After consulting with his doctor, Jerry called his mother, who answered the phone pleasantly, but with a trace of hesitation, as she had done many times in the past when hearing from her son.
“Well, hello, Jerry. How are things going in Avalon?” Dorothy had just turned 83, but always seemed younger than her age on the phone.
“Hello to you, Mom,” Jerry answered back curtly, and proceeded to tell his mother about his recent sleeplessness, the tests, and a possible cause of his imminent illness.
Dorothy listened quietly as Jerry spoke in an measured tone about the very real possibility of having a rare inherited disease that was, at this time, incurable. As part of his questioning, he asked his mother if there was anything she needed to tell him about his condition.
There was a pause on Dorothy’s end of the line, after which she began to speak in a measured tone. “I don’t know what to say, Jerry. Except that I am...so sorry.” And after that Jerry heard crying over the line. He waited a while until the sobbing became a sniffle, and then responded softly, “So, what happened, Mom?”
At first it was difficult for Jerry to understand her, since she blurted out a series of disconnected facts that made little sense. Finally, she calmed down enough to explain the circumstances of her mother’s death in the 1960s. Jerry’s grandmother was in her late forties at the time and had told her husband, Walt, one night that she recently had become very tired and couldn’t seem to get a good night’s sleep. Finding nothing physically wrong with her, her doctor prescribed Valium for anxiety. Over the next few months the drug seemed not to help her sleep, and she then began to experience bouts of shaking and twitching. Her blood pressure increased, so her doctor put her on a new drug, verapamil. After two months with no improvement, she was losing weight and experiencing hallucinations. Her doctor decided for her to discontinue the use of all drugs. The symptoms continued and within six months she began to experience paranoia and other signs of severe dementia. She died within a year of the onset of her illness without any clear prognosis.
“I tried to find out what had turned an otherwise healthy person into an invalid so soon, Jerry, and since she seemed to die of natural causes, no autopsy was performed. There was some talk from her doctor of her having a neurological disease, but all he could do at the time was offer ‘guesses.’
“Then when her sister, your Aunt Hazel, developed some of the same symptoms in the 1980s, Walt and I told her doctors about our mother. But at that time there were no PET scans, and the disease remained undiagnosable and untreatable after her death.
“The doctors had suspicions that Hazel’s death was caused by the same disease that our mother had, but their consensus was inconclusive.
“I, of course, had concerns that the two deaths were connected, and it appeared that they might be genetic, but not enough information was known in the 1980s to research the problem. I had only the hope that you or I wouldn’t contract a similar disease.
“By the time you and Cheryl married, and you had the girls, more information had become available. But at that point I didn’t know what to think or what to say. It was only after both of your daughters were near school age that I heard of FFI, and of it being a very rare and incurable disease. The worst news was that there was a 50-50 chance of a close family member contracting it at some point during their mid-life.”
Jerry interrupted his mother and asked, “So, why didn’t you let me know what you knew, Mom?”
“It’s a poor excuse, I know,” answers his mother.“ I didn’t want to worry you. I was over 50 and in good health, and after you aged past 40, and you didn’t show any signs of the disease, I intentionally blocked it from my mind. Having become quite sure that with all of the genetic testing being done, and no signs of any illness in any of us in the family, I assumed we’d all be okay. You, me, the girls... all of it caused by a misunderstanding on my part.”
“But you knew...Mom! I can’t believe that there wasn’t a time that you could have alerted me to the possibility of a disease when you knew about the possibility of a devastating outcome .”
“I didn’t really know, Jerry. I only suspected there might be a possibility. I didn’t want to be an alarmist. In many ways, I’m as surprised as you are. What could I have done? Tell you not to get married or have children? Let you know that you might not live to see your grandchildren?
“That was the hardest part. Imagine me giving you and the girls a 50-50 chance of a death sentence without any concrete proof.”
“Did Dad know?” asked Jerry.
“He knew what I knew, but that was all. We even talked with our doctor and our minister about our fears. They were of no help. We spoke with a neurologist about it after Hazel died, but there wasn’t enough evidence for them to go on. If Hazel had had a brain scan, they might have found the tiny holes and shrinkage of her thalamus, but MRIs weren’t sophisticated enough in the 80s to catch a rare disease like FFI, and doctors could only hypothesize concerns, not having any means to act on them.
“So, yes, we knew something about a genetically-passed-on disease, but not enough to frighten you and your family. There was nowhere we could turn to confirm our suspicions of Hazel’s or our mother’s brain metabolism.”
Dorothy apologized once again, but Jerry, although still angry, was able to put himself in his mother’s position, since some of his concerns were hers. He had two daughters. Either one, or both, could die early without having children of their own and harboring their same fears.
In that moment, Jerry realized that he had limited time to rescue his daughters from his own fate. And even if he couldn’t live to alter the path for them, he could do everything possible to fight for them to secure their future...besides serving them a death sentence.
Jerry understood that his mother really had had no choice. The technology hadn’t been available to save his grandmother, aunt, or him, but he was living in a time when he could play an active role in preserving the lives of his children, grandchildren, and many future souls in his family from an identical fate.
“Mom,” Jerry said quietly. “This isn’t your fault, but I’m glad I know my mission. It’s a much grander plan than any I might have chosen. While I’m able, I want to take care of my family and protect them from our family’s demise.”
“The first thing I’ll do is speak to my partner at the firm, and work out an arrangement. And then I’ll speak to Cheryl and the girls so that they have the time to understand my fate... but also my mission to do all I can to protect them all from the position we’re now in. They’re old enough to know what’s going on, and Cheryl’s a strong and resourceful woman.
“So, thank you, Mom. Let’s work together on this with Dad. I’m sure he’ll understand.”
“In his way, he’s always understood, Jerry. It’s always been me who hasn’t.”
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