, professor, department of speech pathology and audiology at NIMHANS, discussed factors behind the alterations in speech and swallowing in ALS.
Citing an example from her study, she highlighted the case of a 42-year-old participant who experienced weakness in the bulbar muscles responsible for speech, chewing and swallowing. Initially, the participant’s speech remained coherent, but within a year, he developed dysarthria or speech impairment. Eventually, he completely lost the ability to speak (anarthria).
What are the causes
Dr Yamini explained that speech results from the combined harmonic working of the oral and respiratory systems. It needs breath support for effective communication. Speech issues arise in people with ALS due to the weakening of the jaw muscles. In addition, accessory muscles of the respiratory system weaken, which compromises the respiratory capacity, said Dr Yamini.
In the participant, lip and tongue movements became progressively slower and ultimately ceased in a year.
“The combined effect of all these factors is that the words and sentences became mumbled, leading to complete loss of communication,” she recalls. In ALS, speech slows down and becomes nasal due to a lack of movement in the soft palate.
The challenges
According to Dr Yamini, a significant hurdle lies in effectively communicating the condition to the family. She stresses the importance of educating the family about the progressive nature of the disease and how it affects the entire muscular system, as well as speech and swallowing functions.
“Since the person is going to take longer to speak a sentence, families need to give time to that person and allow them to communicate whatever they want to,” she advises.
Read more: Fading signals: detecting the symptoms of amyotrophic lateral sclerosis
Interventions required
In her talk, Dr Yamini also outlined a few management strategies for people with ALS who face speech difficulties:
- Since breathing is essential for speaking, they should be educated on safe speech breathing techniques. They should be instructed to use breaths effectively and to pause between sentences to prevent fatigue by sustained speech.
- Using alternate modes of communication like writing, gestures, facial expressions, nodding of the head and communication apps on electronic devices can be very helpful.
- Low-technology devices such as communication boards and alphabet charts and high-technology devices like speech generation devices or eye tracking systems can be used for communication. (Hawking used speech-generating devices for communication.)
- Environmental modifications play a role too. Paying full attention to the speaker or taking them to a place with less background noise is essential.
- Another option to consider is voice banking. Here the individuals can record useful and required daily communications when the problem is in the mild stages and use the recordings later when speech worsens.
Swallowing issues
Dr Yamini explained that participants also found it difficult to swallow due to the weakening of the jaw, tongue and respiratory muscles. Swallowing got worse as the condition progressed. “It is important to address this issue to optimise and maintain nutrition, hydration and medication intake for the individual,” she said.
Some of the strategies to manage swallowing issues include:
- Postural techniques: Sitting upright with head down while eating.
- Bolus control: Taking smaller mouthfuls of food, which is properly chewed for safe swallowing.
- Diet modifications: Providing softer food choices if they cannot chew a hard meal.
- Slow chewing and eating: Not rushing a person with ALS to eat quickly.
Dr Yamini emphasised that the family plays a crucial role in ensuring a better quality of life for those affected by ALS.
