In our family, assuming Great-Grandma Mae was passing on a gene, the fact that it seemed clear that she passed it on to two girls (my grandmother Shirley and her sister, Norma) and one boy (my great-uncle Nathan) did not by itself prove anything. But the fact that my great-uncle Nathan had passed his gene to his daughter and not his son offered one small but valuable conclusion—namely, that the gene might be on the X chromosome. When Uncle Norman fell ill, Dr. Kricket had another male subject. Even more conveniently, Uncle Norman had two biological sons. My father had two biological daughters. Hilary and I both had murmurs. My male cousins did not. Again, this made the X chromosome a contender.
In the genetic lottery, my father, Uncle Norman, and Aunt Kathy were each poised to get one of their mother’s X chromosomes. They had a fifty-fifty shot at receiving the X chromosome that my grandmother had received from her father, Rabbi Morris, or the X carrying a mutation, or variant, passed on by her mother, Mae. My dad and Uncle Norman both got the one that carried the variant, and Aunt Kathy got the other one.
It would not be easy to prove what Dr. Kricket was suggesting. Every time my cousin Jeff coughed, it was impossible not to wonder if the Seidmans were wrong. But if they were not wrong and the gene was on the X chromosome, Ellen’s children would never get sick. For his line of descendants, the gene would stop with Norman.
* * *
My uncle didn’t tell anyone he was sick because he had seen what my father’s death had done to his parents, my grandfather in particular. Everyone had heard the stories about my grandmother standing over her husband, who had been weeping desperately nearly every night since he’d lost his son. My grandma had finally convinced him to go on antidepressants, which had taken the edge off his despair. But all of us saw a distinct change in our grandfather. He was quieter since his son’s death and a lot less in the mood to teach us poker or play a game of Scrabble. My aunt Ellen and uncle Norman invented excuses for months about why they were going to skip Thanksgiving that year, why they weren’t interested in a family Passover seder, always avoiding too detailed an explanation. The secret was easy to keep, given that they lived outside of Los Angeles, more than three hundred and seventy miles away from my grandparents in Phoenix. When Uncle Norman contracted pneumonia at one point, it was the easiest and most truthful excuse they used. When my grandmother offered to come out and help while Norman convalesced, he and Ellen had to say no, encouraging her to stay in Phoenix instead.
My uncle wasn’t just keeping secrets from his family—he was also keeping them from his doctors. He hoped to give them a chance to reach an unbiased conclusion about his prognosis. He thought it would help them catch something that, in four years, my dad’s doctors never had. My aunt and uncle actively kept the fact of my father’s death from Uncle Norman’s medical team.
I wonder if this withholding of information, both from his medical team and from his parents, wasn’t also a way for Norman to avoid the terrifying truth, as if, by never saying something, it wouldn’t really be happening. In much the same way that forty years of silence had separated Mae and Nathan from Billy and Norman, this new yearlong silence might have been another way for Uncle Norman to turn away from the isolating and grim reality that awaited him.
Sixteen
As my grandparents stood before their son at my cousin Danny’s wedding, their faces went gray. Three years after losing their eldest child, they were now poised to lose their second. My uncle Norman had the same gaunt look that my father had had. Three of his children and his wife, Ellen, flanked him like guards, or perhaps it was only Norman’s vulnerability that made it seem like his family stood sentry around him that day.
I recall my grandparents dancing at the reception. My grandfather looked heavy in my grandmother’s arms. She leaned into him as if her body weight was keeping him standing up. My grandmother’s own mutated gene was set to claim yet another victim. It was her fault that her husband was going to lose another son. As I watched them, my grandmother in her bright blue pantsuit and diamond earrings, my grandfather with his silver hair, still thick despite his seventy-nine years, that dance was heavy and slow, more like a glorified attempt to remain upright.
From that point onward, my grandfather made a vow to his wife, his son, and himself: he would make amends for all the years that Norman had felt underloved and underappreciated by his parents. He arranged for a trip with Ellen and Norman to Las Vegas. On that trip, he spent meaningful time with his son. He was gentle with his sick boy, helping him carefully maneuver around the slot machines, sitting quietly by his side as they played the games, the noise of Vegas never penetrating the peaceful bubble surrounding father and son as they healed their relationship, even as both their bodies weakened.
* * *
It wasn’t enough that Shirley’s second son’s death was imminent. My great-aunt Norma, my grandmother’s sixteen-months-younger sister, her lifelong companion, and most combative opponent, 2800 miles away in New Jersey, suddenly fell ill.
Norman was not named after Norma, according to my grandmother. Under Jewish law, you did not name children after the living. Still, I always associated Uncle Norman and Aunt Norma simply because of the similarities in their names. That January, Norma had begun to fill up with chylous fluid. Norma knew she had the gene. She had the telltale swelling and the murmur. Her doctor noted that the fluid Aunt Norma was accumulating was “very unusual.” It was lymphatic exudate—that “pretty” light lemon-yellow-hued gravy called chyle.
A generation separated Norma and Norman, but their bodies were arriving at the same point at the same time. Norma was seventy-five and Norman was fifty-one. Similarly, a generation separated Mae and her son Nathan, while only a handful of years separated their deaths.
This reality served to bolster Dr. Kricket’s assertion that the X chromosome was going to be a major player if she could track down the specific gene. The reason is a phenomenon called “X-inactivation.”
If you Google “X-inactivation,” you will almost certainly come across a bunch of pictures of adorable cats, which might have been exactly what our family needed to cheer us up back in 2001, if you could even Google adorable cat pictures back then. The reason for these search results is that a calico cat is a visual reminder that females have two X chromosomes.
Calico cats are so named because of their multicolored, checkered fur coats. For a brief moment, when an embryo that is chromosomally XX is in its zygotic state, both X chromosomes express. Imagine both X chromosomes flashing with light in every cell for just that short period of time. It’s almost like the cells are having a little test run to see which X they like better. The chromosomes express themselves in this way only for a few moments, but in cats, that moment becomes forever etched in fur. If one of the cats’ X chromosomes selects for black fur, and the other X selects for orange, a mixture of both in varying shades will remain. After that moment of dual expression, one X will package itself up and go silent while the other X carries on. This also explains why XY male cats are almost never calicos. In fact, all calico cats are XX females unless they have a chromosomal anomaly.
With all the other twenty-two pairs of chromosomes in our cells, both chromosomes in every pair express with very limited exceptions. Only the X chromosome likes to work alone. Some scientists believe it has to do with evolution. Both the X and Y chromosomes are big mutators. In almost every generation, some degree of mutation takes place in both.
Truth be told, they seem largely to be mutating to avoid killing each other off—you know, like in a marriage. One study of fruit flies allowed a male strain to mutate freely over twenty-nine generations. The female strain was bred repeatedly so that it never evolved and was virtually unchanged. When the twenty-nine-generations-into-the-future male fruit fly fertilized the “ancient” female, his sperm actually poisoned her. In human terms, that’d be like a man from today having sex with someone from the 1500s. Turns out all King Henry VIII needed was a man circa 2016 to take out his queens, and he could
have avoided all the marital bloodbath.
So, perhaps as a tool of self-preservation, the X, unlike all the other chromosomes, has mastered the art of working alone. For a brief moment, her partnering Y must still code for things like testes and James Bond movies.
If the X chromosome’s partner is another X instead of a Y, as in most females, she still won’t want to share her efforts. After that momentary dual activation of both X chromosomes, only one X carries on while the other goes silent. Some scientists believe it is the “better” X that stays active in the cells, but there seems to be some degree of random mixture—a maternally inherited X remaining active in some cells and the paternally inherited X in others. Norma (like her mother, Mae) very likely had some mutated X’s from her mother expressing in some of her cells and in other cells she had the X she inherited from her father, without the mutation. Or it’s also possible that in the moment when both X’s were active together, there remained that lingering residue of the silent X, like on the coat of a calico cat, that would play a role down the line in the life of an XX female. Either way, the probability is that Norma had a better chance of living longer, given that her body had, or momentarily had, another X from which to acquire information. In the cases of Norma and Mae, they each eked out an additional generation.
Infer whatever jokes you will, but the Y chromosome inherited by men is one of the smallest, least complex chromosomes a human can inherit. Once it has completed its task, it is packaged up and silenced, leaving the mighty X to carry on with her solo expression.
Nathan, Billy, and Norman, as men, inherited only one X chromosome. Because Nathan, Billy, and Norman’s bodies had no healthy version of an X chromosome like their female counterparts, the illness in them seemed to come on harder and stronger. It was killing them faster and in more devastating ways. Dr. Kricket liked to remind Hilary and me of this by saying, “You don’t have what your father had.”
Meanwhile, our ever-spry and aging grandma Shirley would echo, “You’ll live a long life like your grandmother.”
Still, Norma’s decline was rapid in the end. Her son-in-law Vinnie concocted rich smoothies for her to drink. It was the only food she could keep down. Her daughters sat with her every day in her hospital room. Like Uncle Norman and the others who preceded her in death, the biggest problem for Norma was an inability to absorb protein. When her medical team inserted a feeding tube through her nose and into her stomach, the hope was that her nutritional levels would improve, but the tube irritated her. She was in pain. Finally, she asked that the tube be removed, but by then the irritation was so great that it brought on an infection.
Norma died in April 2001 with her children, grandchildren, and husband, Charlie, at her side. The infection caused by her feeding tube was listed as the cause of death, but the chylous ascites was everywhere. Dr. Kricket brought Norma’s body to Boston to conduct her autopsy.
* * *
With my uncle dying, my grandparents didn’t want to go to Aunt Norma’s funeral, so Hilary and I decided to go on their behalf. We flew to New Jersey the morning of the funeral. Although it occurred to me that I shared the gene that killed Norma, I was soothed by the fact that she had lived a full life. Hilary thought the funeral was a little more ominous.
I overheard a conversation that day, that at the time I just took as a little bit of gossip floating around among cousins: my cousin Valerie, Aunt Norma’s oldest daughter, had had her spleen removed six weeks prior. I heard people discussing it across Norma’s kitchen table after we returned from the cemetery.
Valerie looked great. It was clear that whatever had happened, she was back to her old self, fully made-up face, healthy, and now laughing about her recent medical scare. No one knew what had happened to the otherwise healthy forty-five-year-old. She had thrown up and the vomit looked like coffee grinds. When the doctors investigated her condition, they discovered her platelets had plummeted to near-fatal levels. Her spleen was grossly enlarged. Upon removal, she told us, it was “mushy.” I’ll admit, I didn’t even perk up at the word “platelets.”
By the time of the funeral, Val was healthy again, and her anecdote provided the background banter of another funeral. Her illness was just another medical mystery in a family who was absolutely steeped in them. At the time, no one connected Valerie’s strange condition with the other strange condition that now had a growing body count. After all, mushy spleens hadn’t been a problem for any of the stricken relatives.
So the next morning, without fanfare, Hilary and I flew back home.
Seventeen
“Over the past few months I have been forced to look at you from a new perspective,” Norman wrote to Ellen on Valentine’s Day, 2001. “I have to sit back and watch you plan for a not so distant future alone.
“I am now blinded by the sheer magnitude of the love I feel from you,” he continues. “The warmth of that love quiets any fears or anxieties I may have for my own future.”
The letter is a heartrending tribute, a testament to a twenty-eight-year love affair, coming to an end. “My life has a validation few people could ever hope to achieve,” he concludes. “Validation based not on the love that I have given to you, but on the love that I have been so honored to receive from you.”
He signed it, “Happy Valentine’s Day. I love you, Norman.”
My uncle grew sicker over the summer. He visited someone best described as a witch doctor. He was sent home with what looked like a Geiger counter and a bottle of vodka. By September, he still lacked any useful answers. On September 11, at two a.m., he woke up. He began frantically hyperventilating in bed.
“I can’t breathe,” he told my aunt, who had woken up in the commotion. She asked him if she should call 911. “No,” he replied, “I need you to hold me.” So my aunt put her arms around him, holding my uncle and trying to soothe his labored breathing. Although across the continent, planes were flying into the World Trade Center towers, that is how my aunt remembers the events of 9/11.
Uncle Norman’s doctors remained most worried about his protein levels, which had plummeted to near-starvation readings. He was sent home with bagfuls of albumin, which had to be pumped directly into his veins through a pick line in his subclavian artery, located just beneath his collarbone. Aunt Ellen also prepared protein shakes (happily made of actual protein, unlike the ones Norman’s uncle drank in the sixties).
In November of 2001, my grandfather was diagnosed with stage-four colon cancer. Two months later, he died. I imagine he just couldn’t watch another of his children go before him. Uncle Norman went to his father’s funeral wheelchair bound. My grandma sat stone-faced beside her dying son, before the casket of her dead husband, with her oldest child five years gone and her sister only months in the ground. Aunt Kathy wept quietly at her side. My grandparents had been married for fifty-seven years.
* * *
Uncle Norman’s son Aaron was engaged, set to marry that July in Los Angeles. Norman promised himself he wouldn’t die before the wedding.
My uncle’s life, much like my father’s, was built around doctors’ visits. My aunt Ellen tried to stay positive, but she didn’t believe he would make it much longer. When they married, they had both signed living wills, agreeing that they would not be resuscitated if doctors ever anticipated that they could not live without life support. Hours later, Norman had torn his up, saying, “I don’t want to be the guy who pulled the plug and the next day they find the cure.” But after seeing what his brother had gone through, he asked to go back to their lawyer to sign a new one.
The week before the wedding, my sister flew to L.A. to take my uncle to doctors’ appointments and free my aunt to deal with coordinating the wedding. There was so much to do, and Ellen wasn’t sure she could handle it all. Uncle Norman loved getting tapped—having a pump inserted to pull out hidden pockets of scar-tissue-locked fluid. Our father had loved it too. Tapping the fluid meant making room for airflow to thirsty lungs. It meant being able to breathe. Hilary took him e
very other day.
At the wedding, Norman slowly walked down the aisle with his wife at his side. It was the last long walk he ever took.
On October 14, 2002, Norman died. The cause of death on his chart read: “Respiratory failure due to massive chylous pleural effusions due to severe lymphedema.”
Eighteen
During my uncle’s illness, I was living in San Francisco. My boyfriend at the time was an avid pot smoker. Once, I asked his best friend if it would be okay to tell my boyfriend that I liked him better sober. The best friend looked shocked and replied, “That would be like telling a fat person you’d like them better skinny.”
As a once-heavy pot smoker myself, I knew I was being somewhat hypocritical, but I had all but stopped smoking. I had moved on to whiskey and denial. My father’s death was firmly in the rearview mirror of my life, and I refused to let all the unknowns of a theoretical gene get in the way of my pleasant, if myopic, view of life. I was healthy. Other than my chronic low-platelet count that sometimes left me with mystery bruises, I felt healthy. Just like it’s hard to make dinner when you’re not hungry, preparing for an unhealthy future is often that thing on your to-do list just below dusting the tchotchke shelf and nose-hair plucking.
I focused with all my might on my healthy octogenarian grandmother. If she wasn’t sick, I wouldn’t get sick. I called and visited her regularly, mostly to feel her place her hands on my cheeks and say, “You’ll live a long long life like your grandmother.”
My grandmother’s legs remained thick with edema. She wore heavy-duty support stockings that she pulled on slowly and with grunts. I never thought about all the effort she put into getting dressed, and although she was otherwise strong and spry, I chalked her difficulties up to old age. So if I was busy comparing myself to her, it shouldn’t have come as a shock that one day I noticed my ankles looked swollen.
The Family Gene Page 11