Jerry was exhausted, fearful, and confused by the information his mother had confessed to knowing. On one hand, he was shocked by the limited time he had left to live, and the realization that he would immediately need to confront his wife with the grim diagnosis of his disease. What was worse, though, was the thought that he would have to pass on to Cheryl the knowledge that their daughters might devastatingly be impacted by the diagnosis and that their lives, from this point forward, would carry the burden he had brought on to them.
Jerry realized that he’d need to select a friend, or a confidant, someone he trusted implicitly, to assist him on his journey. And he also knew that he had to make this and other decisions while being sleep-deprived, and experiencing a decline in his mental abilities and the nightmarish hallucinations that would soon pervade his thoughts.
Unlike his parents, who identified themselves as Christians, Jerry had lived his entire adult life without a belief in God. Cheryl was also a non-believer, but had gained an innate understanding of her purpose in life the moment she knew she was pregnant with their first child, Sharon.
Jerry knew that Cheryl was the more essential parent to their daughters. She was more than a mother, wife, and companion; she was the stabilizing force of the family and the glue that held their relationship together. Jerry had been comfortable being secondary in value, and understood that Cheryl would, by her nature, hold him responsible for the transmission of the disease to them, at whatever age they might develop it after he was gone.
Reflecting on his mother’s reason for holding back information on a disease that she might have suspected to be heritable, he understood that there had been no easy choices for her over the years. Either way she might have been wrong, and had counted on God to answer her prayers.
The $1 million life insurance policy he had purchased ten years ago might assist in his family’s financial survival after he was gone, but the policy mught not be enough to cover the cost of the care he’d require prior to his death. He also knew that if he chose to “do himself in,” the policy might become null and void.
On his way out of the parking lot, Jerry received a message from Cheryl, telling him that she and the girls had just returned home from the rental. Thus far, she had been made aware only of the sleep test and the PET scan prescribed, but had not yet received the results of any tests or the prognosis.
Under the circumstances, Jerry realized that there was no time to waste before taking action. Any delay would only add to his upset and would appear evasive to Cheryl rather than constructive. Although he and his partner had never been the closest of friends, he made the decision to trust in Dave’s judgment prior to confronting his wife.
Dave, 14 years Jerry’s senior, was respected by clients and publishers alike for his thoughtful approach to literature. Although Jerry’s diagnosis would no doubt be a shock to Dave, he knew that his partner processed information quickly and was most likely the optimal person to confide in, especially now, during the initial planning stages.
While veering right on Walnut Street, Jerry picked up his phone and called Dave.
His partner was cheerier than usual and asked, “Welcome back! How was the shore?”
“What can I say?” answered Jerry. “It was the shore [he forced a chuckle], and you know that I’ve been working rather than enjoying the sand and the surf.”
“I’ve suspected as much. Why the call?”
Without hesitation, Jerry blurted out, “I’ve just come from my doctor and have been given some unpleasant news. Because of it, we have to talk. But I’m also in need of your advice and counsel.”
There was a pause, as Dave struggled to comprehend Jerry’s message, but he answered flatly, “Whatever you need, Jerry.”
“Are you busy now?” asked Jerry. “Or should I stop by later today?”
“Nothing that I have going on is that important that it can‘t wait,” responded Dave.
“I’m only a few minutes away, Dave... I really do appreciate this.”
He found his way to the parking lot and took a ticket while formulating his thoughts for his talk with his partner. What he didn’t want to do was overwhelm Dave with details or the emotional magnitude of the upset he was feeling.
Upon arriving at the agency, Jerry was welcomed back by the staff and escorted to Dave’s office. He put on a smile and tried his best not to show the gravity of the situation.
Dave’s door was ajar and he knocked before entering. Dave got up from his desk, walked towards Jerry and put an arm around his shoulders, something Dave’d never done in all the time Jerry’d known him.
“It must be bad, Jerry,” said Dave. “You know that we’ve seldom talked about our lives over the years. Sit down and let’s go through it.”
Dave led him to the leather chair that faced his desk, and then walked around the stack of manuscripts he moved so that they could face one another.
“I’ll start with the worst of it, Dave. I’ve got less than a year to square up everything in my life before I’m either dead or not worth shit.” Jerry paused. “At best, I have another six months before I’ll be a burden to myself, my family and the world.”
Dave folded his hands together and looked squarely into Jerry’s eyes. “I’m here to help, however I can. Tell me what’s happening and what you need of me.”
For the next thirty minutes, Jerry poured out a condensed and somewhat confusing report of his symptoms, including the sleepless nights and weariness, and his final diagnosis of Fatal Familial Insomnia. He also spoke about his discussion with his mother and his family history that, if not hidden, was ignored by his parents and other family members. He also spoke with great emotion of having to tell his wife the truth about the disease that would possibly pass on to one or both of his daughters, as well as any children they might have.
“Imagine, Dave, you do everything right in life, as Cheryl has, and then she finds out that there’s a time bomb ticking in her husband and her daughters.”
Dave stopped him at this point and asked, “Is there absolutely no cure on the horizon? Something that might stop this disease from being passed down?”
“Frankly, I don’t know,” answered Jerry. “And neither does my neurologist and my doctor.”
“This isn’t the 14th century, Jerry. Cures are being found every day for little-known diseases.”
“There are only 50 to 70 families in the world known to carry this genetic mutation. At this exact moment, Dave, I am one of less than 1000 people in the country who have it. They’ve told me it’s a prion disease, whatever that is, but what it means is that the odds of one or both of my daughters of having it are 50-50. What I do know is that it’s a rare and a rapidly progressive neurological disorder that is always fatal.
“In answer to your question, yes, there’s an outside chance that a gene-editing device called CRISPR could, in the next few years, find a way to ‘silence’ the single gene that causes it, if they can find and correct the ‘typo’ in the gene. If discovered and fixed prior to any signs of the disease, my daughters may be granted immunity. But this specific gene is such a rarity, that it’s at the bottom of the list of thousands of diseases being studied for genetic alteration.”
“But there is hope?” asked Dave. “Not for you, but for your daughters?”
“There’s always hope, but that’s going to be a hard sell for Cheryl and the kids. Nobody even knew about FFI—that’s what it’s called—until the 1980s, so my mother had every reason to keep quiet about the deaths of her mother and sister. But there’s no place for me to hide it from my family, or offer any great “hope“ of a recovery.
“My life is over, Dave, and that’s the next thing on my agenda. How I manage the financial burden along with the emotional trauma I’ve inflicted on my family as well as my responsibilities to this business.”
“I understand the issues with your family, Jerry. But as we all know, anyone can be replaced in a business. As far as your financial concerns, you not only have an insurance policy for your family, but our firm also has a life insurance policy on you as a partner.
“When you pass away, your wife will succeed you as a partner in the firm. This was contracted when we first entered into the partnership. And we both were required by law to have an insurance policy on each other.”
“And how much was that for, Dave?”
“I believe each was for $1 million. Since you know that you’ll most likely die within a year, you will help us find your replacement. With that person in place, you remain a partner until death and then the firm gets a payout. Your wife sells your share to the firm for your half of the policy amount, which would be $500,000.
“So she won’t be broke, Jerry. And the firm’s medical policy will cover a great deal of your medical expenses, while you continue to get your salary paid until you pass away. This all was stated in our agreement.”
“But I won’t be really working after a few more months. I’ll be worthless here,” said Jerry.
“I don’t think that will be the case.
“As I’m listening to your story, I find it fascinating. I believe that many others will also find it equally interesting. My suggestion is that you consider becoming a client of our agency as well as a partner.”
“But you know that I’m not a writer. I’m good at what I do, but I’m not skilled at the craft... or have that kind of talent.”
“Let me stop you there, Jerry. You work with many skilled writers, and you’ve sold many of their stories to publishers. Pick a writer from your list, or I will from mine. He or she will do the heavy lifting of the wr
