The Family Gene

by Joselin Linder

  It was a question he asked all of his patients. He wasn’t suggesting anything more than that everyone should have a plan.

  My father didn’t hear it like that. He heard Dr. Delaveris giving up on him. To look at my father, it would have been hard not to consider giving up. But my dad was going down fighting and anyone who didn’t understand that was, as far as he was concerned, deadweight. He relieved Dr. Delaveris of responsibilities for his care immediately. My dad wanted to live and he needed the rest of us to stay very clear about that.

  * * *

  On February 10, 1996, my father had been forty-eight years old for nine days. I was living at home during what would have been the second half of my junior year of college. I had already been a biology major, a history major, and a psych major. I had done all the legwork, changing advisers and registering with each of the different departments. I guess changing majors was a bit like having control. Now, during a break from school, I was majoring in marijuana studies with a minor in avoiding my parents.

  Meanwhile Dr. Larry Lynn, a pulmonologist in Columbus who just might have been Ohio’s answer to television’s Dr. House, found my father. Dr. Lynn worked at Doctors Hospital, where my father was both on staff and had been spending an inordinate amount of time as a patient over the last few years. Dr. Lynn didn’t know my father, but he liked medical mysteries. When I spoke to him recently he told me that some weekends he’d troll the hospital looking for cases that had a lot of holes in them. That’s what happened the day he found my dad. When I asked him how many of his mystery cases he had managed to solve before my father’s, he sounded surprised that I’d even had to ask. “All of them,” he answered.

  Dr. Kricket’s involvement in my father’s case had been relegated to procedures that could only be accomplished in the lab. My father’s diagnostics, so to speak, were not within her jurisdiction. What we needed was a guy who trolled hospitals looking for mysteries. So it was awfully convenient when that guy found us.

  Initially, Dr. Lynn had coordinated with Dr. Delaveris to get my father on a liquid-diet regimen. Since Dr. Delaveris had been dismissed, Dr. Lynn took over my father’s primary care. He had an idea. In addition to the IV nutrients, Dr. Lynn wanted to remove all the fat from my father’s diet to see if he could elevate his albumin levels. Most of the protein my father was eating was leaking into his body cavity in the form of chylous fluid.

  Some lymphatic conditions have been successfully managed by cutting fats completely. Fat thickens lymphatic fluid, so that the structures of the lymph system “drop it” more readily into the body cavity instead of channeling it into the bloodstream. A low-fat diet—or a diet free of triglycerides—would also prevent chyle from forming. If you are coughing up chyle, or suffocating in it, a fat-free diet can keep your body from forming that sticky milky lymph in the first place, or at least from making so much of it. Fluid will still be leaking, but it will be much thinner, more like the liquid you see when you pop a blister. It’s largely more comfortable, and fundamentally easier to manage. Our main hope was that my dad’s body would have an easier time channeling the protein-rich fluid into his bloodstream. A few years earlier, my father thought my mother’s ice cream habit might kill her. Now it looked like foods like ice cream could kill him.

  He was devastated by Dr. Lynn’s fat-free diet plan, but motivated to try anything. Very soon his diet consisted of several fat-, sodium-, and taste-free soups for breakfast, lunch, and dinner, and fat-free dressings on raw and boiled vegetables; it wasn’t tasty but his albumin levels were shooting up. That wasn’t the only trick that Dr. Lynn had up his sleeve. Recently, things had been looking pretty grim for my father. Now his color was better. He was breathing easier, and Dr. Lynn was leading a charge in a whole different direction.

  He actually introduced us to a fairly new technology, something he was calling “the Internet.” By 1996, e-mail was slowly becoming a part of the mainstream. Everyone at my university had access to it on old boxy computers in the library with a flashing horizontal cursor. But at the time, the Internet was still slow, typically loading pages for minutes at a time, and hadn’t quite spread into daily life. I remember overhearing someone suggesting that one day we would all be ordering everything on our computer, and finding the notion truly confounding. Why would anyone look online for an outfit when it took two minutes for a shirt just to appear on your screen?

  Dr. Lynn used the Internet on his mission to research the origins of my father’s illness. He was determined to find a family that had it.

  And then he found them. They were in Turkey. The family was a small group of Turkish Jews who had a condition that was an offshoot of a rare disease called Behçet’s syndrome or Behçet’s disease. Typically, Behçet’s patients have sores all over their bodies. But this family had lymphedema! Both diseases were caused by inflammation in the blood vessels. The best part of all of this was that there was a treatment for Behçet’s, and it was simple. And it worked.

  Steroids.

  I sometimes think of this period as one of those times that I wish could stand alone without anything coming before it or after it. On the day of the Behçet’s diagnosis, it was springtime 1996, and the world was literally and emotionally on the thaw. We hugged and kissed each other and hugged and kissed Dr. Lynn. I drove to the park and sat by myself on a perfect spring day and wrote in my journal, euphoric.

  “Today is the day we prayed would come and it finally has!” I scrawled across the page.

  A sudden jolt in the universe had just set everything right.

  Nearly ecstatic, my dad’s best friend, Jimmy, went to the library and immediately looked up Behçet’s disease. Hilary; Jimmy’s wife, Jackie; and my mom started talking about throwing a party. Someone tossed out the possibility of combining Hilary’s upcoming college graduation party with a celebration of my father’s health. We began suggesting dates to take our abandoned family trip to Key West. My dad, although feeling a little better on his fat-free diet, finally got to eat some real fat.

  Dr. Lynn started him on steroids immediately. He told us that the Behçet’s diagnosis would be validated within the week based on how my father’s body reacted to the steroids.

  The next day my mother called me. She was hysterical. “You have to get to the hospital!” she shouted. “It’s your father. He wants a DNR.”

  A DNR is a directive to hospital staff that stands for “Do Not Resuscitate” should anything go wrong. It was one of many ICU terms I had grown uncomfortably familiar with. My mother was overwhelmed. She was in the parking lot crying, getting ready to leave. So I left work and drove to the hospital. My father was staring at the wall in his ICU room.

  “Daddy?” I said.

  He looked at me. His face was drawn and dark.

  “I heard you asked for a DNR.” This wasn’t like him. My dad would practically kick people out of the room for asking if he was finished reading a magazine, much less suggesting a DNR.

  I sat on his bed and took his hand. “Do you remember that you just started taking steroids?” I asked gently.

  He nodded.

  “Do you remember that Dr. Lynn said they might make you depressed?”

  He nodded again.

  “Why don’t you get forget about the DNR just until we see what happens with the steroids.”

  His eyes filled with tears. “You think I should wait?” he asked cautiously.

  “Yes, Dad. I think you should wait. Let’s just wait.”

  He was crying now and I was hugging him. “We just got this great news,” I went on. “We can be happy for a little while!”

  “We can?” he asked.

  “Yes,” I answered.

  I was so sure. I was so happy; even as he cried, I was so so happy.

  We canceled the DNR.

  That night, by pure coincidence, my father would have died when his heart stopped, forcing him into cardiac arrest. But he was saved because we had canceled the DNR.

  Two days later, Dr. Lynn told
us the steroids weren’t working. He’d been wrong. My dad would have to go back on the fat-free diet. We still didn’t know what he was ill with, but we now knew that it wasn’t Behçet’s.

  * * *

  That summer, my ex-boyfriend Ryan’s band was playing a show in Columbus. My obsession had passed and I decided to go simply to get out of the house. After the show, Ryan didn’t hide from me, and we went for a drive. I told him what had been going on with my family during our brief but intense few weeks of dating several months earlier. Ryan listened compassionately. He’d had no idea. It solidified a new, nonromantic friendship we’ve continued to this day. That night, Ryan came over and met my father. They talked for a while. My dad was a musician himself. He admired Ryan’s ambition.

  As I was driving Ryan back to the hotel where he and his band were staying, he turned to me and said, smiling, “I feel like I understand now why you went kookaburra.”

  I laughed. And then I shrugged. Because . . . well, yeah.

  Ten

  Since the Behçet’s syndrome diagnosis had been proven wrong, no new ideas had been forthcoming. The Behçet’s expert Dr. Lynn reached out to wasn’t interested in exploring possible links between his expertise and what my father was suffering from. It wasn’t certain that the two could, in fact, be linked, but the expert’s complete lack of interest was disheartening. My father’s indefatigable hopefulness was starting to show signs of fatigue. With each new roadblock, we were driven back to square one.

  One day I arrived at the hospital to find my father sitting up, a glassy look in his eyes. When I entered the room, he looked at me, and for a moment, I wasn’t sure he knew who I was. Then his features relaxed into a smile and a look of relief crossed his face.

  “Jos, I had this dream . . .” He looked down and whispered to himself, “Was it a dream?” Then he looked back at me and went on: “You were a baby and I dropped you on your head. And you lost the ability to say no.”

  My dad had a lot of vivid dreams in those days, which he’d recount, but that one resonated because his lack of certainty that the dream hadn’t actually been real was jarring. That, and the fact that I was getting really good at never saying no.

  * * *

  While Dr. Kricket searched for a genetic link, my dad was getting sicker by the day. What was more, he despised the idea that he would have to spend what was left of his life eating Dr. Lynn’s diet of tasteless 100 percent fat-free foods. Dr. Lynn tried reaching out to a company he had read about that was developing a food additive called “olestra” that rendered fats impossible to digest. He wanted a compassionate release of the product for my dad before it had FDA approval.

  Still, as my father waited for the magic additive, wheelchair bound, physically disfigured, and deeply unwell, there was little that he felt excited about. Each new medical puzzle was beginning to feel like another nail in his impending coffin.

  In the summer of 1996, a team of specialists Dr. Lynn had contacted at the University of Arizona—coincidentally my father’s alma mater—invited my father to their office. Dr. Charles Witte and his wife, Dr. Marlys Witte, were the go-to team in the United States for anyone who found themselves with lymphatic or thoracic-duct anomalies. According to a U of A tribute following Dr. Witte’s death in 2003, he was responsible for designing and helping to simplify “methods to evaluate and treat congenital and obstructive lymphedema.”

  Despite the fat-free diet and biweekly tappings, the fluid reaccumulated in my dad’s body more aggressively than ever. So my parents took off and set up camp in Arizona for a monthlong stay during the early days of summer.

  My mother remembers daily walks to the hospital from her hotel in the sweltering Arizona heat. She was alone again, as she had been for much of my father’s illness, spending her days waiting for him to come out of surgeries and procedures. She ate alone and slept alone. Now, because of the heat, she was mostly stuck indoors. She helped the hospital staff with bathing and caring for my father. And after that, she watched a lot of TV.

  Few people in the 1990s, and frankly even today, know much about the lymphatic system. Dr. Witte told me that she and her husband initially started studying it for that very reason. Their research in the 1960s showed that liver conditions like cirrhosis seemed to produce a lot of associative lymphatic fluid, more than what the body could properly circulate. No one knew much else about what happened when the lymphatic system goes awry. The Wittes’ main objective was to develop or seek out treatments for the buildup of lymph, which is exactly what my father needed.

  My dad’s chart showed that one of the first surgeries he’d undergone since his illness in 1994 had been to counter something called a “total cavernous transformation of the portal vein,” or a TIPS procedure. My father’s portal vein, a main pathway through the liver, was blocked, or more specifically, it had shrunk. Doctors used a shunt, a small piece of metal mesh, to try to prop the vein open, but the portal vein didn’t stay open. No one understood why.

  When the Wittes met my dad, they knew about the problem with his portal vein and that something was keeping his lymphatic fluid from staying inside his lymphatic system. My father’s liver was otherwise healthy, though “healthy” as a term no longer applied to my dad. Everything was growing steadily less healthy because he was starving, as every day vital nutrients leaked out in fluid that never made it to his cells.

  The lymphatic vessels are difficult to see. They are thin and colorless, unlike the colorful and more substantial veins and arteries. My father underwent a simple test called a “lymphoscintigram,” in which doctors inject a radioactive element and a bit of blue dye between the toes. The strong pressures in the lymphatic channels should immediately pull the dye up and through all the vessels of the system. A camera records the process. In a healthy system, it should take only about twenty minutes for the dye to run clear through the body. If there are any leaks, however, the camera can pinpoint exactly where some of the fluid exits the system. It can even trace where the fluid goes: if it lingers in various tissues, or pools in the ankles.

  My father’s test didn’t get very far. It lasted less than a second. His lymphatic system was so weak that nothing moved much beyond his ankles and most of the fluid just settled near the injection site in his foot. The vessels in our bodies rely on pressure. When that goes, everything else goes too. There just wasn’t enough pressure in my father’s vessels to channel much of anything.

  The Drs. Witte were at a loss.

  Every test they administered came back inconclusive. Dr. Charles Witte’s opinion lined up with earlier opinions my father had already heard. Dr. Witte believed that tapping the fluid would grow increasingly difficult as scar tissue built up and trapped it. Soon tapping would become both impossible and “debilitating.” To manage this condition, Dr. Witte thought there were two options. The first was to reduce lymph formation, and the second was to accelerate lymph return.

  There was a small team of doctors in Italy at the time testing a new procedure to cut and attach a lymphatic channel directly to a vein. Lymphatics are heavily “fenestrated,” meaning they contain a lot of openings. This is why it’s so dangerous for cancer to enter the lymph nodes. The lymphatics are full of holes, and once cancer gets into them, it has a lot of places it can get out from again.

  Today, doctors sometimes use glue to fill in holes in the lymphatics where there are obvious leaks. These treatments have been tested with limited success on infants born with congenital lymphatic defects, or who suffered heart conditions in utero and are born with lymphatic swelling. The goal of the procedure is to encourage their bodies to form collateral pathways—alternative channels—around the glue and to continue to move the lymph through healthy parts of the system. In 2016, long-term studies still weren’t conclusive. So in 1997, all of this was largely fantasy.

  But my father wasn’t asking for a conventional cure for his unconventional illness. He was okay with long shots. In his case, if all this leaking fluid was channeled directly
into the veins, it was possible the body would find a way to reabsorb it, and keep it from leaking. Everyone was on board with trying to get my dad into the Italian study, but there remained the question of whether or not he would be strong enough to make the journey to Europe, much less survive the procedure itself. In the end, he was never invited to Italy, and no such procedure was offered in Tucson.

  However, the doctors did cave after my father spent weeks begging them for proof that his miserable fat-free diet was actually helping. Dr. Witte sent him to a nutritionist who decided the fat-free diet simply wasn’t doing enough to justify my dad’s discomfort. It’s possible the nutritionist just felt compassion and didn’t see a good outcome for my dad.

  But back in Columbus, Dr. Lynn insisted he had statistical and medical proof that the fat-free diet was elevating my dad’s numbers. This indicated that at least some of the nutrients that had been disappearing into my dad’s lymphedema were finally breaking down and getting into his system. This diet showed at least a tiny bit of possibility that my father’s starvation could be turned around or at least slowed down.

  My dad was a man who desperately wanted to live, and his hatred of the fat-free diet had begun to cloud that objective. When Dr. Witte’s nutritionist suggested that the diet was probably ineffective, my starving and miserable father took it as good news and ran with it to the nearest purveyor of the Chicken McNugget. When the compassionate release of olestra came through, it no longer mattered.

  By the time my parents returned to Columbus, my dad had been eating every fatty food he could get his hands on. His numbers looked terrible to Dr. Lynn.

  * * *

  That spring, although I had technically taken a semester off to help out with my father, I got a full-time job at a drug and alcohol day-treatment center that was run by Jackie and Jimmy. It was ironic for me to work in rehab, as I was enjoying a thriving pot habit and the occasional opiate-based painkiller that I stole from my father. My social life also stayed active. I often went down to Cincinnati to visit my best friends, Amy and Jason, who were going to school there, or they came to Columbus to stay with me during many of the weeks my parents went away.