When Aaron and I came to see Val during those first harrowing days, the waiting room outside the ICU was packed with people from her church. When it was our turn to go back and see her, we went with Aunt Joanie.
“Her mother isn’t alive to be here,” Aunt Joanie told us, having come to the hospital almost every day since the stroke. “I have to be here for her mother.”
We stood over Valerie, a cousin I only vaguely knew. Would she even recognize me if she could open her eyes? Her head was bandaged and her face was bruised. A tube in her mouth was breathing for her. I’d never seen her without lipstick.
Aunt Joanie rubbed her hands and told her she loved her. I kissed her cheek. Even Aaron, who’d never met her, touched her shoulder gently.
* * *
No one in the family, as far as we knew, had been stricken with a gene-correlated illness since Uncle Norman died in 2002. Everyone was living in the same kind of oblivious lull they had enjoyed during the thirty years between Uncle Nathan’s death and my father’s.
My cousin Valerie is my first cousin once removed. That means she is the first cousin of my father. Val and I are first cousins, separated by one generation. Valerie’s sons, Jordan and Storm, are my second cousins. Dr. Kricket usually refers to Val as my aunt because she is of my parents’ generation, although she is not a sibling of my parents. Val’s mother was my great-aunt, and all the descendants of my great-aunt are my cousins, regardless of how our generations match up.
Valerie was more serious than her younger sister, Suzanne, and her big brother, Ken. Always put together in beautiful leather jackets and with impeccable makeup, Val was cool. She had countless friends, and her husband adored her. Her older son, Jordan, had an amazing artistic ability that his grandmother, Aunt Norma, had been immensely proud of. He grew up to become a jewelry designer and international gem trader. Her younger son, Storm, stayed closer to home and became a leading figure in her church. Valerie had two great kids, a steady marriage, and a religion she loved. She also had her mother’s bad gene.
She hadn’t been experiencing any problems because of it, at least to the best of our knowledge. Val’s health issues were vastly different from what anyone else in the family had gone through, so there was very little reason to try to correlate them. At one point, as I’d overheard at Aunt Norma’s funeral, Valerie had undergone a splenectomy to remove an enormous and grossly spongy spleen from her abdomen. No one else in the family, as far as I knew, had suffered from the same problem. But Val and the rest of us did share one important thing: no one knew what the hell was going on. And another thing: her platelet levels had once been very very low.
Why were they low? Why had Val’s healthy spleen been engorged? Why was an otherwise healthy woman in her early forties having to undergo such a catastrophic procedure? And why in her late fifties was she now lying in an ICU on a ventilator?
Perhaps we had all grown so accustomed to hearing “I don’t know” in lieu of answers that their absence actually seemed normal. But even Aunt Norma, a practicing nurse for forty years who lived through her daughter’s first invasive procedure, never compared Valerie’s “I don’t knows” with her own.
As a woman with the family gene, it seemed like she would have an edge, at least compared to the men. Her mother lived into her seventies. My grandmother, Val’s aunt, had made it into her eighties. Any whispers about my great-grandmother, Val’s grandmother, dead by fifty-four, were disregarded. Shh, I told the voice. That was the 1950s. And anyway, Valerie had a stroke. How was a stroke related to lymphedema or massive ascites? It wasn’t. Valerie was exhibiting no swelling; she and I didn’t even share ankle problems. Meanwhile, Grandma Mae hadn’t had a stroke. Aunt Norma hadn’t had a stroke. Uncle Norman, my dad, Uncle Nathan . . . none of them had had a stroke.
Meanwhile, Ken, Valerie’s brother, Aunt Norma’s oldest child and only son, was told definitively that he didn’t have the murmur, although he remained skeptical for years, having watched his cousin Norman receive the same vote of confidence before falling desperately ill. Ken’s two daughters didn’t have the murmur either, which seemed to squarely put him in the clear. Like my own father, Ken’s girls would have inevitably inherited any gene on his X chromosome. If he’d had the gene, so would they. Suzanne, Aunt Norma’s youngest, conversely, had the murmur and therefore the gene like her sister, Val. But she had no health problems to speak of.
When Valerie’s two sons and Suzanne’s daughter and son were tested, neither mother recalls ever asking for or hearing their results. It might have felt easier to carry on not knowing, and Dr. Kricket made it her business never to offer information to families without prompting. She knew if any of us was going to get sick, we’d get sick. But in the meantime, living confident, healthy lives was more important than miring us in a fear that could easily prove illusory.
So none of Valerie or Suzanne’s kids knew if they had the gene, but at the time, knowing really would have mattered only to one of them: Suzanne’s daughter, Denise, who was about to start a family. Though a year younger than me, Denise married when she was twenty-two and gave birth to her two children before she knew the risk that passing our gene posed to them. At the time when Val was convalescing from her stroke, Denise’s children had still never been tested.
Valerie stayed in the ICU for two full months before she was transferred to a rehab facility. Her husband, sister, and sons remained vigilantly at her side. Val had so many regular visitors that the hospital staff began trying to figure out who she was. With all that traffic, she had to be pretty important.
Valerie’s recovery was touch and go. There were setbacks and leaps forward and then stumbles throughout. Her husband, Michael, would say, “Valerie, if you can hear me, squeeze my hand.” And she would. A little while later he’d say, “Lift up your knee.” And she’d do it. But there was no facial reaction. No recognition. No Valerie.
Until, one day, there was. Slowly but surely, Valerie learned how to talk again, and walk again. She grew stronger and stronger until her doctors became confident that she might even enjoy a quality of life similar to the one she had known before the stroke. Michael credits the constant stimulation from friends, family, and church members for helping his wife ultimately regain much of her physical and mental function.
* * *
While Aaron and I were driving away from the Hackensack University Hospital ICU and a motionless Valerie days after her stroke, I leaned my head on the window of our car. It felt good to be out of the hospital. It felt good to not have to go back.
“Do you think . . .” I started to say what I was thinking to Aaron. Was there a correlation between Val’s stroke and my gene? Then Nah, I probably decided, swatting the idea away. No way.
I watched as the Bergen County landscape melted into New York City slurry, and then firmed up again.
Twenty-Five
A year and one rescue dog later, Aaron proposed to me and gave me his grandmother’s ring. He’d had it resized and fitted with my birthstone, a garnet, in the one setting where a diamond chip had fallen out.
I called my mother to tell her about our engagement. Her first impulse, even before congratulating me, was to suggest that I always leave my engagement ring on, even when I slept. I am prone to losing things. I am also prone to dropping them: in the bathroom especially, you can count on me to flick earrings, fumble lipstick, and certainly mishandle engagement rings. I have more than once referred to myself as “floor-blind,” and almost always rely on Aaron to find anything I have dropped, or give up hope. I took my mom’s advice and slept in my ring.
I was thirty-six years old that morning in early spring of 2010 when I woke up to my left ring finger puffed up from my bottom knuckle to about four inches above my wrist. I didn’t know that day that I was only two years older than my great-grandmother Mae had been on the morning she woke up to swelling in her arm.
The choke hold my engagement ring had on that finger that morning was alarming, to say the least. Our
dog zipped under the bed to cower. Aaron was immediately at my side. “Oh, my poor baby,” he said calmly. He clicked his tongue and comforted me with well-timed “aw”s as he guided me from the bed to the bathroom. He left me momentarily clutching my hand under cold water until he returned with the bottle of Dawn from the kitchen sink. He poured most of the bottle onto my hand. As Aaron gently smoothed the soap-smothered ring over the firm gel of that chyle-filled knuckle, it finally slipped off.
My hand, while not wholly remarkable in appearance, now boasted a permanent oblong-shaped bubble beneath my ring finger. It relaxed into a pitted oval when pressed, then slowly re-formed as the fluid settled back into place. We stood there staring from it to each other as the whir of the fan motor lent a sound track to our Sunday morning.
“We’ll call Dr. Seidman,” Aaron intoned in his deep, soothing voice.
I leaned into him as he hugged me. The dog scampered out from where she was hiding under the bed to include herself in our embrace.
* * *
We didn’t get around to calling Dr. Kricket because a few days after that harrowing morning, my mother fell and broke her hip. I flew to Columbus. When I landed, I realized something immediately perplexing: my foot was trapped in my shoe.
As soon as I was alone inside my mother’s house, I rolled up my pant leg as far as I could. Mind you, these were not skinny jeans. The skin of my calf was pulled taut so that it had taken on the look of a loaf of seeded rye bread with flecks of dark stubble that had nowhere to hide. The foot itself, sockless, was puddling over the rim of my shoe. The skin itched a little bit, but other than the discomfort of the weight itself, there was no pain.
My ankles had been swollen for the last ten years. But this swelling in my leg was new and, on the heels of my swollen hand, alarming. Even stranger, the swelling in the ankle of my left foot had diminished substantially. In fact, that ankle, once the more swollen of the two, suddenly looked normal.
I was surprisingly unfazed. Some people would have driven straight to the nearest Urgent Care. I drove myself to a nearby Walmart to buy a replacement pair of shoes and find a pair of scissors to excavate my foot. Luckily, Walmart had scissors, shoes, and a pants aisle. I cut from the hem of my jeans leg and tore the fabric up to the knee, where the swelling began. I worked carefully, sawing at the leather, finally leveraging the scissors beneath my heel. The red Danskin clog slid off. I replaced it with an unlaced yellow work boot in a size bigger than what I usually wore. While fashion wasn’t at the top of my must-have list just then, I felt like the shoes worked as a nineties throwback, even if I tripped a little over them when I tried to walk.
I changed into a pair of wide-leg black yoga pants and took my pile to the front of the store to pay.
“I’m wearing it all out.” I shrugged to the salesgirl, handing over a shoe box with a pair of clogs inside—one mangled—tags from my new yoga pants, and an empty scissors wrapper. I caught her judgmental side eye, but opted to ignore it.
I didn’t go to the doctor. I didn’t even call Dr. Kricket right away. My now-swollen leg and hand felt like an extension of my swollen ankles, which I had already endured for five years. I knew that women in my family had swelling in their limbs, so it didn’t scare me so much as disappoint me. My swollen leg suddenly meant I couldn’t wear skirts, and almost all of my shoes, barring my new extra-large work boots, were out of the question on my right foot. No part of me worried that it was anything more than cosmetic. Anyway, if I was about to be ravaged by lymphedema, then it was probably better to deal with my mother first.
Later that night, I showed my leg to my sister. She and I didn’t say much on the subject.
“Does it hurt?” she asked.
“No,” I answered.
“Grandma’s legs look like that,” she added.
“I should get some of those fitted stockings she wears,” I answered. She agreed. And that was all we really said.
A few weeks later, a college friend came to town, prompting a group of us to meet up in midtown Manhattan for pizza. It was a hot spring night and we drank several pitchers of beer. As I made my way home on the subway, I could feel my leg pulsing against the fabric of my jeans. Already relegated to wide-legged fashion, and just barely figuring out what shoes my foot would fit into, I worried that an expansion of the condition of my leg might push me over the edge in several ways. I called Dr. Kricket and made immediate plans to visit.
Twenty-Six
On June 15, 2011, I visited the Seidman lab to present my newly enormously swollen right leg. Dr. Kricket’s very first question was pretty basic: “So, do you have a doctor yet?” The answer was the same as it had been since college. “Aren’t you my doctor?”
Dr. Kricket is a researcher, not a diagnostician. While she was more than happy to weigh in on my medical predicament, she simply wasn’t in a position to serve as the leader of my health and wellness team. I told her that Aaron’s boss understood that I would go on Aaron’s insurance as soon as the ink on our marriage license was dry. But right then, she was all I had. My swollen leg. My swollen hand. She was all I had.
She ordered an MRI under the umbrella of her study. As I lay still inside the narrow tube, hearing the beeping, clicks, and squeals of a machine that, incidentally, I still can’t believe is the best we have to offer in a post-man-landing-on-the-moon world, I truly believed that Dr. Kricket wouldn’t be able to tell me much.
Joselin, we don’t know what caused your leg to swell. We don’t know why you will need to learn phrases like “removable orthotics” when you buy footwear so that you can add an inch of depth to your shoes by removing the insert. We don’t know why you can’t wear your engagement ring anymore. Shrug. You have a bad gene.
After the MRI, I sat alone in a deserted waiting room. When Dr. Kricket came in, we were the only two bodies within that quiet space. Rows of empty chairs seemed to hold the apparitions of the people who had previously filled them.
Dr. Kricket sat next to me, and then she did something surprising. She took my hand.
“I need you to get a doctor,” she said, leaning in, her blue eyes more intense than usual.
“Okay,” I answered, bubbly, completely unwavering in my expectation. “Next month.”
“Meanwhile, dear,” she went on, “I need you to be careful you don’t throw up.”
I was about to laugh when suddenly it occurred to me that she wasn’t making a joke. She was telling me something. Something about my health, and it wasn’t the status quo.
“You have a blocked portal vein in your liver. Now, you seem fine and I’m not worried”—her sentences were quick and staccato—“but your spleen is big, which explains your low platelet count.”
In the recesses of my mind, I thought about someone else who had a blocked vein and a large spleen. “Mushy,” did they call it?
“Like Valerie?” I asked.
“Yes, exactly like Valerie.”
“Was her portal vein blocked?” I asked.
Dr. Kricket nodded. “But there’s a treatment,” she added. “A procedure to reopen it. Val had it and it worked.”
My dad’s portal vein had been blocked too. His treatment had failed.
Why hadn’t I known about Val’s portal vein? Wait. Had I known? I was suffering from momentary amnesia.
“Now, I want to tell you again, you do not have what your father had, and I know that you are okay, just like your grandmother.”
“But I shouldn’t throw up?” I tacked on at the end. Why? Why shouldn’t I throw up?
“Right. No throwing up.”
“And why does this explain my platelet count?” There were too many questions and it felt like I was asking none of the right ones.
“Your large spleen sucks up the platelets,” she was saying, but the room had grown a bit echoey and I wasn’t sure I could process what she was saying. First of all, how the hell do you not throw up if you have to throw up? Second, wait, no, first of all . . . Why? Why on earth should I not thr
ow up? I do not enjoy throwing up. I do not ever want to throw up. But that isn’t to say that at various points in my life I haven’t, against my will, thrown up.
I led with this concern. “How do you not throw up?”
“Have your doctor prescribe you this . . .” and she wrote down the name of the antinausea pill.
If Dr. Kricket specifically answered my question about why I couldn’t vomit, I can’t remember what she said. Similarly, the note about my platelets getting sucked up into my enlarged spleen also got sucked out of my brain by something else only minutes later.
My friend Molly picked me up from the Brigham, where I’d met with Dr. Kricket. Molly is a nurse. She was Aaron’s best friend’s girlfriend, and when I met her, we had fallen in deep, unabashed friend-love. Having gotten ordained online, Molly had become the go-to wedding officiant among her friends. She was going to officiate at our wedding next month. Molly had the kind of temperament that was also perfect for a nurse, even if she had a tongue piercing, ear gauges, and tattoos all over her arms and back. She was gentle but decisive, confident but empathetic. She had been working for a year at one of New York City’s largest burn units. When I told her I had to come to Boston to see Dr. Kricket, she told me she’d drive me. She had spent the afternoon with friends, but was waiting to pick me up when I emerged from the hospital at the end of the day.
About ten minutes later, I explained that there was something about a blocked portal vein in my liver and an order not to throw up.
“Why is that?” I asked her pointedly. “Why did Dr. Kricket tell me not to throw up?”
Molly directed a brief side eye my way, then squared up her shoulders, both hands on the steering wheel. “You know,” she began, “I’ve only really heard of it in old alcoholic homeless people.”
The Family Gene Page 15