Montgomery, Ohio
SHANE DIGIOVANNA IS WORRIED. Like so many other Americans, the 27-year-old relatively recent college graduate is concerned about what Elon Musk, Donald Trump, and RFK Jr. are doing to the government. For him, it’s not just a matter of effective government, or even democracy vs. oligarchy or autocracy. For Shane, the dismantling of the government could be a matter of life and death.

Shane has a severe form of recessive dystrophic epidermolysis bullosa, or RDEB, a genetic condition that many parents and even most medical providers don’t initially notice because it is so rare, affecting an estimated 3.3 out of every million people.

“ Basically, I am missing a protein that acts as the glue between the different layers of the skin,” DiGiovanna explains. “So that means my skin shears very easily, comes off very easily, and blisters very easily. . . . I’m bandaged pretty much everywhere. . . . I’m missing the majority of my skin, basically.”

Most people take their skin for granted or even resent it for blemishes or imperfections. Living without much of his, DiGiovanna suffers from anemia—a side effect of constant bleeding—as well as gastrointestinal complications from the dermal lining of his mouth and esophagus.

“I love eating,” he says, “but literally, I’ll have a nice meal, then wipe my mouth, and it’ll be blood.”

Patsy DiGiovanna, Shane’s mother, adds, “When he was a baby, before I could feed him, I had to take long needles and pop blisters in the back of his mouth, which you can imagine with a newborn, because they would fill up with fluid and he couldn’t eat.”

“There are several gene therapies and other treatments in trials right now,” Shane told The Bulwark, “and I’m very worried about the future of RDEB patients. RFK Jr. and Musk could devastate the next decade of research and development of a cure for RDEB—my holy grail. . . . Just keep in mind that the weak and marginalized include infants and children who are missing most of their skin and suffering through extreme and utterly pointless pain.”

I met up with Shane and Patsy at their home near Cincinnati. Shane’s first comment on my tape recorder was: “I started reading the Economist when I was 12 or 13.” He’s a big reader, a news junky—he’s been part of the Bulwark subscriber community for half a decade—and a lover of all things space: “I read Cosmos by Carl Sagan when I was 7.” Patsy shares, proudly, “He was reading at a college level in second grade.”

She adds that the news business and love of reading runs in the blood: Her father headed up the ABC affiliate in Boston when she was a child.

Freshly bandaged and in good spirits, Shane tells me he’s already picked out some outfits for photos. “ I love having photos taken of myself. I love myself. I love my face.”

Planning ahead is common for Shane: On his beloved iPad, he has an agenda typed out that appears to be a page and a half long, which he did one letter at a time through a small connection to his skin through a bandage. He has to be a meticulous planner: If you don’t pay attention, RDEB can easily kill you.

And it doesn’t help that Shane is “profoundly deaf,” completely unrelated to RDEB. He’s had cochlear implants since he was a child. “Depending on how you look at it, I either won or lost the genetic lottery.”

SHANE’S LIFE SO FAR ISN’T SO MUCH a miracle as a series of miracles. The DiGiovannas moved to Cincinnati from North Carolina when he was a toddler for his father’s work. It was just a coincidence that their new home boasted the top pediatric hospital in the country, Cincinnati Children’s. Patsy recalls spending two and a half years in Charlotte telling doctors “I think he’s deaf” to no avail—and then, visiting the ENT department Cincinnati Children’s, mentioning “what I was thinking about Shane’s hearing loss” and the doctors immediately keying in to it being auditory neuropathy.

“Then I was like, ‘That worked out well, let me try dermatology.’ Little did I know the world’s biggest expert on Shane’s condition is here.”

That expert, with a name out of Dickens, is Dr. Anne Lucky, who, before her recent retirement, built the interdisciplinary epidermolysis bullosa center at Cincinnati Children’s, one of only three like it in the country. It now serves 250 patients, 80 of whom, like Shane, have RDEB.

The hospital also connected Shane’s family with Ohio Valley Voices, a school for the deaf.

“It’s an oral deaf school, so no sign language,” Shane explains. Instead, it trains children with cochlear implants. “It’s not like you can just put the implant on the child or the person and expect them to hear completely all of a sudden.” He says when he got his first implant, he scarcely knew what language was. The education that helped him become “a member of the hearing world” began at “not even square one,” with much of the early instruction focused on “what language is . . . how to listen, how to interpret . . . how to speak.”

The school is “a magical place.” Patsy says.

Shane agrees. “It was some of the best years of my life.”

Within a month of starting at Ohio Valley Voices, Shane was using three syllable words, Patsy says. Shane chimes in: “My first word was airplane.”

School was tough for Shane—starting with the mere physical realities of being in a classroom and doing schoolwork. “The idea was, you know, that I could hold a pencil at school and participate in school. Hold a cup.”

Shane’s condition has made his fingers essentially useless, an inevitability the DiGiovannas discovered only after a series of painful surgeries. “ They would . . . basically take [each hand] apart and put it back together.”

Patsy adds, with a visible sense of shared pain, “ The healing process was a complete nightmare. And then, as we discovered, ‘Oh, you’re just gonna go back to where they were.’”

In total, Shane has had about 150 surgeries in his life—an average of more than five per year. But some years are worse than others. In 2023, he had surgery nearly every month.

That didn’t stop him from achieving some remarkable things. In high school, Shane gave a TEDx talk about his condition (and his love of space). While his dream of being an astronaut is out of reach, Shane has lived out a few star-gazer’s fantasies. He’s met famous astronauts, been given VIP tours of NASA, seen various Mars-program vehicles, and he even got to take a special “weightless” flight thanks to the Make-a-Wish Foundation.

Shane was also able to earn a degree from Rice University in economics, even attending classes in person until the pandemic hit. He is one of the first people with severe EB to go to college, and as Shane observes, likely one of “the first to go away to college.”

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“I was able to be relatively independent,” Shane beams. “Go to classes, went to a lot of parties. I was quite the party animal. I found a love of jello shots,” Shane explains, giving me a knowing glance. Patty interjects that a series of nurses and emergency backups and last-minute plane tickets made this all possible.

“ My health has declined drastically since that, but for a few short years, I got something that very few people with severe EB have, which is freedom.” But it wasn’t long after this sun-lit period that Shane got his first cancer diagnosis.

Squamous cell carcinoma is usually highly treatable, but for someone with Shane’s skin condition, it’s far more serious. A 2023 study found that “by midadulthood up to 90% of patients with this subtype of EB will have died of metastatic SCC, despite aggressive surgical resection.”

Shane underwent invasive surgery to remove the cancer just as the world shut down and Rice switched to online classes, which allowed him to recuperate and study from home. While he was on immunotherapy for the first cancer, another developed on his foot. Seven more surgeries followed. It almost killed him.

After graduating, Shane held an internship at Procter and Gamble, and he worked for Cincinnati Children’s in donor relations. But “the first cancer hit me like a truck” Shane said, “and ever since then, it’s like every time I get another cancer, it’s another truck hitting me at full speed.”

Now Shane is homebound, where he receives round-the-clock care from his mom and a dedicated nursing team.

“ If I’m feeling really good, guess what I might do?” Shane asks. “I might go to the grocery store. Wow, that might be big!” he jokes with gentle sarcasm. “Jungle Jim’s,” a regional specialty grocer, “is probably the peak of a really good-feeling day.” I ask Shane what he would say to our former neighbor, Vice President JD Vance, if he were to run into him at the Jungle Jim’s, which Vance is known to frequent.

Shane’s mind immediately goes to one of his nurses, an immigrant who’s now an American citizen. He describes him as “a wonderful man, heart of gold, total family man. Loves his family. Loves his kids. Incredible nurse, great all-American man.”

“But, I’m so worried . . . because of what JD Vance did in Springfield,” Shane says, referring to the then–vice presidential candidate’s defense of the lie that immigrants were stealing and eating dogs and cats in the Ohio town. His concern is that Vance will “target [this community] in Cincinnati, and there is one, enough for there to be a couple of churches that cater towards people from [there].”

“So maybe that’s what I would say to JD Vance is: ‘What you did in Springfield—I’m terrified that you’re going to do that to one of my nurses . . . who’s just a simple, good man. You know, you destroyed these people’s lives for no good reason. And I’m worried you’re going to do that to people I know. And I can’t trust you because of that.’”

We’ve been talking for a few hours by this point, and Patsy explains that Shane has probably been more active today to see us than he would be on a normal day.

“My life is very challenging these days, I’m on heavy narcotics, and I’m still sitting here in pain right now,” Shane says. But he wants to talk a little more about Trump and Musk and RFK Jr. and helping more kids like him.

Shane’s medical care costs about $3.5 million every year, according to Patsy. His family pays what they can. Insurance covers a lot of it. Medicaid covers the rest.

One of his medicines, the gene therapy Vyjuvek, costs $24,000 per week—“the price of a used car,” Shane deadpans. Shane participated in the open label extended study for this drug for a year, and the FDA approved it in 2023. But RDEB is an “orphan disease”: It’s so rare that there’s very little research into it or its possible treatments. There’s no way drug companies will make money on RDEB treatments; there just aren’t enough patients. Each study, each trial is a gem.

Which is one of the things that makes the prospect of Trump, Musk, and RFK Jr. so dangerous for people like Shane.

“ I would say there’s a lot of trepidation about what Musk is going to do next. I do worry about, you know, Medicaid and Medicare being targeted, or the medical trials being messed up because RFK is a kook. I would say, you know, so nothing’s been negatively impacted yet, but I would say it’s one more concern, one more worry that’s affecting a community that has far too many worries already.”

“I love Big Pharma. It keeps me alive,” Shane proclaims. “I’m worried that [RFK] is going to disrupt all of these trials in the FDA.  I’m worried that funding from the NIH is going to be cut, into basic EB research. And grants that go to researchers across the country who are working on EB—not for the money, because once again, there’s no money in this. EB is so rare.” He’s not just thinking of patients. He also mentions “the poor people at Stanford,” one of the major drivers of EB research, “who have been working on this for decades, in part because the science is really, really hard, but also because there’s no money.”

SHANE IS TOO INTELLIGENT, resourceful, and energetic just to earn his degree and rest on his laurels. Acutely grateful for the miracles—and the people and money and hard work—that have made his life possible, he’s paying it forward.

When Shane was little, his aunt gave him a stuffed dog, which he named Doggie. Doggie accompanied him for his first surgery—and every one since—and is now the mascot for Shane’s charity, Doggie’s Friends, an endowment at Cincinnati Children’s to provide stuffed animals to kids undergoing surgery.

In a fundraising appeal, Shane writes:

My body is declining, and Doggie is more than half made of patches now, but he still gives me just as much love and comfort as he did when I was a child. When I hold Doggie, I know that I’m okay, even when nothing is okay. As Winnie-the-Pooh once said, “Sometimes the smallest things take up the most room in your heart.”

I created Doggie’s Friends because I want every child at Cincinnati Children’s whose journey is similar to my own to have a companion who can give them as much strength and love as Doggie has for me. This is what I want to leave behind.

So far, Doggie’s Friends has raised more than $100,000 in just over a year, ensuring many years of future Doggies. And Shane is still looking to raise more.

Leaving behind an endowed Doggie’s Friends is a legacy goal for Shane, who despite needing constant help from concentric rings of people—or perhaps because he needs so much help—is constantly thinking of others. Of his family. Of his fellow patients. Of his nurses and doctors. Of medical researchers thousands of miles away whose names he’ll never know.

“I’m alive,” Shane says. “I would like to continue staying alive . . . but it takes an enormous network of people—doctors, nurses, insurance, whatever—to keep me alive.”

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