In the 1986 made-for-TV movie of Deford’s book, there is a moment when the actor Craig T. Nelson hoists a small Gennie James into the air and turns her upside down, pounding relentlessly on her chest and back in the hope of loosening the death grip of that mucus. The actress calls out for him to stop, but Nelson paddles on. In all fairness, those thrusts and taps were all the Defords had to work with. Keeping your child alive is desperate and unpleasant work. Parents of CF babies in the 1970s were given only the most rudimentary of tools to try to slow the death of their young children. Truth be told, in 2016, the tools haven’t gotten much better. But back then, pounding and humidifying were the best they could do.
Eleven years before Alex Deford was born and one state over, Uncle Nathan was about to get a treatment plan. And like the Defords and other CF families, he was desperate to follow it.
* * *
By 1960, Nathan was thirty-three years old and filling up with a fluid that was similar to the stuff that had killed his mother not so long ago. His young wife, Joan, was harried, teaching full-time to make ends meet and raising two young children while her husband was admitted to the National Institutes of Health. The NIH was established as the Marine Hospital Service in 1887 to study cholera, a bacterial infection that European scientists were just beginning to understand. By 1902, it had grown to become the chief U.S. public health agency. For many, including Nathan, the NIH was the last hope when battling a rare or undiagnosed condition.
Over ten months of 1960, Joan worked at her school and took care of her children—Michael, three, and Phyllis, one—with help from her mother, who lived near their home in the Bronx. Every Friday morning she’d kiss her children good-bye for the weekend and leave them with her mother or their aunt Norma in New Jersey. After a long day of teaching, she’d make the four-hour train ride to Washington, D.C., then travel to Bethesda to join her ailing husband.
Meanwhile, Nathan’s pain was intensifying as the fluid infiltrated his body’s tissue. Notes from his chart include disturbing accounts of the agony wrought by a thickening of the tissue in his penis and testicles, among other places.
Nathan’s fluid came back more quickly and aggressively than Mae’s had. The link between his illness and his mother’s was so tenuous that, other than a few offhand notes about swelling in the legs of his sisters Norma and Shirley, there are very few mentions of a family trait. Though his sisters Shirley and Norma, his mother Mae, and his grandmother Ester Bloom all suffered from swollen legs, no doctors drew any significant links between Nathan and those family members.
When the NIH arranged for Nathan’s release, he still did not have a diagnosis. The doctors’ conclusion read: “Etiology of the basic disorder which manifests itself by chylous effusions in the thoracic and abdominal cavities is, after all these many months of hospitalization, still completely unknown.”
Distraught, and riddled with fluid, Nathan was discharged. He was terrified to find himself growing sicker and sicker while his wife and young children stood looking on. His albumin levels, a measure of circulating protein, had plummeted to a point that was considered all but fatal. Albumin is a protein made by the liver and found in the blood. It is imperative for survival as it is primarily responsible for growth and repair. We need to eat protein so that our livers can produce albumin. Most of the protein going into Nathan’s mouth was leaking into his body cavity before being digested and turned into albumin. Nathan was starving to death.
Since there was no way to stop the flood of chyle, doctors focused on alleviating Nathan’s discomfort. They hadn’t the slightest idea of what to do for a cure, so they worked with what they knew. At the time, the only known treatment for extreme cases of massive ascites, the medical term for a large amount of fluid in the abdominal cavity, was to tap it, as had been done to Nathan’s mother before him. They cut a small hole in his abdomen and used a tube and manual pump to suck it out. After they removed as much of the fluid as possible, doctors had to figure out a way to replace the nutrients it carried. Years later, they would attempt to do so using intravenous (IV) technology that continuously infused nutrition back into the bloodstream. No such technology existed during Nathan’s illness, so instead they froze liters of the fluid they pulled out of his body and sent it home in bags with his wife. She prepared some every morning as a beverage for her husband to ingest orally. He had to drink it.
Tapping the fluid and drinking it was my family condition’s version of the CF chest percussions, our version of holding our heads over bowls of boiling water. Doctors couldn’t yet replace every faulty brick in the building that was Nathan’s body, so instead they did what medicine has historically done and continues to do when faced with an untreatable monster: they size it up, take out a squirt gun, and stand there shooting. Or in Nathan’s case, they made you drink your body’s waste.
Nathan died at the age of thirty-four in the spring of 1961. His final diagnosis simply lists his symptoms: “Chylous ascites of undetermined etiology. Ptosis right upper lid of undetermined etiology.”
* * *
My father’s cousin Michael was Nathan’s oldest and only son. My father was eleven years older than him, but they were close, in part because Michael, who grew up in the Bronx, moved to Pittsburgh, just three hours away from us in Columbus, in order to attend graduate school. Michael and his girlfriend Susan, whom he later married, regularly came to visit, and we went to visit them repeatedly over the years.
Michael, only four when his father died, recalls very little mention of his father’s illness among the cousins in the years following his death. The truth was, no one had connected Nathan’s illness to Mae’s. Michael described his father’s illness to Sue before they got married in 1982, referring to it as “an unusual unlucky kind of a thing.” He had called it a “blocked thoracic duct,” which, as a diagnosis, had in fact been ruled out. When Sue asked if the illness was hereditary, Michael told her that the doctors assured his mother that it wasn’t.
He did wonder from time to time if he might get it, until he passed the age of thirty-four—the age his father had been when he died. After his mid-thirties, those fears diminished. He figured if he did contract a disease, it would be something he could beat if he stayed healthy enough. Perhaps the family lore that mostly suggested Nathan had been “sickly” throughout his life, beginning with an early bout of rheumatic fever, encouraged his son to believe that he could beat the illness, should it ever manifest.
Not a single person close to my dad has any recollection of him ever comparing his ailment to his uncle’s until late in his illness. However, Michael claims that my dad referred to a connection before he even looked sick—perhaps sometime around 1990 and our family trip to Israel. My dad suggested to Michael that day that he believed he had “what Uncle Nathan had.”
Michael recalls that this conversation took place during a business trip to Columbus while the two were chatting in my father’s home study. He said my father was unwavering in his belief that he could overcome his condition with the right discipline. My father was healthy. He was at least ten years older than his uncle Nathan had been when he got sick. It might be the same thing that killed Uncle Nathan, but for my father, it would be different. And certainly not deadly.
Nine
All the drainage points for my father’s frequent tappings, as well as IV ports, blood draws, and the other standard skin piercings of the chronically ill, were prone to serious infection. At this time in his medical journal, my dad wrote, “There is no doubt something needs to be done. I am deteriorating slowly but surely. I am pretty much a skeleton I am so skinny. I can hardly walk. I am so fatigued. Forget sex.”
Meanwhile, I was doing my best to maintain a semblance of normalcy in my own life. During my freshman year of college, I finally had my first serious boyfriend. Although I could talk endlessly about boys, I had never been boy crazy in practice. I went to prom and had been kissed a handful of times, but I had never really had a serious boyfriend, until Lucas. Lucas
, my freshman-year boyfriend, was the boyfriend I always thought I’d have. We had so much in common it was almost boring. We even looked alike. We had similar black, wavy, chin-length hair and tall lanky bodies. Another couple I had seen around school seemed similarly matched. When I mentioned to someone that I had trouble telling them apart, they replied, “Well, before I met you and Lucas, I thought the same thing about you guys!”
As my father grew progressively sicker, something strange had started to happen to me. My self-confidence, the certainty I had about my understanding of the world at large, was beginning to erode. I had a therapist who named it “launch-pad disintegration.” She said it was a phenomenon that she believed happened to a lot of people in their late teens and early twenties when their parents died or divorced, or family units otherwise fell apart. There you were, just about to launch—when crash! The whole thing fell out from under you. The foregone conclusion that I would marry a doctor and become a doctor (or maybe a lawyer if I couldn’t pass calculus), that I would marry Lucas who looked like me and shared a background, a religion—who was conveniently planning on becoming a doctor . . . well, that plan was beginning to fray around the edges.
I stopped going to classes. I started smoking a pack of cigarettes a day. I asked for extensions on tests and papers, or settled for mediocre grades when I forgot to get those extensions. I started smoking pot at parties. I stopped cleaning my dorm room. I started smoking pot every morning. I stopped imagining a future in which I would be a productive member of society. I joined a band.
That spring I brought Lucas to the Brigham to meet my father. He had just come out of surgery and was bleary from medication. If this had been any other time and any other place, my dad would have looked at both of us and genially exclaimed, “If I could create the perfect man to date my daughter, it would be you, Lucas!”
Instead, my eighteen-year-old boyfriend, looking over the hospital bed of his first girlfriend’s dad for the very first time, got a different response. My father reached up and put a hand gently around the back of Lucas’s neck. He brought his head toward him, and then he kissed his forehead.
Unfortunately, I did not find this moment touching. I was horrified. Lucas admitted later that he was more embarrassed than horrified, but the energy was similar. I’m sure I apologized for my drugged-out dad and ushered Lucas quickly from the room. By the next school year, we had broken up. Lucas almost immediately started dating a beautiful freshman who looked a lot less like him than I did and whom he would later marry. He also became a neurologist. Of course.
Trying to imagine what my life would have been like if I had remained the girl I was when I started college is a futile exercise. I was not that girl. I immediately started dating musicians and boys who dropped acid at Dead shows.
One of the first people I dated after Lucas was a guitarist named Ryan. I was quietly obsessed with him, and our romance lasted about a month. He called me “kookaburra,” which is a bird referred to in a famous Australian folk song. It is not a compliment: “Kookaburra sits in the old gum tree, / Merry merry king of the bush is he. / Laugh, Kookaburra, laugh, Kookaburra, / Gay your life must be!”
I wasn’t gay—in the sense of happy or otherwise. I wasn’t merry. But I was at least borderline crazy. I went to lunch with Ryan one day and was so overcome with nerves that I didn’t speak. He spoke and I made a noise, and he spoke again, more out of necessity than desire. My body shook during the entire, short meal. If I managed to swallow food, it was an accident. Ryan avoided me until that weekend, when I showed up at his band’s show at a campus fraternity. I never went to fraternity parties and would never have gone if he hadn’t been playing. He snuck out before I could find him after the show.
Ryan wasn’t my only kookaburra experience that year. There was the artist at my sister’s school who was on the receiving end of way too many telephone-call hang-ups, popular among casual stalkers in a pre-caller ID world. There was the blond pot dealer who lived next door to my friend Suzy whose doorbell I sometimes inappropriately rang in the middle of the night to the dismay of his roommates, one of whom still won’t accept my friend request on Facebook. And there were others.
I was in an emotional holding pattern, overcome with two primary feelings—guilt and embarrassment. They had become my default emotions. Things that might have made me angry or sad had shifted to my constantly asking myself, What did I do to cause this to happen? I imagine that subconsciously it was a way to regain some kind of control in my life. My father’s illness was taking a toll on all of us. He was exhausted as his body grew less and less able to absorb the nutrients of the food he was eating. That is, the food he didn’t immediately vomit up from the pressure of the fluids encasing his organs.
We had all become so accustomed to the illness that it was our new normal, but its ability to keep us on our toes never failed to surprise us. Once, my mother and I checked my dad out of the hospital for a night to treat him to a nice dinner at one of Boston’s trendier restaurants on Newbury Street. A friend had told me about an Italian-influenced restaurant, Sonsie’s, that served food I knew he’d like. Outside on the sidewalk, my father’s wheelchair got caught on a pothole. Dozens of people walked by us as my mother and I strained to push the chair out of the deep groove. My father was even trying to help, telling us to push left, then right, using his weakened hands to jiggle the wheel. Embarrassment exploded from deep within me. What I should have been feeling was anger. Rage. Outright fury at those people who acted like we were invisible as they forged past us. Not a single person offered to help.
My father was so sick. His tall, once-lean frame was enormously heavy with fatty fluid bloating him with pockets of swollen flesh. What wasn’t fluid was hardened scar tissue. The enormity of my father—and the enormity of our situation—had become overwhelming. Despite having Dr. Kricket on the case, we still had absolutely no answers. And here was my father sitting in a wheelchair that was stuck on Newbury Street in a pothole.
When my mother and I finally managed to leverage the wheel back onto the sidewalk, we rolled the chair into Sonsie’s, shaking off the momentary horror, a recently mastered skill. The restaurant was classy without being stuffy. The food was good and our banter became relaxed. Eating with my father, though, had become something of a complicated ordeal. In the moment when we had finally managed to experience a tiny spell of normalcy, all three of us forgot that we were anything but normal.
It didn’t last long. My father coughed once, and immediately threw his hands up to his mouth. My mother and I leaped from our seats and started grabbing napkins wherever we could find them. My father often threw up after meals. The pressure surrounding his stomach made it hard for him to keep food down. The other diners were trying not to look. Even the waitstaff was trying not to look. But there was a large, very sick-looking man throwing up in their establishment. Someone was going to have to do something.
So they brought us our check.
I apologized as my father stared blankly at the tablecloth. I explained that he was sick. The waitstaff nodded, and I thanked them for nodding. My father’s expression became morose.
On another occasion, at a dentist’s office, his tapping site started to leak fluid. Although the bandage we used was guaranteed to withstand anything, the pressure behind the chyle in his gut soaked through it until the fluid poured freely from his body. We gathered towels to deal with the sticky mess.
I was conflicted. I was embarrassed one minute, ashamed the next. Mostly, like the rest of my family, I simply couldn’t get a handle on what the hell was going on.
“What’s wrong with your dad?” people asked. But we didn’t have answers. We lacked a language or a guidebook, and instead grew accustomed to the frustration and humiliation of the undiagnosed. Years later I heard the gossip among his patients was that he had AIDS.
With guilt and embarrassment as my only collaborators, I was faltering. Everyone in my family agreed when I asked if I could take off the second semeste
r of my junior year.
* * *
In the winter of 1995, my father installed a lift so he could get up and down the stairs of our house in Columbus. He also decided to sell his business, and found a buyer quickly. He’d cut his hours dramatically in the previous months, but he was managing to continue to work. He felt that if he could sell the clinic right away, he could stay on a little longer to keep its value high. Then, when he got better, he would commit to coming back and working there once again.
Dr. Steven Delaveris became the clinic’s new owner, and also my father’s new primary-care physician. Like his previous doctor, Dr. Delaveris was stumped by my father’s condition, but he followed up on one new avenue of treatment. Patients suffering from some lymphatic conditions are fed intravenously. Since my father was deficient in nutrients, it was the next logical step. If he knew about his uncle Nathan’s “chyle smoothies,” he’d have known that the IV recourse was a technological leap forward. A permanent port was placed in his groin where the IV would attach. Twice a day he was fed through an IV drip—introducing nutrients directly into his bloodstream. He continued to eat by mouth when he was hungry.
The port was uncomfortable and easily became infected, but initial test results showed some slight improvements. One day Dr. Delaveris came to our house, and while he was checking on the port, he casually asked my father if he had thought about his end-of-life plans.
The Family Gene Page 6