LIVEr My Journey of Transplant Survival

By John Hoffman

Born with the rare liver disease biliary atresia, my only hope for survival was the untimely death of another young child, and the success of a very experimental surgery. With the outcome never certain, my parents remained steadfast in the belief that I would get through this and live a long and healthy life. That belief and support carried me through my adolescence, where I was determined not to let my medical history define me. After receiving my second transplant, and third chance at life, I was bound and determined to make it mean something. I struggled with this theme throughout the rest of my high school and brief college career. While medically, I was a resounding success story, emotionally, I was in turmoil. Depression marked a large portion of my late teens and early twenties, as I began to attempt to make my way as an adult. One thought continued to gnaw at me, "Two people have lost their lives to get me to this point, and what do I have to show for it?" It wasn't until years later that I would find my direction - I needed to share what I've been through. While my past shouldn't define me, it helped to mold who I am, and perhaps I can help others going down this same path. The result of my epiphany is this book. I wrote it not only for myself, but for those like me, who have been honored to be organ recipients. This is my story.

• Language: English
• Publisher: John Hoffman
• Release date: Jul 1, 2021
• ISBN: 9798201430917

John Hoffman

JOHN HOFFMAN is Executive Producer of HBO’s  The Weight of the Nation.  He has won Emmy® awards for the documentary series The Alzheimer’s Project and Addiction, among others.

Book preview

Prologue

Dates with Destiny

Most people have one date that signifies the start of their lives, their birthdate. I have three: December 1, January 14, and July 1. These were all days I was given the gift of life. December 1, 1981 was the day I was born. I’m told it was a cold, cloudy Tuesday. My mom's favorite memory: a delivery room nurse exclaiming: He came out like a rubber chicken!

Over two years later, on January 14, 1983, was the date of my first liver transplant. As one of the first infants in the U.S. to ever receive a liver transplant, the surgery signified the end of a long road of sickness and uncertainty and the launch of liver transplantation in the U.S. Of course, I was too young to have any recollection, but I have come to appreciate and understand its impact. At that time, taking an organ out of one body, be it a body that only weighed in at 14 pounds, and putting it into another, was a herculean feat performed by only the most innovative and brave. My parents had no choice but to stake my life on what was then considered an experimental surgery. Being a parent now myself, I understand what it must have been like to put your child's life in the hands of very competent medical experts, but strangers none the less. When the surgery was complete, I had been given a second chance at life before I even knew I needed it.

July 1, 1999 was the date of my second liver transplant. While typical 17-year-olds may rarely have thoughts about their own mortality, I had come to question mine entirely. That day is burned into my memory. I had been sleeping over my Aunt Nancy's (I called her AuntieNan) house in East Hampton, Connecticut, about 35 minutes away from home. At about 2:30 a.m., a phone call would precipitate a frantic car ride up Route 9 in Connecticut. It only took about 15 minutes to get back home and wake Mom to tell her she was about to put her trust in those strangers once again. After a flurry of packing and tearful goodbyes, we were off.

After going at breakneck speed since about 2:30 am, we had to hurry up and wait for the plane. A Learjet that had been arranged courtesy of Dad's employer, Pratt & Whitney Aircraft, was summoned. In general aviation area, you would never know what frantic messes we were just eyeing us in the waiting area. While I listened to music, Dad caught up on some reading, and Mom took a look inside her suitcase, which had been hurriedly packed by my stepfather Tom, while she was in the shower sobbing and shaving her legs earlier that morning. Snowpants! Mom exclaimed, not expecting to see those in there for a mid-summer trip to Pittsburgh, Pennsylvania. I couldn't blame Tom for the mistake; there was a lot going on! It was a much-needed chuckle during the tense waiting period.

Finally, the jet arrived. I'm no airplane connoisseur, but it was a pretty nice private jet. My teenage eyes immediately found the stocked wet bar with sodas, and plenty of junk food ready to go, of which I have could have none, since I was being prepped for abdominal surgery. The brief flight is all a blur to me, as I watched my home state of Connecticut fade into the distance, and the eastern seaboard pass underneath us.

We landed on the tarmac, and carefully stepped down the ladder onto the pavement. An ambulance was waiting, and two medics zoomed past us with a gurney and headed toward the plane. Where's the patient?! one exclaimed. I timidly raised my hand, not sure what I was in for. They looked puzzled, not expecting a patient experiencing liver failure to be standing upright, never mind carrying his own bags. I opted to walk into the ambulance and lie down.

After a wild ride to the hospital, having never ridden in a vehicle that large or going that fast, we arrived at the Children's Hospital of Pittsburgh. Once I was admitted and brought to a pre-op area, a young resident, fresh out of medical school, stopped by to do a preliminary checkup and make sure I was ready to go.

So, John, why are you here? he said.

I was baffled. I had thought of nothing else but this moment for the past two years. Did they not have all my information?

Umm, I'm here for a liver transplant, I said.

I was hoping his question was just his folksy way of getting to know me. The resident proceeded to get some basic information from me and my parents, then he administered a mild sedative, which was always my favorite part, having spent so many years undergoing medical procedures. I drifted off into la la land, preparing for my life saving transplant. But this time, my parents weren't alone in the fight. Although I had been weakened by a failing organ, I knew that what lay on the other side was worth fighting for. One life-saving liver transplant had brought me to this point. Now I was going to need another.

This is my story of cheating death twice through the modern miracle of liver transplantation, the best medical teams anyone could wish for, supportive friends and a loving family, and unbelievable timing—and the havoc it would wreak on our lives.

1

Growing Concern

December is normally the month many of us focus on presents. At ten days past my due date, my parents, Katie and William (Bill) Hoffman, celebrated the ultimate gift—a perfectly healthy baby boy—and a good size one at that, 8 pounds, 7 ounces. Newborn babies on average are about 7 to 7 pounds, 8 ounces.

I made my debut on that clear day of December 1, 1981, marked by frozen puddles on the sidewalk leading up to St. Francis Hospital, in Hartford, Connecticut. At the moment I was born, the attending nurse announced, He came out like a rubber chicken! The silly comment made everyone laugh, adding levity to the uncertainty and drama of labor and delivery.

We all have a birth story and the rubber chicken one is mine. The humorous description of newborn me has been relayed to me countless times throughout the years. It’s what I always associate with my birth. Yet, I’ll never know why the nurse described me in that particular way. I wasn’t a rubber-chicken or even baby-bird scrawny. In fact, I was the largest baby in the hospital nursery that day. All of the other babies seemed small in their hospital bassinets; then there was me, looking like I had already outgrown mine. You could pick John right out, Dad would recall about that day. As my parents gazed at the bassinet line-up through the paned glass window, Mom said I was almost embarrassingly large, compared to the others. Dad told her she had done a stellar job incubating me, apparently, and they laughed. Being the biggest is one of those proud parent facts, especially for first timers. My son, the brute! That was me.

At my six-week well-child checkup, however, I began losing ground in the hefty baby department. Infants on average should gain a pound per month for their first six months, but the needle on the analog scale in the pediatrician’s office wasn’t budging.

Hmm … John should have gained a pound and a half by now, the pediatrician said, squinting with concern at my growth chart. Why was I still stuck at my birth weight, especially since I was a good eater?

With a quick shrug, the pediatrician said I probably just needed a change of infant formula. It’s probably nothing, he said.

Mom and Dad nodded. They weren’t alarmed. We just thought John was perfect, Mom said, about baby me.

We didn’t expect anything but flying colors, Dad said.

The pediatrician gave Mom about week to try different infant formulas without results. Then, with a whisk of his pen across a prescription pad, he sent us to a specialist for a series of tests at nearby Hartford Hospital, a teaching hospital with no shortage of medical residents with repetitive questions about seven-week-old me every time there was a new staff rotation.

At Hartford Hospital, I had a series of blood draws, injections, and scans with contrast dye. It was hard on us, watching them poke holes in John’s perfect little body, Dad would recall. In fact, the poking was so torturous for my parents to witness they quickly took charge of the situation. If the medical staff was unsuccessful at inserting an IV into my tiny infant veins after two tries, my parents announced that was it! The staff had to find somebody else more competent to do the job.

Nothing medically definitive turned up after the first round of tests, so more tests were ordered in rapid succession. The tests were being used to rule out biliary atresia, a potentially fatal liver disease, which was the worst-case scenario. Dr. Jeffrey Hyams, a leading pediatric gastroenterologist, said he was pretty sure I didn’t have it, and that gave my parents comfort. Plus, some test results were coming back okay. But the emerging picture was a muddle. Invasive exploratory abdominal surgery would be needed to hopefully yield definitive answers. That was all doctors had left.

My trip to the OR seemed to drag on forever. Afterwards, my parents waited with me for several hours in the drab, antiseptic hospital recovery room, complete with monochromatic walls. It was an adult surgery ward in the process of being transformed into a pediatric gastroenterology unit. The cheerful children’s hospital setting you might see today was yet to come. After my surgery, my perfect baby body now had a large scar that snaked from my belly, above my belly button around my side to my lower back. What happened to perfect me? My parents were shocked. It wasn’t just a little scar; it was huge, Dad said.

When Dr. Hyams entered the room, he didn’t attempt to brighten the mood. That’s when my parents got the full story. Even though Dr. Hyams was so certain I didn’t have the most serious illness mentioned only as a remote possibility, that’s exactly what I had: biliary atresia. The potentially fatal diagnosis was like a stab to my parents’ hearts, as they sat there in my hospital room, stunned. I couldn’t move, Mom said. My world fell apart. She could almost feel the earth crumbling beneath her.

The diagnosis was a big shock, Dad said.

What do you do when you’ve been delivered such terrible news? You blame the messenger, of course. Mom instantly hated Dr. Hyams because he had said biliary atresia was so unlikely. The only consolation was that at least now, my symptoms finally made sense.

The only 100 percent cure for biliary atresia was a full liver transplant, Dr. Hyams said. However, this was 1981 when the field of liver transplantation was just like me--very much in its infancy.

Both 29, Mom and Dad had been married for eight years by then. It’s good we waited so long to have you, Mom said. We needed to be just a little bit more grown-up before we faced what we had to.

Like buying your dream home when the market takes a dive, however, timing is everything. Little did my parents know that just as I was diagnosed with biliary atresia on that fateful day in December 1981, the University of Pittsburgh would launch the nation’s first liver transplant program under the direction of their new hire, Thomas Starzl, MD, who would eventually become known as the father of transplantation. Children’s Hospital of Pittsburgh, which was affiliated with the University of Pittsburgh, had yet to perform the surgery on a child as young and as small as I was. In other words, a liver transplant was a long shot to say the least, for little me or anyone. Still, with this first ever liver transplant program, there was hope. Dr. Hyams told my shattered parents that his job was to keep me alive long enough until a donor liver became available.

After such devastating medical news, Mom and Dad had no choice but to just take me home. A whole team of doctors and nurses had been caring for me for a week in the hospital. Now, it was just us. You suddenly seemed so fragile, Dad said. From there, Mom and Dad tossed and turned each tearful night and just did their best to keep me alive until my next doctor’s appointment. There must have been something I did in my pregnancy, Mom kept wondering, a persistent thought that kept her awake at night.

We were scared all the time, Dad said.

Dad and I, pre-transplant - 1982

2

Small Baby, Big Problem

When Mom and Dad met, they had been sophomores and coworkers in Findlay Dining Hall at Pennsylvania State University at University Park. As the story goes, Mom’s roommate, Julie, who also served sandwiches and French fries to ravenous underclassmen, had a crush on Dad, a dishwasher and busboy. During an intramural football game Dad was playing in, Mom’s roommate introduced Mom to Dad as this is roommate. She never mentioned Mom by name. However, Mom was already on Dad’s radar. Katie looked cute with her long hair piled up in a hair net in her white dining hall food server uniform, Dad said. I had noticed her long before the football game.

Soon, Dad was in Mom’s sights too. I was drawn to Bill’s sensitive and caring nature. Plus, he looked great in a gray T-shirt, which was his dining hall uniform, Mom said. Things seemed so simple then. Mom was outgoing and