Stephen Hawking and Amyotrophic Lateral Sclerosis (ALS)
His speech control and intelligibility also diminished, and only those who were well acquainted with him were able to understand him. In 1985, he had to have a tracheotomy and, as a result, he completely lost his ability to speak.
By: Zouheir Kabbara and Samantha Mora
Who is Stephen Hawking?
Who is Stephen Hawking?Stephen Hawking is an incredibly reputable name in scientific circles and other communities around the world. Born on January 8, 1942, Hawking was diagnosed with Amyotrophic Lateral Sclerosis (ALS) in 1963 while he was attending the University of Cambridge. While the diagnosis certainly changed his life, he continued his education and is now known for ground-breaking work in both physics and cosmology.
What is Amyotrophic lateral sclerosis (ALS) and how does it affect speech?
ALS is a rare, neurodegenerative disease that affects both upper and lower motor neurons, resulting in muscle weakness and eventual paralysis. An estimated 20,000 Americans are currently living with ALS and, according to the ALS CARE Database, 60% are men. ALS initially presents in one of two ways: some patients will have a bulbar-onset, in which dysarthria and dysphagia are the primary symptoms in the beginning of the disease, while others, like Hawking, will have a spinal-onset, in which muscle weakness in the limbs is the initial symptom. Along with motor dysfunction, up to 50% of patients develop cognitive or behavioral symptoms as well. Although the exact cause of ALS is unknown, genetic factors have been implicated in some cases. Unfortunately, there is no cure for ALS, and most people with ALS have a life expectancy of 2-5 years after diagnosis. There are two drugs currently available that can possibly slow the disease progression, but the main focus for healthcare providers is still on the management of symptoms, such as providing muscle relaxants for spasticity and speech therapy for dysarthria, to improve patients’ quality of life. Regardless of the type of onset, most ALS patients develop spastic dysarthria (and potentially flaccid dysarthria later) at some point in their disease process and many eventually become anarthric. While speech therapy is useful in delaying the progression of the dysarthria, it cannot reverse the effects due to the degenerative nature of the disorder. Communication supports, on the other hand, can assist ALS patients in addressing their daily communication needs throughout the course of their disease. A study published by Neurorehabilitation in 2015 concluded that using communication supports, such as augmentative and alternative communication (AAC) devices, should be standard practice for patients with ALS, as it can help them maintain functional communication and allow them to remain active in daily activities for the rest of their lives.
Hawking’s journey with ALS
As mentioned earlier, Stephen Hawking was diagnosed with early onset, slow progressing ALS at the young age of 21. He initially noticed some symptoms but was reported to have kept them to himself until his father detected them and took him to a doctor. After receiving his diagnosis, the disease started to take effect. His motor abilities diminished, resulting in him becoming wheelchair bound and, by the mid 1970s, he required assistance for many of his activities of daily living. His speech control and intelligibility also diminished, and only those who were well acquainted with him were able to understand him. In 1985, he had to have a tracheotomy and, as a result, he completely lost his ability to speak. Following that surgery, he needed 24-hour nursing care.Soon after that, Hawking was contacted by a computer programmer who was developing an AAC program called Assistive Context Aware Toolkit (ACAT) through Intel that would allow the user to essentially speak by using eye or head movement to choose what to communicate, and then the program would synthetically speak on the user’s behalf. Hawking, whom was integral to the development of the now widely available program, originally started using the program with a handheld clicker but now uses a sensor attached to his cheek muscle, as he has lost control over most of his body muscles. This high-tech speech generating device technology has been a successful tool for Hawking, allowing him to effectively communicate with peers, write scientific papers, publish several books, and continue to lecture at Cambridge University all the way up until his recent retirement in April.
How does Stephen Hawking’s ALS differ from the norm?
According to the ALS association, the average life expectancy of an individual with ALS is 2-5 years after diagnosis, but it can be variable, and about 5% of individuals living with ALS live longer than 20 years. Hawking has lived with ALS for an astonishing 54 years now, which is definitely considered an outlier in this population. Additionally, most individuals are diagnosed with ALS between the ages of 40 and 70 years old. Hawking was diagnosed at an extremely young age, which may help to explain his longevity, as people who are diagnosed at younger ages tend to have slower progressing disease processes and longer lifespans. Regardless, Hawking stands out as a true anomaly and is a prime example of how truly variable ALS can be. Furthermore, his accomplishments demonstrate how people with neurodegenerative disease can still be successful, and how AAC devices can be effective in greatly improving their lives.
ALS Association (2016). Retrieved from http://www.alsa.org/about-als/facts-you-should-know.htmlBiography.com (2017).
Stephen Hawking. Retrieved from https://www.biography.com/people/stephen-hawking-9331710
Fried-Oken, M., Mooney, A., & Peters, B. (2015). Supporting communication for patients with neurodegenerative disease. Neurorehabilitation, 37(1), 69-87. doi:10.3233/NRE-151241
Hardiman, O. et al. Amyotrophic lateral sclerosis. Nat. Rev. Dis. Primers3, 17071 (2017).
The Computer. (n.d.). Retrieved October 25, 2017, from http://www.hawking.org.uk/the-computer.html