In the summer of 1996, James Perri was 24 years old. And he was invincible.
In high school they called him the Iceman. Stoic. Unshakeable. The kind of guy who inspires loyalty, the kind of guy who has more than one best friend.
Ask the best friends from Manalapan, New Jersey: Pat Ronan, Mike Masone ’94, Pat Hanlon ’94. First bound together by the baseball games in the Perri backyard. Kids in the same classes throughout grade school. Then high school lab partners. Friends so close they’re family.
Or ask Ron Mnieckowski ’94, who at Notre Dame bonded with Perri and Hanlon, the “Jersey ruffians,” or Mike Towers ’94, whom Perri and Hanlon nicknamed “Hammer,” as in MC, because of his dancing ability (or lack thereof, depending on who’s telling the story).
They love James Perri ’94: Superhero tall, lantern-jawed, genuine Jersey tough guy with a heart of gold. The quiet genius, the guy who gets an A on the most challenging organic chemistry exam at Notre Dame. Invincible.
So Perri ignored the dizzy spells he encountered as a student at Rutgers Robert Wood Johnson Medical School. He ignored them while taking the exhausting board exams that determined his future as a doctor. But then the dizzy spells came six or seven times a day, each lasting about a minute, while he studied or drove or played basketball. And he started hearing music and voices inside his head.
So Perri drove up to the medical school and talked to a doctor, who referred him to an ear, nose and throat specialist, who ordered an MRI. After the test, as Perri left the office, the MRI tech said, “Good luck.” The way he said it had Perri, the Iceman, driving back to Manalapan in tears.
The day after Perri received his grades on his board exams — outstanding, one of the top 200 scores in America — the doctor called back. We got the MRI results, Jim. Why don’t you come in? And Perri drove up by himself, not yet knowing that doctors ask patients to return to the office only when the news is not good.
So he was alone when the doctor showed him the MRI results, pointed to the glowing mass deep within the right temporal lobe of his brain, told him about something called oligodendroglioma. He was alone on the drive back home, sobbing into the steering wheel. Alone, until he walked into the house and said, “Mom, I have a brain tumor.”
Somehow, in the midst of the shock, tears and a mother’s fury that her son heard the diagnosis by himself, Maureen Perri held her second child tight and said, “Don’t worry, we’ll get through this.” Then, during the evening’s family dinner, sister Amy — normally cheerful, quick to laugh — stood at the table and shouted: “How can you just sit there eating dinner? My brother has a brain tumor!” Then she broke down and hugged her brother — James Perri, the Iceman, invincible no longer.
The tumor begins with a single cell. And what a cell it is.
Oligodendrocytes — oh-lee-go-DEN-dro-sights — exist only in the brain and spinal cord. The name comes from Greek: combine oligos (“a few”) with dendron (“tree”) and cytion (“box” or “cell”) and you get “cells with a few branches.” It’s actually more than a few: A single oligodendrocyte can sprout a dense network of up to 50 connecting arms, growing from neuron to neuron like a delicate rose climbing a garden lattice. Oligodendrocytes warmly envelop neurons in a protective coating called a myelin sheath. The myelin sheath nourishes neurons, helping to transmit their messages — thoughts, feelings, sensations — throughout the body.
In exceptional cases, for reasons unknown, an oligodendrocyte loses its way. The transformation could result from the over-expression of something called platelet-derived growth factor receptor (PDGF-R), which regulates how cells grow, proliferate and develop. When one of these oligodendrocytes loses its way, it becomes something else entirely. That something else grows, slowly but uncontrollably, where healthy brain cells once thrived. That something else is oligodendroglioma.
Even by brain cancer standards, “oligo” is especially rare: A 2012 study found only 1.9 percent of all brain and central nervous system tumors are oligodendroglioma. (Some rough math: Out of almost 24,000 new brain cancer diagnoses in 2014, about 450 will be oligo.) And unlike more common forms of brain cancer, which result from lifelong accumulations of genetic glitches, oligo tends to disproportionately affect young people from 20 to 30 years old.
From one cell, oligo begins to grow within the brain. If healthy oligodendrocytes are like delicate roses climbing over a lattice of neurons, the cancerous oligo is a choking vine: In its metastatic stages, it interweaves tendrils through healthy brain tissue so tightly that removing the tumor means removing healthy tissue as well — essentially bulldozing the lattice and the roses with it. Then irradiating the vines. Then spraying the vines with chemicals. And hoping that there aren’t any vine roots alive to grow back.
When James Perri returned to medical school in the fall of 1996, some jaws dropped.
“We thought you were going out for something like a knee surgery,” one of his classmates said, eyeing Perri’s shaved head and the neat scar snaking above his right ear. “We didn’t realize you were getting your brain cut open.”
Perri had gotten his brain cut open. Not long after Perri’s initial diagnosis, Dr. G. Rees Cosgrove, a veteran neurosurgeon at Massachusetts General Hospital, had sawed through a portion of Perri’s skull, pulled the section back and literally scooped out a small chunk of brain tissue where the tumor had grown. Cosgrove (carefully) resected the vine, and the roses and the lattice with it. Five weeks later, Perri went back to school. No big deal, he thought. It’s done.
“When you get the surgery,” Perri says, “part of you just wants to say, ‘Take out as much as you need to. Keep going. Take out extra.’ But the question is: Do you want to go on without use of the left side of your face? Without your right arm? Maybe that helps you live longer, maybe it doesn’t. They take out whatever they think they can.”
So here was James Perri, only five weeks removed from highly invasive brain surgery, working again at the obstetrics rotation he started while suffering from oligo-induced temporal lobe seizures.
Iceman.
Years later, James Perri meets a man who reminds him of that surgery.
The man had passed out in a supermarket, and when he arrives at the hospital, Perri examines him and orders a CAT scan in case he had injured his head in the fall. The other ER doctors say he suffered a stroke, but, Perri thinks that he wouldn’t have passed out from a stroke. No. Something else.
So he admits this man to the hospital for an MRI. Another test, to be administered by someone else. A few days later, Perri hears the news. He was right. It was no stroke. The man had suffered a seizure caused by oligodendroglioma. And, it turns out, he’s Perri’s neighbor. He lives right down the block.
What are the chances? What are the odds that this patient would live half a mile from Perri’s house, that he would arrive at the hospital where Perri works, that Perri would pick up his case, that it would point to the same exceptional diagnosis Perri suffered? Could it have been a coincidence?
His mother calls these moments Godwinks. There are no coincidences, she says. Only God-incidences.
For a time in that autumn of 1996, Perri thought he was back to normal. Cured, even. Ask his med-school friends. Frank Nezu, who helped him put the pieces of a social life back together. Charlie Getz, his workout buddy, his pickup basketball guy, who helped guide him back into a medical student’s normal routine.
Normal. What was normal? Every three months Perri had to visit Memorial Sloan Kettering for surveillance MRIs of his brain as doctors periodically checked for the return of the disease that nobody had heard of, but that doctors said was incurable. And slowly, fitfully, Perri realized that the oligo would return someday.
In the midst of constant reminders of his own mortality, James Perri fell in love.
Nancy DeFrancesco is petite and pretty, with Italian features reminiscent of a vintage Vespa ad. Underneath her sanguine exterior, she exudes steeliness, the street-smart tenacity of a Philadelphia-born daughter of blue-collar Sicilian immigrants.
In 2000, she was an insurance rep at Blue Cross, and James was grinding his way through an internship at Albert Einstein College of Medicine. So naturally their first conversation in some Philadelphia bar was essentially a two-hour debate about healthcare. Star-crossed.
Then, a few days later, her friend called:
“Nancy. Remember that guy you were talking to? He’s gonna call you.”
“Okay, that’s fine.”
“No. Now.” And her friend hung up.
About five seconds later the phone rang. It was that roguishly handsome med student from New Jersey, and he had things to talk about besides healthcare.
They kept talking, even after he moved up to New York for an internship at New York University. Old-time buddies Pat Hanlon, Mike Masone and Ron Mnieckowski were in Manhattan: Notre Dame guys reunited. But still he’d journey to see her: Twelve bucks and two and a half hours — NJ Transit, then Septa — and he’d be back with her in Philly, or on the beach at the Jersey shore. Back with her, away from the dark hospital room where — interning in radiology — he’d stage MRIs of brain cancer, thinking, That’s gonna be my brain. That’s gonna be me someday. The Iceman was strong, but looking at brain cancer all day?
Then, during one of his own surveillance visits, the doctors at Memorial took a look at Perri’s MRI and called him back to the office. It seemed there was a recurrence, they said. Something small was up there in his right temporal lobe.
He went into a tailspin. How could he get married now? It’s not fair for me to waste your time, he told Nancy. It’s not the right thing to do. Why even continue to pursue a medical career? And kids — how could he have kids? But his doctor called him back, said it could be nothing. Frank Nezu’s father, a psychologist specializing in physicians, helped give Perri some perspective.
“If you live like that, constantly thinking the end’s around the corner, it actually makes you more stressed and depressed,” Perri says. “You have to find a happy medium. Yeah, I don’t want to waste time, but I also don’t want to live every day like [death] is right around the corner, because then you’re just on pins and needles the whole time.”
So in 2000 he decided to start living like the oligo wasn’t coming back. He switched to an emergency medicine residency in Delaware, where he wouldn’t have to stage cancer tests all day. Where he could be close to the woman he loved.
And he asked Nancy DeFrancesco to marry him. She said yes, in sickness and in health.
Their wedding song was “Let’s Stay Together.”
For 12 years the cancer stayed silent, stayed mostly undetected on the surveillance MRIs at Memorial. For Nancy, maybe the cancer was an abstraction. Maybe it was a ghost story she didn’t really believe.
Twelve years. The Perris in 2008, a thriving young family in the picturesque Charlotte suburb of Huntersville, North Carolina. A house with the flattest backyard James and Nancy could find, with just the right number of trees, so the kids could play ball like he did growing up in New Jersey. “The first house,” he says, “you buy for your kids.”
Him, one of the most productive emergency room surgeons in the state. Her, a fitness instructor and a full-time mom to little Jack and Sofia. Somewhere there’s a picture of the young family, and it looks like the kind of picture that comes inside frames at the store. Good genes, that family. Mostly.
July 11, 2008. A gorgeous afternoon, the sky an impossible blue, Nancy outside, watching the kids toddling around the driveway. James driving up, parking the car, stepping out.
When James starts crying right there in the driveway, Nancy knows. She sees the ghost. The ghost is real.
And life changes, again and forever.
Because of oligo’s rarity, the disease still holds some mystery. But in the 12 years between Perri’s diagnosis and his oligo’s recurrence, researchers have discovered a wealth of valuable information about treating it.
They’ve learned that treatment is more effective if a tumor exhibits either (or both) of two favorable genetic mutations. Perri’s tumor has one of those favorable mutations. Aggressive forms demand immediate attention. If the tumor spreads to the frontal lobe, it could affect logic and emotion. Another part, vision. Another part, speech. But removing the tumor is a delicate and potentially dangerous procedure. This being brain cancer, surgeons are careful to remove only what is absolutely necessary, since total removal can be even worse than cancerous tissue.
So doctors often treat with some combination of chemotherapy and radiation. A new Merck drug called Temodar is indicated to treat oligos with less toxicity than older drugs. There is some debate whether Temodar is the best — some oncologists point to an older drug called PCV — but conclusive trials could take 20 years. By that point, perhaps researchers will discover a new drug. Perhaps.
James and Nancy knew the tumor would return, but the recurrence still came as a shock. There were no symptoms, no warning, until a routine surveillance MRI showed a bright little spot that meant the oligo had returned.
“I see it as a science problem,” James says. “Statistics. I got the chromosomes. The same way I got chromosomes to be an intelligent person. . . . It would be disingenuous to say, ‘Why me?’ when I have such great parents, great siblings. Life’s not fair. The reality is that life is a lot of random events, plenty of which you don’t have control over. This being one of them. The problem is trying to get control over something that you don’t have control over, so you’re actually doing something and you’re not sitting helplessly, waiting.”
Five days of agonizing stress after the MRI results (What do we do? What’s the treatment?) he was in the office of Dr. Lisa M. DeAngelis, the co-executive director of Memorial Sloan Kettering’s Brain Tumor Center. DeAngelis had been James’s neuro-oncologist at Memorial since 2000, and had checked all of his surveillance MRIs since taking him on as a patient. For the Perris, DeAngelis’ catchphrase had always been, “All clear!”
But even in July 2008, DeAngelis had good news: The tumor was not an immediate risk. In fact, James wouldn’t need immediate surgery — just 18 months of chemotherapy. Temodar, once a month, at a dosage low enough so he could continue working. He’d even keep his hair, so everything would appear normal. He and Nancy told no one but their closest family and friends. The kids wouldn’t ask. The kids wouldn’t have to know.
James: “I think my dad said once, ‘My job is to raise you until you’re 18, and then you can be my friend. Until then, it doesn’t matter.’ . . . I always told Nancy that my goal was at least to get them through college. Kind of like my dad saying, ‘My job is to raise you.’ If I get them there, I’ve done my job raising them. Obviously I’d like to see them get married and have families and all, but at the very least I’ve done my job.
“My dad was the tough guy. But he got to see the final product. I won’t be around to see the final product. I don’t want my kids to resent me for that.”
When James downed his Temodar pill each month, he’d endure profound nausea and exhaustion. Then he’d go to work. No one at the hospital knew, except his close friend and fellow emergency room doctor David Rentz. Rentz had moved to North Carolina and joined the practice around the same time as James, and they were fast friends. Plus, being a bachelor for most of those years, Rentz frequented the Perri home for home-cooked Italian food before his night shifts.
For 18 months, Rentz marveled in secret as his friend logged long hours in the emergency room — James never once called in sick — and still outperformed most other doctors at the hospital. James would treat 20 patients in a shift, and he’d be sicker than most everyone. People would come into the ER never knowing that the man treating them was so chemo-sick that by the time he got home he could barely eat or say hello to his children or do anything besides crumple into an exhausted heap.
“I just wanted to feel normal,” he says. “And if everyone’s coming up to you, saying ‘Hey, how you doing? How you feeling?’ that’s not normal.”
Every three months, James’ checkup MRIs at Memorial showed that the Temodar seemed to be holding the tumor in check. But chemo took its toll. After a year and a half, James’ immune system was shot, his platelet count so low that he could start bleeding spontaneously. In March 2010, DeAngelis decided more of the tumor had to come out.
Dr. Kevin Judy, a rock-star neurosurgeon at the University of Pennsylvania, performed the surgery. For five hours on April 28, 2010, Judy and his team followed the pre-existing blueprint from James’ first surgery in 1996: open a hole, pull back the skull and claim as much of the tumor as possible from the pre-existing cavity in his brain. There was one “tricky spot.” A portion of the tumor had snaked around a major artery in James’ right temporal lobe. Nicking the artery even slightly could produce a clot, which could in turn lead to a catastrophic stroke. Otherwise, the surgery had been a success: Most of the tumor was gone, and the rest of James’ brain was intact.
James’ post-surgery pathology report was grim. The tumor had become malignant: grade III, officially cancerous, meaning it could start spreading throughout the body at any time. The Perris decided to pursue treatment at Penn — pinpoint radiation plus another round of Temodar. If James needed immediate surgery, at least he’d have Dr. Judy in his corner. He bought gift cards for the radiation techs, figuring, If you guys are in charge of shooting beams into my brain, you should at least like me.
And so began, in June of 2010, what Nancy calls “the longest month-long vacation ever.” The Notre Dame guys — Hanlon, Mnieckowski, Masone, Jim Borger ’96 — assembled in the Jersey shore town of Brigantine, where their families shared rental houses during James’ treatment in one more grasp at normalcy.
Now the Notre Dame guys were husbands. Now they were fathers, with children of their own, children who played together in the Atlantic surf during an otherwise idyllic summer. Now they had front-row seats to a grueling treatment regimen that reduced their friend to a fatigued wreck. Hanlon cried when he first saw him. Each guy — plus James’ father, Art — took turns driving James back and forth on the Atlantic City Expressway for treatments at Penn. Marveling at his stoicism; sensing his worry. Marveling at Nancy’s strength in the face of a nagging question: How do we tell the kids? There’s no textbook for that.
James: “What I don’t want is [Jack and Sofia] awake at night, worrying, midnight, 1 in the morning, thinking: ‘Is Daddy gonna die?’ I try to protect them from those nightmarish thoughts as long as possible. I think they understand it as: ‘Daddy had this tumor. It’s gone. We just make sure it doesn’t come back.’”
Nancy: “My mom just died in January from breast cancer. Seeing her debilitate — James knows. Seeing what cancer can do to someone. James is Jack’s hero. It’s kind of scary to think your hero could get that way.”
Night after night, after the kids were scrubbed of sand and put in bed, Nancy stayed up and looked for a cure. Hours of scanning through Internet forums, hospital websites, anything. For every bit of good news there was a crushing wave of bad. She called doctors, who told her: There’s no money in oligos. She called Dr. J. Gregory Cairncross, who pioneered oligo research at Southern Alberta Cancer Research Institute. He said the same: If you can raise the money, that’s great.
At the conclusion of James’chemo and radiation, an MRI showed that the cancer was static. They know it’s coming back; they just don’t know when. A game of surveillance.
That’s when Nancy realized the battle was just beginning.
It sounds crass, but finding treatments for rare diseases — ALS, Niemann-Pick Type C, cancers like oligo — isn’t necessarily about patients. It’s about money. Or the lack of it.
Even for pharmaceutical juggernauts, bringing a new drug from bench to bedside requires an average investment of five years and $2 billion. Only exceptional treatments for orphan diseases — those affecting fewer than 200,000 people in the United States — will recoup that investment. And while taxpayer-funded bodies such as the National Cancer Institute and the Department of Defense do fund research on rare cancers, they prefer to fund research on more common cancers of the prostate, breast or lung.
Pharmaceutical companies have some incentives to develop orphan drugs. Some are regulatory: Clinical trials for orphan drugs typically require fewer participants than those for mass-market drugs. Some are financial: The first companies to develop orphan drugs enjoy anywhere from three to seven years of market exclusivity. Total control of even a small drug market can be a financial windfall: Celgene’s orphan drug Revlimid, developed for mantle-cell lymphoma and multiple myeloma, netted nearly $3.8 billion in sales in 2012 alone.
In the past decade, large pharmaceutical companies have started spending less on in-house research in favor of buying early-stage drugs from research labs. So the task of creating new drugs increasingly falls to researchers, who must grow ideas into viable drugs before companies swoop in and shepherd those drugs to market. Growing those ideas takes money. And as Nancy Perri discovered, there’s not a lot of money in oligo research.
James and Nancy made a deal: If he could get on Who Wants to Be a Millionaire and win the money — they had always been fans when Regis Philbin ’53 hosted it — then she would use the winnings to start a foundation to fund oligo research.
James applied in the spring of 2010, after surgery but before treatments. He told the producers his story, maybe got a little emotional, and of course the producers were stunned, because here’s a doctor trying to raise money to cure a rare form of brain cancer. They sent him a postcard saying he was accepted, and they’d call when they needed him to tape the episode.
A year passed without a phone call. So when the production team was recruiting contestants in Charlotte in the fall of 2011, he stopped by and said: “Listen, I got through all the interviews earlier, but I haven’t heard from anyone. I just want you to know that I’m not dead.”
Throughout 2011, Nancy was struggling to wade through the morass of IRS paperwork necessary to start a nonprofit. She was, in her own words, “just a chick with an insurance background” (and, it bears mentioning, mother to a 5-year-old and a 4-year-old).
“It was me doing the research, finding connections, building the website,” she says. “That was the challenge — I had to learn everything. There are so many components.”
Oh, but Nancy Perri was a worthy opponent. This woman is tough. The kind of girl who used to call Philadelphia’s Homework Help Hotline in grade school because her mom was working the graveyard shift at Pat’s Cheesesteaks until 3 in the morning. The kind of woman who ground through insurance work at Blue Cross to put herself through Temple University. The kind of woman who stands by her mother dying from breast cancer, even as her husband recovers from chemotherapy.
“It really depended on the week, to be honest with you,” she says. “Sometimes I would pray, and be thankful for what I had. Sometimes I would be angry. Saying, ‘This is ridiculous.’ Faith brought me up more than it tore me down. It’s a cycle that anyone goes through when you’re going through that. ‘Oh God, thank you. Oh God, please. Oh God, why me?’
“I would go to church and pray. I still do sometimes. I’m going to cry saying this, but I seem to have lost my faith since my mom passed away. In fact, I told a priest that. In confession. Oddly enough, he said it’s okay. . . . I wasn’t expecting the priest to say, ‘Well, I can see how you feel that way.’”
So when a yearlong journey through mountains of paperwork separated her from a cure for her husband’s brain cancer, well, Nancy Perri had seen worse.
Things did get worse. In 2010, James had undergone a type of radiation called IMRT, short for intensity-modulated radiation therapy, which minimizes side effects by concentrating radiation in a small spot. But Blue Cross denied him coverage. The bill: $300,000.
“As if it’s not stressful enough that we’ve got all this going on,” James says. “I’m like, ‘All right, I’m gonna die and I’m gonna bankrupt my family.’ Not a good way to go out.”
IMRT was a standard of care in Pennsylvania but not in North Carolina. Dr. Robert Lustig, a Penn radiation oncologist specializing in brain tumor treatments, had written letters to Blue Cross emphasizing how IMRT is gentler on healthy brain tissue. But coverage ultimately rested on a final face-to-face appeal in Raleigh. “If you’re gonna deny me,” James told the insurance execs, “you’re going to do it to my face. And my father. And Nancy. And Sofia.”
About halfway to Raleigh — a sunny day in North Carolina, the Perris on I-85 outside Burlington — James’ phone rang. The conversation, in his words:
“Dr. Perri?”
“Yes?”
“Are you still coming?”
“Yeah, we’re on our way there.”
“Well, you can turn around now. We’re going to approve the coverage.”
“Really?”
“Yeah, we’ve thought it through, and we’re going to change our policy in North Carolina. We’re going to cover IMRT for brain tumors.”
“Okay. But I’m going to need this in writing. You’ve got to fax it to me as soon as I get home.”
Cheering briefly rocked the car and, finally, at 5:30, the letter came rolling off the fax. Relief, to the tune of $300,000.
Two things are immediately obvious after spending any amount of time with the Perris. First, they are preoccupied solely with living. Even when James and Nancy journey to Memorial every six months for James’s surveillance MRIs, their friends welcome them for a celebration at some Italian restaurant. Second, they are a family of spontaneous action. Jack, now 9, produces enough wattage to power most of metropolitan Charlotte; 7-year-old Sofia is shy for all of 29 minutes before she bursts into conversation.
“Sometimes,” Nancy says, “God gives you a glimpse of mortality, and it shows you how precious your family and friends are. We’re not down. This is our life. And we roll with it.”
Example: One morning in October of 2011, James and Nancy rouse Jack and Sofia at 6 a.m. “Daddy’s gotta go to a business meeting,” he tells them, “so we have to get up really early.” The kids, of course, are miserable: In the car, through airport security, on the plane to Orlando, Jack complaining the whole time that he wants to stay home with his friends.
When they arrive for James’ “business meeting,” the kids are so enthralled by the live African animals outside their hotel — “Dad, it’s a giraffe! Holy cow, it’s a zebra too!” — they somehow miss the fact that they are outside Disney’s Animal Kingdom Lodge. Instead of taking the usual ad-plastered shuttle to the main park, the Perris take a cab. “Not a word about Disney,” James tells the cabbie.
So when the family Perri finally arrives at the Magic Kingdom, James and Nancy say: “Kids, do you know where you are? You’re at Disney World.” And there, right in front of the family’s rolling camera, the kids burst into joyous screaming. Disney World!
But James has one more trick up his sleeve. “If you could wish for anybody to be here,” he says to Jack and Sofia, “who would it be?” They say, “Nana and Papa!” And when James’ parents come walking out of the Magic Kingdom castle, Jack is so stunned he drops his soda. (“This would have been the perfect commercial,” Nancy says.)
That week — of all the weeks! — James got a call. The person on the other end of the phone wanted to know: Do you want to be a millionaire, James Perri?
And yet.
Sitting in the studio chair across from hostess Meredith Vieira in November 2011, Art and Nancy in the audience, James wrestled with a paradox. He was in the studio to raise money and get out the word about an ultra-rare form of brain cancer. But he knew Jack and Sofia would watch the show eventually, see what their daddy was doing, hear the scary c-word for the first time. He begged the producers not to mention anything.
But after the show’s taping, when James coaxed Jack and Sofia into watching Who Wants to Be A Millionaire on April 23, 2012, their only surprise came when the announcer welcomed a doctor from Huntersville, North Carolina, who miraculously managed to be on the TV and in the Perri living room at the same time. (“Dad! Mom! But you’re here! But you’re there!”)
Even though their dad didn’t make it too far in the game show — stumped by some stupid pop culture question — he walked away with $15,800, enough money to get the nonprofit foundation off the ground. Nancy decided to call it Operation Oligo Cure.
To finalize the foundation, Nancy had to assemble a board of directors: men and women of action who would realize her mission, who would succeed where few were even trying.
So she called the boys from Manalapan, the old backyard baseball team. Mike Masone, now a lawyer who spent his pro-bono time setting up nonprofit organizations; Pat Ronan, a powerhouse pharmaceutical consultant with contacts across the industry; Pat Hanlon, a real estate executive who knew Manhattan power players.
She called the rest of the Notre Dame guys: Ron Mnieckowski, an executive at a large real-estate partnership, and Mike Towers, an information security expert with a catbird seat in the pharmaceutical industry.
She called the med-school classmates who talked James back into normalcy. Charlie Getz and Frank Nezu, now established doctors who understood the intricacies of clinical research.
Nancy called Dr. Dave Rentz, who understood before anyone else why James Perri preferred not to pick up brain-related cases in the ER. She called her in-laws: Amy Perri (now Amy Coffey) and Art Perri, who sat at the kitchen table in 1996 and faced down oligo when it first darkened their horizon.
And she would be the cornerstone: Nancy Perri, the self-professed overachiever from the fighting city of Philadelphia, the woman who would save her husband through sheer force of will. Invincible.
One by one, she heard an instantaneous yes.
Hanlon: “When you’re friends with someone for a long time — middle school, high school, college, post-grad, to life — you don’t take that for granted.”
Mnieckowski: “We’re not just gonna sit around and watch our friend die from a disease without a cure.”
What were the chances that, after all these decades, James’ friends — their friends — formed a constellation of hearts and minds so ideal for her fundraising effort? Could it have been a coincidence? Or part of some divine plan?
Godwinks, his mother called them.
With Operation Oligo Cure an official 501©(3) and a board of directors in place, Nancy directed her energies to raising money. But the question then became: Where would it have the most impact?
Says Ronan, who spearheaded the research effort, “It all boiled down to: How do we work best to make sure James is here in 25 years?”
Eventually the board focused on the University of Texas MD Anderson Cancer Center. The center’s Brain Tumor Research Program has pioneered a number of breakthrough treatments for cancers of the brain and central nervous system. In February of 2013, the board unanimously voted to dedicate their fundraising toward a professorship at MD Anderson.
“[We’ll] fund the position, and then have — in perpetuity — a devoted academic who is dedicated to researching oligo,” Masone says. “It’s a powerful, powerful thing. It’s targeted research with a finite amount of time and a requirement for progress, and that’s what James needs right now. It’s our best hope for saving our friend.”
With his team of researchers and technicians, Dr. Charles Conrad, a leading neuro-oncologist in the Brain Tumor Research Program, is overseeing three specific lines of research into new oligo treatments.
One of those therapies is a specially engineered virus that rips through oligo tumors but leaves healthy brain cells untouched. Some pharmaceutical companies have working versions, but Conrad says a virotherapy could take a decade to pass FDA approval.
Two other avenues represent more immediate opportunities. One set of drugs, ipilimumab and nivolumab, is indicated to disrupt oligo’s ability to blindfold the immune system. Another option: A drug that can cross the blood-brain barrier and clamp down on the rogue PDGF-R receptor that (probably) triggers oligos in the first place. Researchers are trying to retrofit an existing drug — like Novartis’ leukemia drug GleeVec — to create a version that will effectively cross the blood-brain barrier.
“We could develop these drugs within two years,” Conrad says, “if we had enough money.”
Enough money. That’s the biggest obstacle. Nancy spearheaded a fundraising gala in autumn of 2013 that netted $42,000. As of October 2014, Operation Oligo Cure has reached the $400,000 mark; now it’s aiming for a million. MD Anderson can begin the search for a professor, and money also is being donated to a dedicated research fund that can be used for immediate projects, although there’s no guarantee of a successful treatment.
But the goal is as much spiritual as it is practical: A specific goal creates the sense of control. A sense of control creates a groundswell of hope.
Hanlon: “It’s almost a race, but it seems like there’s something we can do. That’s really encouraging, and I think that energy drives a lot of us here. It’s actually something we can do.”
Michael Rodio is a writer in New Jersey and an editor at NBC in New York City.