Some are given a death sentence

There are some diseases that are pretty scary. I suspect even when a physician informs a patient that there are treatments available for a newly diagnosed cancer that doctor and the patient are not totally convinced they will be cured. However, today prognosis is pretty good for most cancers.

There is one diagnosis which is essentially a death sentence. When a doctor says the words, ALS, or amyotrophic lateral sclerosis, the individual on the receiving end of those words quickly understands they do not have much time left.

An article in the Chronicle Herald's Lifestyles section brought tears to my eyes – and memories of a friend lost a number of years ago. A 24-year-old Dartmouth woman, Erin Hines, was diagnosed with ALS this past January. She had been a NSCC student and was looking forward to graduating. Now...instead of a career in a doctor's office as an administrator she is facing a very bleak future.

Then there is William Duff, a 31-year-old father of two very young children. In 2015, he began to notice twitching in his muscles and his hands were cramping. A year later he started seeing doctors. They suspected ALS, but it was unusual for someone so young to develop that disease. He got the official diagnosis in 2017. At this time, he is in a wheel chair and is unable to hold his cards while playing a board game with his children. This young man, who had been working as an engineer in the Navy, is also facing a very bleak future.

The fact that these two individuals are young adults would have made diagnosis more difficult. Most times the onset of ALS occurs well into middle age, with most victims being in their 50's and 60's. Even when this condition occurs in an older person it is difficult to diagnose. There are no tests for ALS, and the diagnosis comes through a process of elimination. All other possibilities are put aside after testing for those conditions.

ALS is a motor neuron disease and the average survival from onset of the disease is two to four or five years. Approximately 10 per cent of those with ALS survive for 10 years. The physicist, Stephen Hawking was diagnosed in 1963 and passed away earlier this year. Hawking was definitely the exception.

Because of the success of the Ice Bucket Challenge, $20 million was raised this past year. However, it is expected that amount will drop to between $1.5 to $2 million this year. Since ALS is quite rare – about two cases per 100,000 – it is has an “orphan” disease status in Australia, Japan, and the United States which allows for some tax breaks and special funding for research. That status has not been adopted in Canada.

William Duff would like to try experimental drugs prior to approval. Apparently, there is one drug that has slowed down...and even stopped progression of ALS in animals but at this point he is not allowed to give it a try. I can understand his frustration. It is not likely the drug would cause more harm than the disease.

June is ALS Awareness Month. As stated earlier, I had a good friend who passed away from ALS. I think I will visit the ALS Society of Canada on-line site and make a contribution in her memory. Maybe a few folks would like to donate so young people like Erin and William have a chance to live long and happy lives.

Shirley Hallee is a freelance writer living in Amherst. Her column appears weekly in the Amherst News.