Oh Dear, What Can the Matter Be ?
(with apologies for the plagiarized lyrics from the traditional English song)

Ian Edgars’s Voyage of Discovery in Learning to Live with Primary Amyloidosis.

January, 2010

In 1990 and 1992 I experienced two minor strokes which led to my taking early retirement from my employer of 37 years. At the same time, my wife Nancy and I moved from our home on the North Shore of Vancouver, Canada, to Maple Ridge in the lower Fraser Valley. Having moved, we of course changed Doctors. My wife, Nancy found a woman doctor who came highly recommended for her efficiency and good medical practices, who insisted on interviewing us to determine if she would accept us both as patients. This is fairly unheard of in Canada and we evidently passed muster and were accepted.

As part of the process, she checked my blood and kept track of things, keeping in mind the history of the two previous strokes. Although diagnosed as minor T.I.A.s, they did not seem minor to me, affecting my left side and then my right side, cognitive speech and writing ability, etc. We embarked on a regimen of regular visits and monitoring of my blood and general good health. It seems, after the fact, that she was trying to improve my blood count, but I had no idea what the numbers were or what they meant. Over the next several years, our Doctor tried several prescription “cocktails” to improve the readings, without significant improvement. I was somewhat anaemic and lacking in iron, but it was not critical and I put it down to advancing age.

Sometime later, we suggested that I be checked for celiac disease. I was somewhat familiar with the symptoms as my daughter had been gluten intolerant since age 16, and maintained a very strict diet, avoiding wheat products, etc. I was referred to an internist who performed blood tests, a colonoscopy and an upper GI. After some time and many follow-up visits and a biopsy, he confirmed that I was celiac and placed me on a wheat free diet, where I eschewed my beloved beer, cookies, pasta and all forms of wheat. We changed our habits and shopped entirely differently. We later read in Time magazine that celiac disease typically takes up to 11 years to diagnose. That was about the time it took to diagnose me.

After a couple of years, I began to cheat, sneaking the occasional beer or hamburger to relieve the humdrum of my boring diet. This alarmed my wife and my daughter warned me that when she slipped off her celiac diet she paid for it with pains like having a baby, expecting that I would be similarly affected. But because I had lost almost 50 pounds and there appeared to be no complications from eating the treats, I began to suspect an initial misdiagnosis.

Previously, I had complained of occasional numbness in my hands, which the Doctor had diagnosed as carpal tunnel syndrome and referred me to a physiotherapist for treatment, although I chose not to go because I didn’t think it would do any good, deciding to just live with the problem. Our family Doctor chose to retire from private practice at this time and took a salaried position at the local hospital, causing us to search for another family Doctor.

Sometime later, one evening I began walking as if I was drunk and generally unsteady. Concerned that it might be a reoccurrence of the strokes, we went to our new family Doctor and were immediately referred to the Emergency Ward of the local hospital. The ER Doctor put me through a battery of tests and determined that my heart and brain were mostly in order and there was no sign of a new stroke. He also observed and commented on my swollen neck, which I had presumed was just normal for old people. My wife often nagged me about talking with marbles in my mouth, but we did not think too much about it.

I was referred to a New Westminster specialist, who referred me to an ear/nose and throat specialist, who presumed it was a thyroid problem and sent me away. A further E, N & T specialist also examined me with similar results, but posed the possibility that it was macro glossia (swollen tongue).

I was eventually referred to my current Haematologist, who did a biopsy on my hip and arranged a further biopsy on my tongue, finally determining that I had amyloidosis. The disease appeared localized in my throat and tongue and did not appear to be attacking any other organs, such as the heart or kidneys, etc. My Haematologist, in consultation with the cancer specialists at the Vancouver General Hospital, recommended chemo therapy, which took place over 9 months with a monthly oral dosage of Melphalan and Dexemethasone taken over a 4 day span. This series of treatments lasted until March, 2008, with monthly blood tests at the local Lab, monitoring my counts, which is continuing to this day.

I experienced no particular discomfort or nausea from the treatment, apart from a general weakened condition and general tiredness. I did not lose my hair as I was already bald !

I also added an Iron supplement to my other prescriptions and regularly checked in with the specialist and my family Doctor. Once the chemo was concluded, I went back to my Dentist, who had not wanted to treat me during the process because of my weakened state of immunity. He found that there had been a considerable deterioration in my mouth and I was losing several teeth, etc. potentially costing several thousands of dollars. I was able to be referred to the Dental Department of the Cancer Clinic, where my case is being used as a teaching example due to the rarity of Amyloidosis.

As I understand the present situation, the amyloid is in a partial remission state, neither getting worse or better. That said, my voice and eating has been severely restricted and my strength and energy levels are reduced, and my appetite and weight are similarly reduced. I have to be extremely careful eating slowly and choosing my foods so that I don’t choke on the meal.

In retrospect, follow up visits to my earlier, Celiac Doctors have pretty well confirmed that I was misdiagnosed and, with the benefit of 20/20 hindsight, could probably have been dealing with the disease several years earlier. I count myself lucky that my experience was not as severe as other cases I have heard about and have been most appreciative of the support and information disseminated by the Association. Also, and perhaps most importantly, I am thankful to my wife, Nancy for her support and encouragement.

Soldiering on,
Best regards, Ian Edgar.

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Ian and Nancy Edgar
Maple Ridge, BC, Canada
Telephone/Facsimile: 604–463-2999
E-Mail: ianandnancyedgar@telus.net