Here is the full summary.
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.
This book will be released in print and e-book on July 1, 2021! Stay tuned for more information.
All told, N6A operated like a large close-knit family. “GI kids were frequent flyers. They were there for a long time and kept coming back,” said Lisa Locasio, a staff nurse who was also just starting her career then. “We got very close to those kids and their families. It was an amazing unit. Everyone was so invested in the care of these children.”
Today, nurses aren’t allowed to snap photos of their patients, but this wasn’t the case in the early 1980s. “I took pictures of all of my patients on my personal camera and I made photo albums,” Locasio said. The photo albums were her own personal stash of memorabilia so she could remember each and every one of her young patients.
Nurse Lisa also bought stuffed animals with her own money to place in her patients’ cribs. Having anything in a crib is considered unsafe today due to SIDS (sudden infant death syndrome) and suffocation hazards, but not back then. The nurses also bought me outfits. One nurse, Jan, even tape-recorded my baby chatter at night so my parents could listen to what I said when they weren’t there. Jan dubbed me “Little jabber John.” I sounded comfortable and happy, which gave my parents peace of mind. They worried when they left me each night, but they needed sleep and trusted the nurses, who provided an amazing support system. “If we hadn’t been comfortable, it would have been very hard to leave the hospital at all,” Dad said.
To stay as healthy as possible, I required a special infant formula and supplemental nutrition through a feeding tube that was taped to my face. The nutritional values of the infant formula had to be higher in fats and vitamins than standard formulas. With biliary atresia, babies don’t absorb calories and fat-soluble vitamins like they should. The special formula was used to help offset these issues.
“To be eligible for liver-transplant surgery, we had to keep John growing,” said Pat Dacunha, the registered dietitian at Hartford Hospital, who managed my nutritional needs.
If I didn’t reach a certain physical size, they couldn’t operate. It was as simple as that. Consequently, my medical care required daily weigh-ins, calorie counts, and even the monitoring of every little ounce of fluid and every morsel of food I consumed. Under Dr. Hyams’ careful direction and guidance, Head Nurse Nancy Etienne was also instrumental in my medical management. She tracked every ounce of fluid, every medication, and every laboratory value, including urine output in a notebook. Careful fluid management and dosing of IV medication was part of the reason I had to stay in the hospital. Because computers weren’t used for data compilation yet, everything had to be handwritten or typed on a typewriter into a written record.
At 6:00 a.m. daily, Dr. Hyams made his rounds. “It was hard to beat Dr. Hyams in the door in the morning,” Dacunha said, who was just 24 at the time. If baby me hadn’t gained any weight or if I even lost weight, it was Dacunha’s job to figure out why, hopefully before Dr. Hyams arrived. Dr. Hyams ran a tight ship, lighting a fire under everyone with his expectations.
“We all got better at our jobs because Dr. Hyams set the stage for accuracy and exact measurements for intake and output,” Dacunha said. Medications and fluids were given to patients on time. Nurses were held accountable too. If labs were drawn, the results had better be written on the flow chart. ‟If labs were ordered, but not drawn, you didn’t want to be around to hear what went on,” Dacunha said. If a nurse forgot to weigh me during the night shift, Dacunha quickly got me on the baby scale before Dr. Hyams could notice the missing data. His patients’ care was top priority and all the data was important to maintain or improve health. The numbers were everything. Even though Dr. Hyams was a young doctor, Mom and Dad could tell I was in capable hands.
Looking back on that time, there were so many ‘what-if’s.’ What if my parents hadn’t just moved to Rocky Hill? What if Dr. Hyams had started his career somewhere else? Where would we have been after this was all said and done? When you meet with a good doctor, you know it. It’s a feeling of trust that stays with you.
“Bill-ee-air-ee uh-trees-ya.” When Dr. Hyams delivered my diagnosis, dramatically annunciating each syllable with emphasis, Mom and Dad repeated it out loud slowly so they could remember it later when they had to relay the news to our extended family and friends. As serious health issues go, Dr. Hyams bluntly started with the facts breaking it down as easily as possible for them. Biliary atresia is a rare liver disease that only affects infants. In 1981, it affected one in 20,000 babies in the United States. A rare disease affects fewer than 200,000 people. It was indeed rare.
The liver, even a baby’s liver like mine, which was the size of a lima bean, plays a big part in helping food digest, Dr. Hyams explained. Biliary atresia targets the liver’s bile ducts, a system of tubes that carry bile (a digestive liquid) from the liver to the gallbladder and the small intestine. Bile is essential for digestion. Bile breaks down fats into fatty acids, which the digestive tract can use to fuel the body. With biliary atresia, however, the cause of which is unknown, the bile duct system becomes scarred and blocked. Consequently, bile can’t flow into the intestine like it should, so bile builds up in the liver, which leads to scarring (cirrhosis) and loss of liver function; food can’t digest like it’s supposed to, which is why I wasn’t gaining weight. Biliary atresia can be fatal especially without a transplant, Dr. Hyams repeated. But luckily, my biliary atresia was caught early, which was good, he said.
Dr. Hyams was already well known in the field of gastroenterology by the time he started at Hartford Hospital in July of 1980. When I became his tiny patient in 1981, I was one of his very first patients with biliary atresia. He was young in his career, but brilliant in his field.
“I was Doogie Howser,” Dr. Hyams said, referring to Doogie Howser, M.D., the medical comedy drama TV series about a 16 year old genius doctor that ran for four seasons, from September 1989 to March 1993, starring Neil Patrick Harris, who played the title role of the teenage Doogie Howser, M.D.
Dr. Hyams explained that the first treatment for biliary atresia is the Kasai procedure, which was the routine standard of care for the time. Named after Dr. Morio Kasai, a Japanese surgeon who developed the technique in 1951, the Kasai procedure revolutionized the treatment of biliary atresia in infants throughout the world. The Kasai procedure involves the liver’s plumbing being rerouted. Doctors carefully remove the delicate liver’s damaged bile ducts and replace them with new ducts, constructed from a small segment of a tiny patient's own intestine. The new ducts connect the liver to the intestine, forming a Y-shaped passageway, so bile could drain like it should normally.
“We understood the Kasai procedure wasn’t a full-time or long-term fix,” Dad admitted. Still if successful, the surgery could slow liver damage and delay or prevent complications later, buying me more time. In thirty percent of children, the Kasai procedure was a permanent success, negating the need for a liver transplant. My parents clung to that hope.
The downside—seventy percent of children with biliary atresia who underwent the Kasai procedure would eventually develop cirrhosis and end-stage liver disease. In other words, biliary atresia was fatal if the Kasai procedure didn’t work. However, my parents had no other choice but to take this next step.
At my six-week well-child checkup, however, it became apparent I was 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? The doctor mentioned my skin was a little yellow too, even noticeable against my fine wisp of blond hair.
“I see what you mean,” Mom said, suddenly noticing the sallow tinge to my cherub-like complexion.
With a quick shrug, the pediatrician said I probably just needed a change of infant formula. Almost like a flight attendant during a bout of turbulence, his face was suddenly emotionless though. Was this just a bumpy ride or a sign of something truly serious? The pediatrician didn’t seem to know any more than he was letting on, but Mom and Dad couldn’t be sure.
“It’s probably nothing,” the pediatrician said, as if reading their worried minds.
Still, the doctor wasn’t taking any chances. With a whisk of his pen across a prescription pad, he sent us to a specialist for a series of increasingly more inclusive testing at nearby Hartford Hospital.
After a week of blood tests and ultrasound scans, the specialist, Dr. Jeffrey Hyams, a leading pediatric gastroenterologist, said he thought he knew what the problem was, but he couldn’t be sure. Exploratory surgery would be needed and hopefully yield some definitive answers.
Soon, with my trip to the operating room complete, my parents got the full story. Even though the doctors had been 99 percent sure I didn’t have the most serious illness mentioned only as a remote possibility, that’s in fact exactly what I had: biliary atresia, a potentially fatal liver disease. The diagnosis was like a stab to my parents’ hearts, as they sat there in my hospital room, stunned.