When Trey Kearns was two, he had a terrible itch. His itch wasn’t like yours – it was inside the body, meaning it’s an itch you can’t scratch. They came to learn it was because he had PFIC, a rare disorder known for its characteristic itch due to liver failure. Unfamiliar with the disease, they tried a slew of options to find what worked and what didn’t. Vitamins D, E, and K, then A; and yet his symptoms continued to progress as he entered kindergarten. His mother Tara explained,
“When he was in kindergarten, he started to be able to articulate his itch a lot more. He would ask us why he was so itchy, and he wished he could not be anymore. The more he was talking about it, we decided to explore with his doctor what his options were.”
Progressive Familial Intrahepatic Cholestasis
Progressive familial intrahepatic cholestasis (PFIC) is a type of rare genetic disease effecting the liver and bile. The symptoms begin in infancy, during which patients tend to experience jaundice, cholestasis, slower growth, difficulty absorbing vitamins, broken bones, and a characteristic itch due to a failing liver. Many patients use medicines to help or pursue a liver transplant, which resolves the bile issues but leaves patients immunocompromised.
Seeking Surgical Solutions
His continued health issues led them to pursue an internal biliary inversion surgery that successfully took his itch from a 10 to a 3. After about a year though he began to experience additional complications and increased trouble with vitamin absorption, so they set out with doctors to find answers and discovered his liver was failing. It was decided that Trey needed to begin the process of being listed for a liver transplant. Tara said, “After 20 days on the list, we got a donor.”
“In the beginning, I remember feeling really, really devastated. It felt so big and so uncertain. I’m a person that naturally asks a lot of questions, and I felt I didn’t really have a place for that, a place to put that energy.”
Finding a New Normal
With time they adjusted, but much of it they figured out on their own. She said, “It became so normal for us. You just do it because your kid needs them, but you didn’t realize how much you’re doing.” She explained that before she found a community of PFIC families, she had adapted to not having others to share the experience with:
“It was really nice when I did find a group of parents that also had kids with PFIC. I didn’t realize how much I missed having it until I had it.”
A few years ago Tara took control of a PFIC website along with two other moms, Emily and Melanie, when the prior administrator wanted to relinquish responsibility over the site. They grew it into something much bigger than before. She said,
“We decided the three of us would take this on together and it really just evolved from there. We had a chance to share our hearts and how profoundly this had affected each of us, and how much we wish we had known when our children were first diagnosed. We knew we had more information now, and felt a little more confident, but we thought- ‘Man, what if we had information, what if we’d had news, community, what if?’ So we thought, well, we could make a non-profit?”
So they did.
The PFIC Network
Together, the moms made the PFIC Network into their non-profit. They equipped it with a brand new website, have hosted a PFIC conference, and have another one in the works. There’s a parent ambassador program in which parents who have been in the community longer mentor parents with more recently diagnosed children. They also have included medical professionals who provide further guidance on a medical advisory board. The moms have been able to weave together a community space with resources that they had longed for since their children first received their diagnosis. She said,
“It was cool because it started out as a dream and something we all wished we’d had in our early phases of diagnosis, and we just went with it. We were three moms that had this passion, and we decided to figure it out.”
The Pandemic and An Immunocompromised Son
When the pandemic began to loom shortly after his transplant, the family’s already strict germ regime became that much stricter. They washed their hands more often and began to screen companionship more closely to protect Trey’s safety.
“In the beginning, we were definitely scared because we didn’t know what would unfold. It’s not like I ever stopped being scared, but we settled into our quarantine. We turned off the news and we just spent time together.We worked from home, and the kids settled into distance learning, and just enjoyed each other.”
They live in a tight community in rural Maryland, and Tara explained how supportive the community has been. Trey was able to continue baseball because his teammates all learned the safety code for him, quickly adapting to masks. She said, “His teammates don’t sit in a dugout. They all have folding chairs and they all put them 6 feet apart and they know they can’t be in his space and all that.”
The PFIC Community Through the Pandemic
While the family has been making their space from the in-person community, the PFIC Network is just as connected. The families are all grappling with the same questions- how to engage in life in a world with coronavirus. They host an ongoing conversation where parents ask questions, share advice, and are provided with answers from a medical advisory board when a question seems too big. She said,
“Right now, what I’m seeing in our PFIC community is a lot of uncertainty. Because there’s not one answer for all of our kids. Some are immunocompromised from transplants, others are in trials or on medications that don’t leave them with the same sort of immunosuppression as transplants do. There doesn’t seem to be any universal thought, we’re all just uncertain.”
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With the school year on the horizon, her family is focused on keeping themselves healthy to protect Trey. She explained that learning to adjust and do what you need to do has always been part of their medical odyssey. Tara said,
“It’s a weird experience just because it has definitely changed me in ways that I don’t know if I’ll ever be able to articulate. When you end up caring for a kid who is medically complex, survival becomes part of your normal.”
As much work and dedication as Tara puts into Trey’s health and building the community, she is grateful for all of the blessings that the disease brought into their lives. She said,
“We never realized exactly how healthy he could be, and he’s incredibly healthy now. It’s so cool to just watch him grow, watch him get back to doing things that he always loved, but with his brand new body that’s stronger and healthier than we ever thought it could be.”
