When I was thirteen years old, I was diagnosed with a genetic syndrome so rare that my general practitioner had heard of it only once and had to read an article from a medical journal out loud to my parents and me to tell us what the symptoms were. Multiple endocrine neoplasia type I — or MEN1, for short — causes tumors on the endocrine glands that often require surgery to remove.
I had my first surgery at fifteen. After an eight-hour drive, I arrived at the Mayo Clinic in Rochester, Minnesota — one of three medical facilities with surgeons who specialized in MEN1. (The other two were in Maryland and Switzerland.) As poor, rural Iowans, my parents didn’t have the money to take us on vacations, so a long car ride to a new place was as thrilling to me as a visit to Disneyland. The fact that my mother was so anxious about the trip made it all the more exciting.
The day I arrived, I was subjected to a battery of tests, including inspection by a bevy of endocrinologists who were fascinated by my rare illness. They examined me the way my grandfather might a prizewinning pig at the state fair. The head doctor discussed my case with the circle of nodding, peering white-coated men, then asked me to lift my shirt and remove my bra so a couple of them could squeeze my nipples and demonstrate that, due to high prolactin levels, MEN1 patients lactate even when not pregnant. Apparently taking my word for it wasn’t an option. My face turned red, and I tried to shrink into the examining table and away from the prodding fingers and staring eyes.
The day of the operation was much less stressful. As they wheeled me into the operating room, I smiled and silently repeated the prayer Father Tim, our priest, had taught me: God, whatever happens, I know it is Your will, and although I hope I wake up, I know if I don’t that I’ll be with You in heaven. I wasn’t scared, either way. This kind of calm faith and acceptance is nearly unbelievable to me now, but then I felt ready for whatever would happen.
In the operating room the focus was less on me as a specimen and more on the tasks at hand. I chatted with the surgical team, as if the occasion of being put into a drug-induced sleep and sliced open were just another opportunity to make new friends. I asked questions about each little wire and tube and needle. The surgeon mentioned that he was also going to remove the lipoma, a type of benign tumor, that was growing just under the skin of my tummy. I nodded as if giving my consent, though he wasn’t asking for it.
It took them four tries to get the needle into my vein to deliver the anesthesia that would put me to sleep, but even then my curiosity kept my spirits high. I had decided I wanted to study medicine in college and become a doctor someday.
When the anesthesiologist told me to count backward from twenty, I eagerly obliged, not getting any farther than sixteen before I blacked out.
When I woke up, I was missing three of my four pea-sized parathyroid glands. The surgeon had assured me he would do everything he could to make the scar as small as possible, but even after the incision had healed, it still looked, in the words of a neighbor, “like someone took a branding iron” to my neck. That neighbor was a retired woman who liked me because I would play Scrabble with her on Saturday afternoons. When she loaned me a large choker necklace to wear to my junior prom to cover up the scar, I was grateful.
The second surgery, when I was a twenty-five-year-old newlywed, was to remove my remaining parathyroid. It seemed impractical to travel all the way back to Rochester, so instead I went to a surgeon at the University Hospitals in Iowa City. He wasn’t a MEN1 expert, but he had experience with thyroid procedures and felt confident he could locate the tissues that were growing uncontrollably and remove them. When I asked why he thought I needed the second surgery ten years after the first, he suggested that the one gland they’d left behind might have grown in size and become a megaproducer, or maybe I had one or more hidden parathyroid glands. These things happen. Some people have six instead of four. The surgeon referred to himself as “we” throughout the discussion, as in: “All we know is we need to get in there and debulk the parathyroid tissue or your kidneys will calcify, slowly filling up like bags of gravel.” I imagined my petrified kidneys, no longer able to filter the blood and excrete the impurities, and the inevitable result: an excruciating death by kidney failure.
I had health insurance through my husband’s plan. Even so, I was told to expect a bill for thousands of dollars because the surgeon was not part of the insurer’s network.
My anesthesiologist had a charming Indian accent. He and the nurses all tried several times to insert the needle for the IV, but my vein was not cooperating. Per my doctor’s orders, I hadn’t had anything to drink since midnight, and the anesthesiologist explained that when a patient is dehydrated, blood vessels shrink, turning a simple needle poke into a game of hide-and-seek with the vein. I wasn’t worried. I loved listening to him talk.
Finally he told me that they would have to use a different method to put me to sleep: a gas mask over my face. He instructed me to think of a happy place. I picked a beach and began imagining the sound of waves. “Good, good,” he said as the mask went on. The taste was unpleasant. I felt myself drifting on the imaginary water, and then sinking, and then fighting the waves, my heart racing. I tried to lift an arm but could manage only to squeeze the nurse’s hand.
“Remember your happy place,” the anesthesiologist told me. “You are going to your happy place now.”
I wanted to scream, No, I’m not ready! I made eye contact with the nurse holding my hand and tried to tell her with my eyes that this couldn’t be right. Then came the blackness.
A few months after that, another doctor told me I had twelve tumors attached to my pancreas, and they would have to be removed. He showed me MRI scans, charts and graphs, percentages and statistics, as if making a sales presentation. He said I should be sure to call the insurance company before the procedure and get approval. The surgery was scheduled for after the new year, so I’d have to pay my deductible again, though I’d just paid it a few months before. In all, the operation might cost $100,000. My husband frowned at this. The doctor said we should expect at least a week of postsurgical care in the hospital. My insurance covered three days, tops.
“Are you sure we have to do this?” I asked the surgeon.
Irritated, he said, “Those tumors could spread. They might be malignant. Do you want to die?”
No, I didn’t. I was only twenty-five. I’d been married less than a year. And I knew the odds: 50 percent of MEN1 patients eventually die from cancerous tumors in the pancreas.
The surgeon told me the risks of the surgery. This would not be an easy-in, easy-out procedure like the ones to remove my parathyroid glands. (Those had been easy?) He was going to slice my abdomen open from one side to the other, cutting through all my abdominal muscles. I wouldn’t be able to sit up on my own until the muscles repaired themselves. I’d need to grab a special pulley overhead to lift myself into a sitting position.
The list of dangers was long: I could bleed out during the surgery. I could hemorrhage afterward, and they might not detect it. Because it was such a lengthy procedure, the chance of my not waking up from the anesthesia was greater. And the longer you are under, the more difficult it is to regain bodily functions even after you do wake up. My intestines, my stomach — really my whole digestive system — would have to be carefully monitored for warning signs.
“Sign here and here and here,” said the nurse. “Do you know what a DNR is?”
I did: Do not resuscitate. I thought of the George Carlin routine about how the secret of life is not dying. “Don’t be pulling any plugs on me,” he says. “Leave my plugs alone. Get an extension cord for my plug.”
I placed a check mark next to the box on the insurance form that said “pre-existing condition.” I placed a check mark next to “nonsmoker.” I placed a check mark next to dog owner, homeowner, married, employed, college educated, drinks socially, and has no savings or family members with money to turn to for help. I placed a check mark next to I want to live to be twenty-six.
When I woke up from the surgery, my torso had been stapled back together along a line shaped, appropriately, like a giant check mark, which later turned into a scar in the shape of a check mark that still stretches from my left flank to my right. The days passed in a nauseating haze of pain and morphine. I was told they’d had to take out my spleen, too: the spleen is a sensitive organ; any nick or bump might cause it to bleed, and once the spleen starts to bleed, it just doesn’t stop. So they’d removed it. I tried to remember what a spleen does.
Other surgeries followed for more than a decade. My husband joked that we should simply apply for a body transplant: swap my defective body for a new one, but keep my brain. Well, the tumor attached to my pituitary gland, at the base of my brain, would have to go, too. The pituitary is the master of the endocrine system and controls all the other glands in your body, including your reproductive glands. Right now my pituitary is telling my body that I’m pregnant. I’m not, but my breasts are lactating and my abdomen is swollen and I’ve put on so much weight I don’t recognize myself anymore.
I no longer find my body to be a point of interest. I am no longer inspired by medicine and technology. When I have questions, they usually revolve around one imperative: not dying. Do what you have to do, I say. Just leave my plug alone.
My last surgery was a kidney operation. Afterward I was lying in the hospital with the strange sensation that my body was swelling up. I complained to the nurses, who said I just needed to relax and ignore those feelings: What? You haven’t peed in twelve hours? Don’t worry about it. You’ll pee eventually.
When no one would pay attention to my concerns, I called my friend Michelle, who has a PhD in nursing. After listening to me describe my symptoms, she told me to walk to the nurses’ station and say that either my bladder was about to burst or I was having massive internal bleeding; either way, I would be dead if they didn’t do a bladder ultrasound — now. She saved my life.
Twenty minutes later two young doctors, both residents training to be kidney specialists, were working hard on my naked lower half, inserting a tube into my urethra to suction out a blood clot that had jammed up the waterworks, causing the trouble in my bladder.
“I’m breaking up with you,” I said. “Both of you.”
Dr. Steinberg, whom I’d gotten to know, laughed loudly. The other resident chortled in surprise.
“Don’t get me wrong,” I continued. “I can’t imagine any other place I’d rather be on a Saturday night than right here with the two of you. I just feel like we’ve been spending too much time together. You need to see other bladders.”
I’ve learned that joking with the medical staff puts them at ease and helps me connect with these people who are seeing me at my most vulnerable. Plus, if nurses or doctors like you, they share more information, and you get a better understanding of your condition. Also I can’t help but think that if they have to save my life one day, they might try a little harder if they see me as a likable person and not just another procedure: Don’t give up yet — she said the funniest thing today!
This connection that I try to make with the people who hold my life in their hands is double-edged. Sometimes it lulls me into a false sense of security. I believe in them too much and ignore my self-protective instincts, as I had just before that kidney surgery, when I’d been getting prepped:
“You want me to do what?” I asked the anesthesiology team. I thought maybe they were joking or I just didn’t understand what they were asking me to do. They wanted to insert a breathing tube all the way down my trachea while I was awake. I’d never had anyone ask me to agree to this before.
“Can’t you do that after I’m knocked out?” I asked.
“We need you upright to insert the tube,” the lead anesthesiologist told me. After they had it in, I could settle into a comfortable position on my stomach.
I told him I was reluctant to go through with it.
“This is how we have to do this,” he said.
I liked him. He and I had been bantering earlier when they’d given me the Ativan I’d asked for to calm my anxiety. He had kind brown eyes. He was careful with his needle pokes. I wanted to show him that I could be brave. If they were asking me to do this, it couldn’t be that bad. Right?
I looked over at my friend Michelle, who was there to provide support. She would stand up to doctors if she thought they were making a mistake. I relied on her to do that. I asked her with my eyes if this seemed like a normal request to make of a human being. She looked worried but didn’t say anything.
I agreed to be awake while they inserted the tube.
The operating room was cold and seemed like a small space for six people to work in — seven once my surgeon joined the party. It was also crammed with equipment, as if they were squeezing my kidney operation into an old storage space. It’ll be fine. What’s the worst that could happen?
I wore a faded white-and-yellow hospital gown that covered only my front and tied behind my neck. The metal snaps were like ice against my skin. I had blue surgical booties over my bare feet, just like the nurses and anesthesiologists. Why did I need booties? Everyone but me would walk out of there. Everyone but me would sleep at home that night.
As they pumped anti-anxiety medication through my IV, I tried not to wonder about the percentage of people who suffocated during this procedure, whether it was a low or a high number. Soon I felt as if I’d drunk a twelve-pack of beer and might tip over at any moment. I was so relaxed I had to remember to breathe while they began to insert the tube. Wait, I thought. That’s not the problem. The problem is I can’t breathe with this plastic hose being shoved down my windpipe. That’s when my adrenaline overrode the drugs. I grabbed someone’s arm and tried to get off the table. Hands held me from all sides. I was in a half-kneeling position, one leg bent under me and the other foot flat on the table, while the nurses fought to keep me from yanking the tube from my throat.
I heard the lead anesthesiologist shout, “Give her the Propofol. Now!”
No gradual fade-out this time. I was catapulted into the void. Someone must have caught me as I fell from the table.
The operation took twelve hours, and it probably took some medical-coding specialist another half-hour to document everything they did and every drug they gave me, placing a dollar figure next to each check-marked box.
Why did I agree to be awake while they ran that tube down my throat and into my lungs? Because I’d been trying so hard to prove to them that I was cool, the kind of patient they would like, the kind who would cooperate. I wanted them to know they could ask me to do anything.