by Julie Kurth
When Adam was prepped and awaiting his transplant operation in pre-op, a nice-looking anesthesiologist came in to talk with him. He explained how the operation would proceed and the medicines that would be used keep to Adam unconscious for the long procedure, which can last up to 12 hours.
After this was explained, the doctor looked at Adam and said, “Don’t ever go to Vegas.”
Adam said, “Why not?”
The physician said, “Because of your luck. This is your second attempt at transplant, since the second one fell through … I wouldn’t count on your odds.”
Of course, the doctor was joking and meant well, but his words would replay in my mind over the next few weeks. I was protective of Adam’s embraced optimism and didn’t want chinks in his armor. Certainly, a failing liver doesn’t happen to every teenager. But was Adam unlucky? We chose to think quite the opposite. In fact, Adam was so lucky that I want to recount the various good fortunes he has had on his perilous journey to better health.
What’s your rush? After Adam turned 18, we started the process to get him on the Mayo Clinic liver transplant list. Mayo has a live donor program, so his PCH hepatologist suggested it would increase his chances for transplant to also be listed there. Anticipating that he would eventually age out of going to a pediatric hospital, it made sense to establish him at Mayo Clinic. We had considered having him listed at Stanford Children’s Health, where pediatric organs were more plentiful, but the interstate travel was a nonstarter due to the costs.
Getting on a hospital’s transplant list takes some effort, involving months of testing, at least in the way we had scheduled it — appointments here and there, so I didn’t miss as much work. Luckily, we live very close to the Mayo Clinic Hospital, Phoenix.
The first Mayo hepatologist we saw asked Adam, “What’s your hurry? Why do you want to be listed for transplant now? Surely, a boy your age would rather be doing just about anything else.” The physician was not familiar with Adam’s case, had not seen his test results yet and just didn’t know how unique this case was. Had we listened and decided there was no big rush, we may have let the pediatric listing with PCH go. I am convinced that for Adam’s strangely presenting PSC liver disease to have been taken seriously in the adult world, he would had to have become very ill.
Listings. It must be said that pediatric onset of disease is typically more aggressive. In Adam’s case, his mysterious liver issues in October 2018 slid downhill quickly. In just two months, the decline was dramatic. At Phoenix Children’s they moved quickly to have Adam listed, tested and treated. Importantly, they also pushed hard to request “exception points” to bump his MELD score higher.
What is a MELD score? Patients waiting for transplant are given priority based largely on a score called model for end-state liver disease. MELD scores span from a low 6 to a high of 40. Adam was originally listed as an 8 at PCH, but progressed to 12, 14, then 17 and finally 21. At transplant, 17 months later, Adam had a MELD score of 20.
About the time Adam hit a MELD of 14 at PCH, Mayo initially listed him as a 7, which is very low. His chance of transplant at this score was pretty much nil. At first, our Mayo team had never heard of continually stenting bile ducts for PSC. However, Adam was stent-dependent. His stents would become blocked, so Adam went under general anesthesia every six to eight weeks to have them replaced. In adult patients, stenting is typically only done temporarily, and adults often live years with the disease before it progresses. When Mayo tried taking the stents out, Adam soon became sick and was back in the hospital within a week. To be fair, Adam presented to everyone as a happy teenager with excellent quality of life compared to Mayo’s other adult patients.
Child-adult shuffle. Once we were through all of Mayo’s testing and Adam was on its transplant list, it soon became a hassle to deal with two transplant teams and coordinate between the two systems with weekly labs and routine appointments. The Mayo and PCH transplant programs were frequently in touch, but I had to make sure one knew what the other was doing. Being listed at both locations also complicated coordination for the doctors.
The pace of medical appointments was too much for us to manage, requiring Adam to miss a lot of his senior year of high school. It was at this point, our family discussed transferring Adam’s transplant listing to Mayo and letting go of his listing at PCH. We were warned by PCH that – due to his age – there would be no going back if we left.
But decisions about transplant should not be based on inconveniences. Instead, these decisions should be made on the urgency for a new liver. Mayo Clinic serves mostly adult patients — quite the opposite clientele from Phoenix Children’s. Being in between the adult and pediatric hospital systems taught us there is a big difference in the way they approach transplantation. The whole experience was very confusing.
The best liver. Our first call about a possible new liver for Adam came from a transplant coordinator at PCH Dec. 5, 2019, while I was walking the dog. Since this was pre-COVID-19, our whole family packed their go-bags to accompany Adam and stay with him as long as possible. Adam stopped eating and drinking right away to ensure he was ready for surgery when the organ was available. In fact, these fire drills often meant Adam went without eating for 24+ hours. Since he was 18, Adam signed a lot of paperwork himself and had a whole lot of blood drawn. We were all sitting around Adam’s hospital bed in pre-op: mom, dad, brother and grandma. Understandably, Adam had a very scared look on his face. We waited beyond the time we had expected. His surgery time came and went. Finally, the transplant surgeon told us the donor liver was not acceptable, especially since it would need to last Adam the remainder of his life.
These false alarms had been emotionally exhausting. We went through nearly the same drill, starting with early morning calls and emails Saturday, Feb. 22. This time since it was RSV season, I scuttled off with Adam, but the rest of our family stayed home. I remember being with Adam in pre-op. Adam said, “Mom, I’m not sure I want to do this now.” Due to the surgically inserted stents, he had not yet realized how sick he would soon get, so having the surgery felt like a choice. Several physicians reminded Adam why the transplant was his only option to better health, which helped. Once again, the available liver did not pass the surgeon’s test. Due to privacy issues, the doctors are not able to say much as to why, but I suspect this was the second liver that was too fatty.
By the time the call offering a liver in March arrived, Adam had a lot he was looking forward to, namely his high school graduation and graduation trip to Disneyland were only a few weeks away. I woke him on a Sunday morning, explaining he should not eat anything and to get ready to leave by 10 a.m. Adam flatly refused. He said, “I’m not doing this today. Let’s wait until after graduation.” We talked and talked about it, until it was time to leave. Adam defiantly looked me in the eye and ate the eggs he had just prepared. We went for a walk to talk things over, and I got the transplant coordinator on the phone for reinforcement. Timing is everything with transplants, so she was eager to hear if the surgery was a-go or not. No matter what anyone said, Adam refused to be transplanted that day. He soon realized his mistake.
Finally, on April Fool’s Day, we received another call from our transplant team at PCH. This time, Adam was ready. He had been hospitalized in January, February and March. His liver was failing, and there was no denying that it was time. Fortunately for him, opportunity knocked three times. The liver he received looked the best of the majority of livers our surgeon had transplanted that year. It was the right time and the right organ for Adam.
Transplant during COVID-19 initial outbreak. Right about the time Adam had his transplant in early April, Phoenix Children’s Hospital started to reduce the number of visitors to one parent at a time per patient. Chris would visit whenever he was able, and Adam’s brother David and friends would FaceTime him. This largely meant that for many days and weeks, I was the only person from home that Adam saw.
The hospital had canceled all elective procedures due to the pandemic. ICU was hauntingly quiet. In fact, for at least one or two of the days Adam was in ICU, he was the only patient in the ward. When he graduated to a regular hospital bed on the floor, I was the first parent to arrive. The nursing staff were unsure if I should be tested for coronavirus or not. All the rules the staff had to follow had changed seemingly overnight.
Working bedside. Although I did spend a few initial nights at the hospital, once Adam was out of ICU, I trekked home to my own bed each night. My mornings consisted of two key routines on the 25-mile commute in to Phoenix Children’s. First, I would dial in to a daily ASU COVID clinical lab team call at 8 a.m. The ASU research institute where I work was then beginning to offer clinical testing for COVID-19, so work was intense. While on my morning calls, I would pick up coffee.
Waving at our favorite security guard, friendly Anthony Bragg, I would proceed to the hospital room, rolling my laptop bag behind me. The room was usually dark upon my arrival, with Adam sleeping in after a night of interruptions for vitals, medicine and labs. I would work by his bedside, watching for the liver team to do its rounds. Once he awoke, he greedily reached for his cold brew, pulled opened the blinds – letting sunlight stream into his room, and told me about his night. During my 12-hour days at the hospital, I periodically moved to the family lounge to join virtual work meetings. Other times, I would be beside Adam, as the revolving door of hospital workers came and went. The cast of characters included therapy dogs, coffee cart, case manager, Child Life, pediatric floor attending, hepatologist on duty, liver coordinator, medical tech, nurses and more.
We were glad to see them; in fact, they seemed like friends. After Adam’s transplant in early April, most of these drop-in visits stopped, except the doctors, nurses the techs, due to Adam’s immune suppression and COVID-19. Soon, we missed the volunteer who pushed the coffee cart and the therapy dog owners who used to visit. Many of our medical consults were via telephone.
Searching for cancer. Adam’s liver disease had presented so aggressively that everyone thought he had cholangiocarcinoma. Neither Mayo nor PCH could not find signs of cancer, however. Even so, diffused large B-cell lymphoma grows aggressive. Fortunately, Mayo Clinic had tried Adam on 40 mg per day of Prednisone in early 2020. This seems to have controlled the lymphoma’s spread.
When Adam was listed for transplant, the nurses explain there are only two ways to bypass the MELD score ranking: to have severe and sudden onset of end-state liver disease and only have a few days to live without transplant (called 1A) or to be very sick, chronically ill and under 18 years of age (called 1B). For example, if you had cancer inside the organ to be transplanted, you could be deemed 1B. Those in 1A or 1B received the first available livers that matched their needs. Patients with cancer that had migrated outside of the liver, could never be transplanted and would not be able to be listed again – ever.
Adam received his transplant in the first few minutes after midnight of April 3. Two weeks later, the pathology study from his original liver and one or two nearby lymph nodes came back as positive for lymphoma. If the diagnosis would have come before the transplant operation, Adam would have been removed from the transplant list. After that, he would not have been a good transplant candidate.
More treatable cancers. When Adam was first diagnosed with PSC, I had stopped at Safeway to buy milk on my way home from work. In my parked car, I read about his liver disease on my phone. I had not felt brave enough to do this until then. I learned that patients with PSC had a 10-15% higher than normal chance of cholangiocarcinoma, or cancer of the bile ducts. Over the lifetime of these PSC patients, 20% are diagnosed with cholangiocarcinoma. And only 15% of patients with bile duct cancer caught in early stages lived 5 years. I have to admit, this was one of the times I cried.
Due to his rapid decline, Adam’s doctors at both Mayo and PCH thought Adam had cholangiocarcinoma. He went through every test, but all came back negative. You see, Adam had lymphoma, which was not what they were looking for. As Adam’s hepatologist explained, lymphoma is more treatable than bile duct cancer. Those with diffuse large B-cell lymphoma have a 64% chance of being alive 5 years later. So, Adam had PSC, which is a rare disease, but his cancer was the most common form of non-Hodgkin lymphoma. The two did not go together. In fact, the combination of PSC and this form of lymphoma was relatively unheard of, with only 5 documented cases worldwide.
Transplanting during a pandemic. Since taking care of a transplanted organ means you must take medications that suppress the immune system. Adam will be immunocompromised for the rest of his life. No one knows how COVID-19 will affect them. Those at high risk, like Adam, have to be especially careful.
Upon returning home from the hospital, it dawned on me that whereas before COVID-19 post-transplant Adam would have been the only one practicing social distancing and wearing a mask, the pandemic meant that everyone should be doing these same things. We were in no way happy to see COVID spread, but it felt as though Adam would not be missing much during recovery. Adam’s friends – mostly recent virtual high-school graduates – were not really doing that much. Many businesses were closed, and social gatherings were at least discouraged.
Friends and funds. Friends and neighbors reached out to see how Adam was feeling and to ask what they could do to help. Our neighbor brought over dinners and regularly dropped off coffees for Adam. When Adam returned home after transplant. His friends held a parade in front of our home. About 20 honking cars slowly made their way down our street, and friends waved signs with sayings like “Chicks dig scars!”
Since Adam is going to need prescriptions, tests, insurance and hospitalizations not just at transplant, but for 60+ years of his life, our PCH social worker suggested we contact the Children’s Organ Transplant Association to start a fundraising campaign. Currently, we are halfway to our goal of raising $60,000 for COTA and have great prospects for completing our campaign with a golf tournament, set for early May.
As you can see, Adam is very lucky. His infectious gratitude during this difficult time made me want to correct the anesthesiologist. Adam has a precious new liver, expected to last his lifetime. He is cancer free. And through all of this turmoil, Adam has grown into a man who appreciates his life and his new-found health.