Anne Bulger was a 44-year old technical assistant with a major Canadian insurance company when she began experiencing problems with her speech and mobility. After six months of tests and delays, she was diagnosed with Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's Disease. Given approximately three years to live, she died a mere six months later.
Anne Bulger was an unusual young woman. Prior to her illness she was known to be intelligent, earnest, and hardworking. It was during her battle with amyotrophic lateral sclerosis - ALS - that other facets of her character emerged, including an enormous courage and strength of will - as well as the shocking eloquence she revealed in describing her own illness and impending death.
ALS is not a good disease to have. It is a progressive and incurable neuromuscular disease characterized by gradual paralysis of the muscles of speech, swallowing, and breathing. Death usually results from respiratory failure 2 - 5 years after the appearance of initial symptoms. Most of my patients elect to prolong their lives with a feeding tube, but Anne Bulger would not accept this treatment. For all of us who cared for Anne, we accepted this decision as another expression of her courage to choose, even when the available options had dwindled to a vestige of their normal human abundance.
We have few clues as to the cause of ALS, a neurodegenerative syndrome caused by death of nerve cells that supply the voluntary muscles. Though inexorable and merciless, ALS spares many neurological functions, including eye movement, bladder function, and sensation. Mental function is also spared, so that memory, personality, and emotional life are sustained into the last hours of life, along with the precious interactions with family and friends. This is, perhaps, a good thing about a dreadful disease.
The consequence of preserved mentation is also a prolongation of the capacity to suffer. Anne clearly articulated the anger and sense of abandonment that a fatal illness can evoke in our self-conscious species. I last saw her in a palliative care unit about a week before she finished this journal, unable to eat or drink, still struggling to communicate by indicating letters on an alphabet board.
At that meeting, we "talked" about the faith that informs us of a reason for everything. You hold in your hands the honest account of a young woman's suffering and death. Its value is neither to instruct nor uplift, but to document Anne's ALS. It is surely the most harrowing testimony of this malevolent disease I have spent the last 20 years trying to treat and understand.
Dr. Neil Cashman, M.D., F.R.C.P. (C)
Director, ALS Centre,
G.F. Strong Rehabilitation Hospital,
Vancouver, B.C.
Professor and Canada Research Chair,
Brain Research Centre,
University of British Columbia,
Vancouver, B.C.