Supporting Families During Pediatric Transplant Journeys

In May 2022, my son, Al, broke his hand while playing soccer on a Saturday. On Sunday, he complained about a gastrointestinal (GI) bug and by Monday, Al said he had thrown up blood. It didn’t seem particularly likely to me, but we took him to the ED. We were told his labs looked fine; he was given an antiemetic and sent home. In mid-August, we had a follow-up with his PCP, who heard a murmur that she had not heard before and sent us to see a cardiologist the next day. This is when everything began to unravel. The cardiologist did an echo and said that while Al’s heart looked OK structurally, something was not. I was told to take Al for labs first thing the next morning. I was pulling into the parking garage at work when my phone rang. Al had distinct clinical pancytopenia; all blood cell type results were markedly reduced, and his hemoglobin was 3. By that afternoon, Al was receiving multiple blood transfusions in the ED at Children’s Hospital, while we waited for a bed in the oncology unit. The healthcare staff were all thinking the same thing: leukemia, and a bone marrow biopsy was scheduled for the next day.

Importance of Family Support

It was categorically the scariest news that I had ever received as a parent. I called my mom on my way back to school to get Al before heading to the hospital. I originally told her: “Don’t come. We’ll be in the ED forever. I will call you when we get settled.” But after leukemia was mentioned as the most likely outcome three or four times, I changed my tune. I called again and said, “I need you now.” Thankfully, they had already come and were in the parking lot.

I cry at Hallmark commercials, I cry when something touches my heart (which is often), I cry when I’m relieved, I cry when things are beautiful. But on this day, I shut down emotionally until late in the afternoon when I stood in the middle of a busy waiting room with my brother, and we both just sobbed. How was this even possible?

Finding the Right Diagnosis

Over the next two days, a barrage of tests was run and we went home to wait for the results. We then received news that it was not leukemia but more likely myelodysplastic syndrome (MDS), which is often a precursor to leukemia. We then entered a season of visiting a variety of specialists. The hematology-oncology doctor said he had enough information to diagnose Al with MDS and wanted to put Al on the bone marrow transplant list right away, as chemotherapy and a bone marrow transplant were, in his mind, on the horizon. Al endured months of testing and follow-up appointments with infectious disease and immunology. In October, Al was finally improving markedly, but we had no clear diagnosis, which led to the recommendation that Al undergo a full GI workup.

We met with GI and were told the enlargement of Al’s liver and spleen was possibly due to schistosomiasis from drinking unclean water, a common issue in Ethiopia. Al was born in Ethiopia and diagnosed with schistosomiasis before we adopted him. We were aware at the time of the adoption that he had enlargement of both the liver and spleen resulting from schistosomiasis infection. The GI doctor ordered a CT scan in November, and then we waited. The days dragged on with no news. Finally, on my birthday, which is cemented in my mind, I was told that Al’s liver was likely cirrhotic, and he would almost certainly need a liver transplant.

Understanding Pediatric Transplantation and the Diagnosis

This diagnosis triggered my deepest fears. I grew up with a wonderful friend who had been diagnosed with a cirrhotic liver, too. He had also received a transplant, in fact two transplants, before succumbing to complications resulting from the transplant attempts. He was only in his 30s. On this day, the news that Al would likely need a liver transplant was interpreted in my mind as, “You are going to lose him in the most painful and protracted way imaginable.”

Al was scheduled for a liver biopsy three days after Christmas. When we got home that night, Al had a massive GI bleed and was admitted emergently to the ICU. Once he was stable, Al was taken for an esophagogastroduodenoscopy. I sat in the procedure waiting area like I had been carved from stone. Al had esophageal varices. Many of them, we were told, would require serial bandings to decrease the life-threatening risk of another bleed. For the first time, there was a certainty of what had caused all of Al’s issues: portal hypertension and esophageal varices.

My memories from that day are not sharp. I was exhausted. I was terrified and felt outside of my own body. In a space of relief with the certainty of what had caused all the issues, and in grief at the uncertainty that remained, I broke down and sobbed in the waiting room. The GI doctor and my mom took me to the procedural waiting room trying to comfort me. Through my sobs, I shared the story of my friend who died after a second liver transplant and all my fears that my son would share the same fate. And beside me, holding my hand, was a woman with kind eyes who looked at me over the top of her mask with a new understanding of our pain. She would become our liver specialist and our greatest advocate and ally for the journey ahead.

The Need for Pediatric Organ Donation

After months of serial scopes and a second bleeding episode requiring ICU admission, Al was placed on the transplant list on Sept. 7, 2023. On Jan. 13, 2024, at 9 a.m., I received a call that there was a match! We had to wait 13 hours for the liver to arrive, and Al was rushed into surgery at 10:30 p.m. I knew that even as we received the answer to our prayers, another family was planning a funeral. The depth of gratitude that I feel for this gift, and the depth of grief that I have felt for a family unknown to us, is nearly impossible for me to talk about even with some passage of time. The transplant itself went beautifully.

The post-transplant landscape has been one of appointments, lab draws and new medications. All of this, while also learning to navigate homeschooling and remote work. Our post-transplant journey has not been without complications, not without new uncertainty and fear at times. In general, the recovery has been miraculous. It’s been a long journey, and the story is still unfolding. I’d like to share some of what we learned along the way.

How Nurses Can Support the Family of a Transplant Patient

Nurses can support the family of a transplant patient in several ways. Here are some of the ways my family was supported during this really tough time in our lives.

• 1Advocate, advocate, advocate. Make the family part of your team and include them in decision making. Teach your patients to advocate for the care team that is the right fit for them. Encourage them to share their concerns and ask questions when they don’t understand. Make sure they know they do not have to do it all alone. The healthcare team wants to engage and partner with them to achieve the best outcome.

• 2Learn about trauma informed care. The kindness I saw in our healthcare provider’s eyes was priceless. Those eyes saw not only the trauma and the fear but helped us reframe it in a healthier way. If you have developed a connection and longevity with the patient and family along the journey, offer your ear. Your compassion can be viewed as priceless during such a critical time.

• 3Understand healthcare providers as patients/families of patients. For the healthcare provider who suddenly finds themself as the family member of a patient, it can be difficult to separate their personal relationship with the patient from their professional knowledge, experience and expertise. In this space I found myself knowing that my clinical expertise could function as a safety valve: “No you can’t give him ibuprofen; he has varices,” while at the same time wanting so desperately to just be mom. The only part of the healthcare team that I wanted to exist in was the grieving parent wanting to be informed of options and all the ramifications and potential outcomes as a partner in decision making rather than feeling like I needed to lean into my own professional experience under so much stress. During the hospitalizations, I appreciated the staff who didn’t hear that I am a nurse and say, “Oh you got this” … I don’t, I don’t “got” this.

• 4Involve Social Service/Chaplain Services early in the admission to aid with community support. One of the most profoundly overwhelming aspects of having a child with complex medical needs is the loneliness of navigating the healthcare system. As a single parent with a very close/supportive family and community, I was struck by how lonely it can feel to be solely or mostly responsible for the myriad tasks that must be dealt with, such as appointments, medication management and navigating obstacles with insurance companies. Case managers, social workers and chaplains can help in a very tangible way to fill the gaps in those responsibilities and alleviate some of the weight. Show Kindness: Small acts of kindness are immeasurable. Patients and families need encouragement and good news, but when those things are in short supply during times of critical illness, the little things matter. Being offered toothpaste, an extra blanket, a bed to sleep in or showing me where to shower meant so much to me. Encourage families to plan to go home once in a while, so they can get some uninterrupted sleep in their own beds. These acts of kindness will mean more to the exhausted, weary caregiver than you can imagine. In all my years of nursing, I absolutely never knew, but I can tell you now those simple things are some of the greatest kindnesses imaginable.

• 5Encourage families to share their story when they are ready. As part of my role at the hospital, I attend new grad RN residency classes. Over the last year, I have started to share how having a great nurse/provider who is attentive to details instills trust in me as a parent, allowing me to take my hands off the wheel for just a minute.

How will you provide optimal support to families of pediatric liver transplant recipients?