Amyotrophic lateral sclerosis (ALS) is one of nature’s worst diseases, up there with the dementias and some cancers.

For some, cancer has become a manageable disease. Not so with ALS.

It destroys motor nerve cells in the brain and spinal cord, leading to progressive paralysis in muscles that underpin posture and movements of the limbs, neck and trunk, breathing, swallowing and speech, all while leaving the mind intact.

Unfortunately, with ALS, like most other degenerative neurological diseases, drugs do little to slow the disease and certainly don’t stop or cure it.

ALS shows up in differing ways. Sometimes via weakness begins in a limb, other times with weakness holding the head up, difficulty articulating speech, trouble swallowing or perhaps shortness of breath.

Then it spreads to motor nerve cells in other regions of the spinal cord, brainstem and motor cortex and to a minor extent, regions beyond the motor system. 

The result is relentless, progressive and extensive paralysis. Worst for many who have ALS is their increasingly laboured speech, which makes it difficult for others to understand what the patient is saying — efforts that sap the energy of patients and visitors alike. 

Sometimes visitors and medical staff become baffled and uncertain about how to help the patient to speak with more ease and less frustration, while managing their own uneasiness and the unfamiliar strain of trying to make sense of what ALS patients with speech problems are saying. It’s tough. 

It’s best to let patients take as long as needed to make themselves understood while avoiding the temptation to interrupt them by verbally stepping on their efforts to “get to the point” — because the real point is to let them get there on their own without being hurried.

Patience goes a long way to making what seems indecipherable speech, decipherable. The struggle is similar to trying to figure out what someone is saying in a language with which you might be passingly familiar, but not enough to make sense of what they are saying to you.

But unlike Goggle, which does an admirable job of translating languages on the spot, so far there’s no such option for translating the slow, laboured and slurred speech typical of patients with ALS.

For that conundrum, there is some progress. Implantable electrodes positioned over the speech area of the neocortex coupled with AI-assisted computers have been harnessed to record and make sense of the patterns of complex electrical signals generated by hundreds, even thousands of nerve cells associated with specific syllables, words and phrases as patients speak. 

Progress is promising but slow and the technology is not ready for patients with ALS. Studies involve small numbers of patients, intensive fatiguing training and technical resources available only in a few high-tech centres in the world.

One simpler option would be to record what the patient says as they repeatedly read a prepared script containing common useful words and phrase and analyze them with self-learning algorithms for translating the patients’ speech into clear text or spoken language. 

Which brings me to Peter Earle, who I met 16 months ago and learned he has ALS.

Less than two years ago, the paralysis was confined to his right arm and hand, which later spread to the left arm, leaving him unable now to use both arms and hands.

Then last summer Peter developed the first hint that his speech was affected. It has progressed to where Peter labours with great effort to make himself understood and others struggle to understand him, creating frustration and misunderstanding for all. 

Should his speech decline much further he would be “locked in,” with a sharp mind but unable to make his thoughts, intentions and feelings known except by using an eye-tracking device to spell out words and sentences, one letter or perhaps a word at a time.

Eye-tracking works but is dreadfully slow and doesn’t offer the free give-and-take of social conversation of which Peter was a master. It was one of his gifts others enjoyed about him just a few months ago. 

Despite the escalating pace of his ALS, Peter remains who he was when I first met him: a naturally warm, engaging man with a knack for storytelling and well-honed social sense and humour, coupled with incredible resolve despite huge ongoing and increasing challenges.