On Transparency

Cackling, my classmates point at my face. “You look like a ghost!” one sneers. Confused, with no access to a mirror, I ignore their taunting and turn back to my work. When I feel the nausea creeping through me, I know something is wrong. Instinctively, I reach down to feel the tubing through the fabric of my shirt. Wet. Uh-oh.

Later, I would learn that I turned ghost-white when my intravenous Flolan wasn’t getting through my Broviac catheter to my heart. That afternoon, way back in second grade, a connection in my tubing had broken, and medication was leaking out of it.

The drug has a six-minute half-life, so I start to feel the effects of withdrawals quickly. My mom rushes to the school to replace the tubing, our house conveniently – and intentionally – located only a couple blocks away. We sit in an empty classroom until I start to feel better.

The event set off a lifelong tradition of transparency about my condition. My mom and I gave presentations about pulmonary hypertension to my class every year after. We explained how the disease affected me, and what they could do to help in the event of an emergency – namely, inform someone who could get ahold of my parents. In middle school, I began presenting to all six of my classes myself. The process was intimidating, but always warranted a positive response.

I’ve been trying unsuccessfully to think of a time when educating my peers paid off – when one of my classmates really helped me out. Perhaps they never did – I knew better than anyone what I needed and how to get it. But these presentations took a lot of stress off me. They transformed my illness from an invisible secret to a topic of discussion. My candor eliminated any imagined need to hide my pump. I took the questions all at once, and told my classmates to ask my mom if they had more.

November is PH Awareness month. Raising awareness is a form of survival for me. In college, I needed my roommates to know about PH. Of course, it was pretty conspicuous when I pulled out a tray of supplies and began using needles and syringes to prepare the medication for my pump. Between midnight trips to emergency rooms and frequent deliveries of oxygen tanks, my disease inevitably factored into my living situation. Now that I am immunosuppressed to protect my transplanted organs, it’s critical that my friends understand my susceptibility to infection so they won’t come over to my house when they’re sick, or take bites of my food.

When an inebriated young man with a charming European accent blocks my path at a party to ask me about my mask, I politely explain the reason. Honestly, I’m taken aback that he cares about my flimsy filter mask at a party where a space helmet and inflatable T-Rex costume are universally accepted. The young man insists, to his companions’ dismay, that he can’t understand what I’m saying. He starts repeating, “I can’t hear you. I just asked you your name.” Friends come to my rescue and echo my explanation. They encircle us, trying to protect me and keep me moving toward the rest of our group. Gratitude for my friends eclipses my annoyance with the man’s drunken behavior.

Involving my friends in my disease strengthens our bond. They’ve shown up for early morning fundraisers, and visited me in the hospital. Reactions to my medical emergencies have come along way from mocking my ghost-like appearance.

“Team Kathleen” represented at San Francisco’s 02 Breathe Walk one rainy morning in March, 2016.

Originally published by Pulmonary Hypertension News.

Pre-Op

5 Things I Wish I Knew Before My Transplant

Knowing transplant was my plan when all else failed, I lacked curiosity to gather information before making a decision. I focused on survival: more information only increased my anxiety. During my training class, I scribbled in a sketchbook and relied on humor to gloss over the enormity of what I was facing. I still don’t wish I had known that my transplant would come with unanticipated and persistent complications, or that it would involve more physical pain than I had ever experienced before. I could not prepare for those aspects, and knowing they were coming would only have drained my energy to fight them.

Some notes I took during transplant training. “Grapefruit sucks” comment courtesy of my friend, Max. (Photo by Kathleen Sheffer)

Friends who are looking ahead to a possible transplant in their future often ask me for advice to prepare. With that in mind, I’ve listed a few things I believe I would have benefitted from knowing before my transplant:

1. Transplant comes with permanent dietary restrictions.

When I had pulmonary hypertension, I did not appreciate that I could eat anything I wanted (probably because I rarely wanted to eat anything at all). I no longer battle nausea or struggle to maintain a healthy weight, but chronic immunosuppression does come with restrictions. Grapefruit interferes with immunosuppressants (and many other medications), but that was easy to give up since I don’t regularly encounter grapefruit in restaurants or friend’s homes. Not until after my surgery did I learn that all meats must be hot within an hour of consumption. That means no sushi. No cold cuts. Ever again. Had I known this before, I would have found a nice spot to park my oxygen concentrator and started pigging out on spicy tuna rolls and deli sandwiches.

2. Chest tube scars heal.

While listed for transplant, one of my main preoccupations was the impact chest tube scars would have on my body image. I already had a chest tube scar from one open-heart surgery, and I wasn’t fond of it. Chest tubes would be better labeled chest hoses–they’re huge! But my chest tube scars healed, and they look more like an awesome tattoo than six additional blemishes to my already scarred torso. Honestly, I think I should have more impressive scars to show for the battle I fought.

3. It takes a full year to recover. Don’t kid yourself.

Imagining myself younger, stronger, and more determined than most patients, I assumed I would recover quickly and surpass expectations. But it was a year before I tapered down to the lowest dose of Prednisone. A year before my doctors felt comfortable spacing appointments months instead of weeks apart. A year before my health stopped being my number one focus and I could find other things to talk and dream about.

4. Patients are surviving.

Going into transplant, I had a few friends who had received heart-lung and double-lung transplants, but the longest survivor I knew lost her battle after 12 years. I had less of an optimistic outlook on survival rates than I do now that I know more about the statistics and their inaccuracy. There are recipients alive today who had their transplants when centers first started performing heart-lung transplants (over 30 years ago), so doctors truly cannot determine a limit for how long these transplants can last.

5. Quality of life does improve.

Oxygenation had far-reaching effects that make me able to say with confidence that my quality of life after transplant is immeasurably better than before transplant. Simply by virtue of spending more hours a day awake, I get more done, appreciate life more, and have more experiences with the people I love. The best part? Joining my parents on hikes and sensing their joy as I speed ahead.

Hiking with my parents in Sedona, Arizona, six months post-transplant. (Photo by Monica Sheffer)

This article was originally published to my column, Life After PH through Pulmonary Hypertension News. Since then, I’ve gotten feedback from other transplant patients adding to my list, and patients pre-transplant determined to savor the foods they can still eat. Still others have asked questions only their doctor can answer. I have a hard time empathizing with those who obsess over certain sacrifices necessary for transplantation. No, I’m not another voice in transplant support groups chanting, “But you can breathe!” as if that fact eases every trial for a lung transplant patient, despite its inefficacy on the rest of the oxygenated world. What I keep going back to is that transplant is a choice between life and death. One does not receive donated organs otherwise. Most will choose to live, and find ways to adapt to whatever life brings.

Pre-Op

The Wait That Helped Me Heal

The Hard Part

I’ve heard that waiting for the transplant is the hardest part. Every day after my transplant, I heard, “This is the hardest part,” leaving me a firm believer in relativism and forever poised for future “hardest parts.”

When I went on the heart-lung transplant list, I didn’t know if I would survive the wait for donor organs. My health was declining at a rate I’d never experienced in my 17 years with severe Pulmonary Hypertension (PH). I woke up knowing each day might be my last and fell asleep questioning whether I would wake up.

Born with a prognosis that I wouldn’t live to attend high school, I always struggled to plan for a limited life, balancing the temptation to accept medical expectations with my desire to set similar goals to my peers who weren’t battling chronic illness. When college became particularly demanding, I considered giving up. “If this is my last year alive,” I thought, “do I really want to spend it shut up in a concrete tower of architecture studios?” Instead, I graduated with a degree in a related field that allowed for fewer dizzying all-nighters.

Waiting for a transplant was a new version of my indeterminable life, with additional limitations and unanswerable questions. The four-hour radius my transplant center gave me was less limiting than the nasal cannula tethering me to my oxygen concentrator. Walking a block made me light-headed and climbing the stairs to my room left me gasping for breath.

Feeling sick and tired and sick and tired of waiting.

After cancelling several trips I’d scheduled before my hemoptysis event, I was hesitant to make new plans, however small. I met a friend for coffee the day before I got the phone call offering me my new heart and lungs: we discussed meeting again soon, but I couldn’t commit. “Maybe I’ll have the transplant tomorrow. Who knows!”

The Transformation

I kept a journal during this period: “Now I’m in purgatory waiting for transplant and no one has any idea when I will get the call,” I wrote. “Could be tonight; could be two years from now.” My phone was always charged with the ringer at full volume. Knowing it might never ring, I had to consider how I wanted to spend my last days and the legacy I would leave.

While coughing up bright red blood into my friend’s toilet bowl in Seattle, I was quite sure I would die on the cold tiled floor. In that moment I cared little about the places I had yet to visit, and the milestones I had yet to reach. I cared about the impact I had made on other people and words left unsaid.

Immobilized by an unknowable future, the wait for transplant gave me time to cover emotional distance. I sorted through past traumas to get to the root of my fears about going into surgery. I had candid conversations with my caregivers about what I wanted in the event of my death. Most importantly, I expressed my love for my family and friends.

A cartoon of my internal organs celebrating a transplanted heart and lungs. that I drew 8 hours before my call for transplant.

Though I moved in with my parents and away from my local friend circle while I waited, friends from all over the country began reaching out. A friend with whom I’d only spent one week at summer camp quickly became my closest confidant, our friendship existing only through text messages and care packages until five months after my transplant.

Reading and writing were chores I did just to get by in school. Unable to do much else, I embraced the two as a distraction and creative outlet. I started reading memoirs with the idea that I might write my own during what I imagined would be at least a year spent waiting for a donor.

28 days after listing, I got the call that ended my wait. In the almost 14 months since my transplant, I read 29 books, started a blog, and had my writing published by The Mighty and Pulmonary Hypertension News. Watching my health improve for the first time, I can plan for the future like I never dared before.

Originally published by Pulmonary Hypertension News.

Pre-Op

Recognizing My Window of Opportunity for Transplantation

The changes were gradual enough not to alarm me. I went from a steady 110 to 118 pounds in less than a year, but always celebrated weight gain. The blue coloring in my lips and fingernails was more pronounced, but I figured I was just paying more attention. Determined to live as normal a life as possible, I conditioned myself to ignore the signals my body sent me.

Originally listed for a heart-lung transplant in 2002, I deactivated when the U.S. Food and Drug Administration approved sildenafil (OK, it’s VIAGRA; make all the jokes you want about my miracle drug) to treat pulmonary hypertension (PH). My pressures dramatically improved, but transplantation remained the backup plan. After another full evaluation and workup in 2015, the team told me I was sick, but not sick enough to earn necessary priority — listing me at a “Status 2” on the heart transplant list (the lungs follow the heart in a heart-lung transplant) would be “purely academic.”

My mother and me at Stanford for transplant evaluation in 2002.

Though I profess to have learned the true meaning of Christmas long ago, my heart (like that of Dr. Seuss’ Grinch) grew three sizes, compensating for failing lungs and pushing them aside in the process. My heart pounded against the constricting fabric of a dress that had fit just right months before. Instead of resenting my boyfriend who fumbled with the zipper when I needed the dress OFF NOW, I should have wondered why cushy corporate parties six months into my post-grad life were more exhausting than nights spent hiking up and down the steep hills that connect UC Berkeley’s Greek system.

Shouldering a portable oxygen concentrator and a suitcase packed with equal parts clothes, medication, and camera equipment, I boarded a plane to Seattle. Racking up business expenses for my first out-of-state photography gig, I took an Uber from the airport to a cafe. Later, I met up with a friend from college to explore Capitol Hill, eat Thai food, and crash on her couch.

When I met Xio at orientation, I didn’t think our friendship would make it past the awkward incoming freshman stage, or that she would save my life. My classmates and I stared sleepy and green-eyed as she impressed professors by consistently interjecting insightful questions and creatively arranging sliced bell peppers. Xio and I spent most of our time together late at night under pressure, but I never imagined it would prepare us for anything other than surviving future architecture studios.

I woke up coughing, afraid to disturb Xio’s sleep. Under the light, what I had coughed into my hand was bright red, as was the mass I was now coughing up into the toilet bowl. Wheezing blood, I was busy contemplating how unfortunate it was that I was going to die in a bathroom, when I heard her voice. “9-1-1?” Xio asked. I cough-moaned agreement and listened to her calmly give directions to an operator.

View from ICU room at the University of Washington.

Two ambulance rides and three hospital rooms later, I learned that this event (“massive hemoptysis”) signaled a serious progression of my disease. My doctors said I was lucky to have survived it, and might not survive another event. I believed them.

My pulmonologist rejected my parents’ designs to drive me home, and instead advocated to have me medically transported by Learjet to Stanford, listed for transplant, and prioritized at Status 1B. At age 23, the decision didn’t require debate. My oxygen saturation had dropped as low as 37% and was still hovering below 90% while lying in bed on supplemental oxygen. Without new treatment options to try, I couldn’t wait another few years for improvements to transplantation. I had maxed out. After fighting PH for 16 years with transplantation locked away in savings, I wiped my account.

To say I was lucky would be an understatement. My know-it-all friend knew how to get me the help I needed, and my pulmonologist recognized my window of opportunity. Twenty-eight days after being listed, my donor and surgeons gifted me a healthy heart and lungs that not only reshaped my perspective on life, but also physically reshaped my body, making it possible for me to fit back into that dress.

Chest x-rays before and after transplant.

Originally published by Pulmonary Hypertension News.

Pre-Op

Donate Life Month

April is National Donate Life Month, and my birthday month! I am so excited to turn 24, and this milestone is only possible because of my donor’s generosity. I’ve committed myself to promoting organ donor registration all month long through my social media.

I’m writing this having just returned from a six-hour photoshoot at 8pm. The images are being imported into my Lightroom catalog and I will have the energy to begin processing them tonight. I would never have been able to do this before my transplant and I don’t yet have all the words to express the joy and confidence this gives me.

This morning I flipped through the journal I wrote in while I was waiting on the heart-lung transplant list. The following is the last entry in it:

I’m not just waiting–I’m also sick. The story that I’m waiting around until I get a call that will allow my life to begin takes away every last strand of control I had. The reality is that I get out of breath going from my room to the bathroom and back. The reality is that I cannot photograph a wedding. I cannot walk my dog multiple times a day. I do not have the energy to cook and clean and do laundry. The idea is that I’m waiting for a future where I can do all that again. Maybe that future will come, maybe it won’t and maybe it will take an unbearably long time to get here. But there are lots of things I can do and I do not have to wait for that future to start living. Today I am in the present. I have control over what happens today–and I’m not going to spend it twiddling my thumbs and watching the clock.

After this perspective shift I started oil painting and going on occasional outings. But I only waited 17 more days before getting a call offering me my new heart and lungs. I was incredibly lucky. Having lost several friends to Pulmonary Hypertension while they were on the waiting list, I wrote in this journal knowing there was a good chance I would never get my call. There are currently 120,000 people waiting for organ transplants in the United States.

The same day I wrote this journal entry (thank you to my past self for having the good sense to date these things!), the White House hosted a summit announcing the Obama Administration’s call to action to reduce the waiting list for organ transplants. According to their statistics, 95% of Americans support organ donation, but only 50% are registered as donors. Are you registered? Make sure you are by visiting RegisterMe.org, and please talk to your family about your wishes.

My organ donor gave me a future in which I can do more than I ever thought possible. They are remembered and celebrated daily.

Pre-Op

The Call

I didn’t stress about packing absolutely everything I needed because I believed it would be a dry run. These are never actually rehearsals, but they’re referred to as dry runs after it’s determined that the potential match is not a good one. Almost every transplant patient I knew had had at least one—one friend even entered the Operating Room before they called it off. I figured it was a rite of passage: I would go through the motions today, but I still had a long time to wait. Just 28 days on the list of transplant candidates and they had a match for me? Impossible! My heart was racing too fast for me to think clearly about what I wanted to bring. I shoved a few books and art supplies in a bag, grateful for my parents for handling the backup of medical supplies so I could focus on the important things, like updating Facebook and locating the henna ink. My dad returned from work and my younger sister, Monica, was woken upstairs. Our house was filled with hurried footsteps and phone conversations. Excitement, fear, and confusion reigned. I stood in my newly converted downstairs bedroom—taking the stairs was out of the question this morning so I dictated clothing requests to Monica in a routine we’d cemented into the foundation of our relationship. After 21 years together as Monica, Kathleen, and Chronic Illness, it had become her implied responsibility to get up and walk across the room to retrieve the scissors I wanted from the shelf two feet away from me. Three weeks later I headed up a different set of stairs to retrieve her shoes and wallet in an attempt to restructure our roles around my new health status.

I had imagined this phone call several times a day for the last month. After one too many heart-stopping calls from numbers with the 650 area code (“Ok, Melissa, you’re calling from the Stanford Chest Clinic, but do you have a donor for me? Speak faster so I can breathe again!”), I called my nurse coordinator to ask what sort of an introduction I could expect. She said they would make it clear immediately that this was not your standard appointment-scheduling phone call that I seemed to be getting so many of and patiently talked me through the questions they would ask me. The phone rang at 7:50am and as soon as the first words were out of his mouth, I knew the man on the phone was calling to offer me organs. He rushed through the script, stumbling over some parts. I eagerly confirmed I did not have any cold symptoms, and had not eaten since the night before. I informed him that I was a two hour drive away from the hospital and he told me they would have a bed ready for me at 10am. It went exactly the way my nurse coordinator had described it.

By 8:40 we were in the car—breathless and jittery, each convinced we’d forgotten the most important item on the packing list we never finished making. The drive to Stanford was less than two hours, but it felt like traffic moved slower than ever specifically to heighten my unresolved panic. I was excited, impatient, and afraid. My heart had not stopped pounding since the phone rang. Normally my heart rate hung between 60-80 Beats Per Minute, but on July 30^th it was consistently between 100-130 BPM. Once admitted, I leaned back in the bed that would transport me to the OR ten hours later, closed my eyes, breathed steadily, and relaxed. Still the screen read 122 BPM. My heart could not ignore the adrenaline pulsing through me, however much I tried to trick myself into staying calm. Somehow it knew this was our last hurrah together and my heart was giving me every last bit of strength it had left.

I have an insane level of respect for the organs that kept me alive for 23 years, which is part of why this experience has been wrought with grief. Don’t get me wrong—I have railed against my tangled heart and poor excuses for lungs enough times to exhaust my most understanding confidantes. It appeared to me that the entire world had great lungs they took for granted and that it was my fate to watch them squander them, often at my expense. I noticed cigarette smoke more frequently when temperatures dropped. Cold air irritated my tight airways, making it harder to breathe and often triggering asthma. I would shed hot, angry tears as I gulped frigid smoke on my way to class, huffing and puffing up the unrelenting Berkeley hills. No matter how hard I tried to find some divine purpose for my illness, it was impossible for me to reconcile the injustice. My bitterness made me feel alone on a campus of 35,000—and all the more bonded to my deteriorating insides.

I waited in room B201 for ten hours. More and more friends arrived throughout the day: it was a festive occasion. We played card games, listened to music, drew henna tattoos on each other, and laughed a lot. The anticipation electrified us all. Amidst all the excitement, my donor was constantly on my mind. I knew that the hours I spent waiting were some of the worst hours in the lives of my donor’s loved ones. I smiled and celebrated new hope gifted to me by a stranger whose friends and family were simultaneously learning of a loss that will bring grief into their lives forever. I am intent on making use of that generous stranger’s most vital organs. I may never get to know who my donor was. Even if I never meet their family, I know we navigate the same waters. My ship has been rocked by grief more times in my 23 years than in some lifetimes. I know how a rediscovered handwriting sample can take your breath away and how a number on the calendar can spoil an entire week. I know that the pain can be just as strong five years after the initial loss. I will be forever indebted to my donor and their family for choosing to be selfless in a moment of intense suffering. Every breath I take for the rest of my life is only possible because of their gift. My hope is that someday I can share some fragment of the joy that gift has brought me with the wave-beaten voyagers my donor left behind.

I solicited my friends to witness the advance directive I filled out as I waited, and forwarded my mom the informal list of final wishes I had written as a junior in college. It includes important things like what kinds of trees to plant in my honor, instructions for how long to keep my Facebook profile active, and various demands for the party that will be thrown in lieu of a funeral. Waiting outside the doors to the Operating Room with just my parents, I apologized for some of the more outdated references. In the two years since I started planning this, much like my peers do their wedding days, my prized possessions have changed and friendships have evolved, along with my social media passwords. During what could have been our final moments together, my mom and I talked animatedly about where to direct donations in the event of my death. Organizing like this is one of our practiced coping mechanisms: our theory is that once we do everything that needs doing, we can return to a state of denial and escape our worries. I’m not going to pretend it’s incredibly healthy, but it works for us.

The whole experience of being in the operating room was surreal: I waited hours, watching as nurses, their backs turned to me, carefully arranged metallic-sounding instruments. Behind me, my gentle anesthesiologist tended to his assigned tasks, starting up conversations with me from time to time. He let me choose the Pandora station: after a few seconds of intense deliberation I settled on Blind Pilot and was pleased that the only complaints coming from our small group were about his lack of a paid subscription and not my selection. The idea that this might be the last playlist I listened to added a whole new level of pressure to a decision I normally left up to more musically literate friends. I asked for a pen and paper because drawing had lowered my heart rate while I waited upstairs. I sketched the scene before me, channeling my focus into the pen and away from the useless worries welling up around me. I sought images of strength and wisdom, and guardian angels appeared in my agnostic mind like deceased wizards from a connection of simultaneously cast spells. My silly grin went unnoticed as I convened with my wise, witty, and spirited angels: I needed Michelle’s charming practicality, Amelia’s stubborn assuredness, and Sarah’s fiery perseverance. I could feel their presence and hear their voices. I felt lucky to be the patient and have nothing left to do—just lie there and trust my body not to give up. My only concern as the team started the infusion to put me to sleep was for those who waited outside the room. I knew I would have no memory of the hours that would pass slowly for my family and friends. My body has an impressive record of defying odds without my awareness so I embraced unconsciousness with confidence. At that point I had no idea the extent to which my body was working to keep me alive, but two weeks later I would see just how far my organs had been pushed.

A note to my beloved readers: Thank you for following along, sharing my posts and sending encouragement my way. Stay tuned if you are curious about the state of my original heart and lungs when they were studied in Stanford’s Pathology lab. I am still piecing together the full experience of receiving “The Call” and would love to hear stories from your perspectives, if you are inclined to share them.