Astonishing stories of patients who cheated death or permanent disability, and the physicians who saved their lives.
Photos by Becky Thurner Braddock
The presents had been spirited into the hospital and stashed. The Ball family was ready for Christmas morning — a makeshift, strained celebration, but Christmas morning nonetheless. They were determined to celebrate the holiday as a family with 6-year-old Luke, but his body just wouldn’t cooperate. “We were losing him,” recalls his father, John, a nuclear engineer for Westinghouse. “His body just couldn’t sustain anymore.”
On Dec. 24, 2010, Luke, who had been on the list for a heart transplant since early November, was moved into intensive care. The gifts went home, unopened. Although the Child Life team tried to raise their spirits, this holiday still felt very dark. The doctors had one more thing they could try: a last-ditch medication. And it worked, for the moment. The crisis ebbed, if not passed. But the Balls heard the clock ticking. They knew that more than 15 percent of kids on the donor list die waiting.
For kids with life-threatening heart complications, the problem is a familiar one: Not enough energy. Procedure after procedure, hope after hope and, finally, if the cards land the right way, a transplant. For Luke, it began in the Pacific Northwest in 2004, with a prenatal ultrasound that revealed a congenital defect — a single-ventricle heart that wasn’t sending enough oxygen to his body. After countless treatments and surgeries, a near-fatal seizure and even a stroke, John and Stacie Ball moved the family to Pittsburgh, and Luke found himself on an operating table under the care of three doctors at the Heart Institute at Children’s Hospital of Pittsburgh of UPMC: Dr. Victor Morell, the chief of cardiothoracic surgery; Dr. Peter Wearden, a pediatric heart surgeon; and Dr. Steven Webber, chief of pediatric cardiology.
At 4 a.m., on Dec. 29, 2010, the call came: A donor heart was available for Luke. How does a parent begin to process such feelings? “Elation. Fear. Anxiety,” Ball says. “And of course, as you go through these waves of emotions, you realize there’s this other family that is going through so much pain and anguish.”
The extended family spent that morning with Luke. They played checkers, Uno and Candyland (Luke’s favorite). Then they waited. At first, the post-transplant news was good — not much bleeding. Then he went through 10 more units of transfused blood, the beginning of a “very sketchy night.” Alarms went off every few minutes, all night long. But the doctors were calm, which, in turn, calmed the family. “We were terrified,” Ball says. “But somehow we were comfortable.”
After a few days, Luke was pointing, talking, communicating and sitting up. “We had him back,” Ball says. “You saw the look in his eyes. It was so different. It was so new.”
“If Luke had been born in the ’70s, he’d probably be dead,” Dr. Morell says. But techniques developed throughout the past two decades allow doctors to “redo the plumbing” of faulty hearts. When transplant time comes, though, surgeons have to essentially nullify that redoing to implant the healthy, properly working heart — which is what happened in Luke’s case.
“We’ve come a long way,” Dr. Wearden says. “And now he has a chance at many more years.”
Luke still struggles with his energy level, but he’s catching up. He’s playing baseball — and even basketball and soccer. And the terrain hasn’t been entirely rough: Luke’s experiences have brought some of his heroes to the foot of his hospital bed — including Pirates infielder Neil Walker and his brother-in-law, Tigers infielder Don Kelly.
The Balls know a transplant doesn’t exactly carry a lifetime guarantee. But a year after receiving the new heart, on Dec. 29, 2011 (the date that the Ball family calls “Happy Heart Day”), Luke celebrated. Kelly and Walker visited. Last year, Luke, who’s now 8, had the thrill of a lifetime when he threw out the first pitch at a Pirates game. That’s the first step in his plan to eventually become the team’s second baseman.
“We told the Pirates to look out,” his father says. “Because if Luke says he’s going to do it, I wouldn’t doubt it for one minute.”
— Ted Anthony
In 2008, 17-year-old Hannah Snyder’s car was forced off the road in Altoona by another driver who didn’t stop. She flipped down a long embankment, and in the awful minutes that followed, she could tell her back was badly injured. “But I knew I had to get back to the road to get help,” she says. Hannah crawled up the embankment and flagged down a car. Incredibly, she did this with a severe spinal fracture.
She was airlifted to UPMC Presbyterian, where neurosurgeons Dr. Adam Kanter and Dr. David Okonkwo performed an innovative, minimally invasive spine surgery that not only saved her from paralysis, but also allowed her to fully recover from her devastating injury.
“One of the first things they told me [at UPMC] was that I could have been paralyzed just by moving,” Hannah remembers. “That was a crazy thing to wrap my mind around.”
“By the time she got to us, she still had use of her legs, but her spine was unstable,” says Dr. Okonkwo. “Any movement, including her climb for help, could have led to paralysis. Fortunately, in her case, it didn’t.”
Hannah had suffered a severe lumbar burst-fracture in multiple directions. A fragment of her spine had broken off and was putting pressure on her spinal cord. “The spinal cord is such a critical area, one that’s important to motor and sensory abilities,” says Dr. Kanter. “We needed to remove that fragment from her spinal canal quickly.”
Traditional surgeries for injuries like Hannah’s are called “shark bites” — and they’re almost as traumatic as their name. Drs. Kanter and Okonkwo have worked together to develop a less-invasive technique, which reduces the severity and complications of major surgery — and means a faster and more-complete recovery.
“To us, ‘minimally invasive’ means ‘minimally destructive,’” says Dr. Kanter. It begins with a 2-inch incision, instead of a 10- or 12-inch one. “And typically, her problem would then be dealt with by taking down healthy young muscles from her back,” he explains, “which is not something you want to do to such viable structures.”
“Dr. Kanter worked his magic and got us to Hannah’s spine through that small incision,” says Dr. Okonkwo.
The pair then removed the fracture fragment, alleviating the pressure on her spinal cord, and replaced the damaged part of the spine with an expandable cage filled with portions of her own bone to aid in the healing process. This cage also allowed Dr. Okonkwo to reduce the spinal deformity caused by the injury. “It essentially works as a jack, which helps restore the natural alignment of the spine,” he explains.
Hannah was out of bed the day after her operation and returned home from the hospital a few days later. “With a shark-bite operation, the patient’s just starting to eat at four days,” notes Dr. Okonkwo. “And their hospital stay can be [up to] two weeks.”
Today, Hannah’s as active as she was before the accident. “I really haven’t found any handicaps,” she says. But she has found something else: a new perspective. “My accident and my recovery make me believe I was put here for a reason. It makes me a lot more grateful for what I have.”
“Seeing Hannah the way she is now is the validation of 17 years of hard work,” says Dr. Okonkwo, “of getting out of bed at 4 a.m., of sleepless nights, long days, and making sacrifices in our personal and professional lives. Her recovery is the reason we do this.”
— Joshua Mooney
The doctors told Allen Fennell that if he’d waited for an ambulance, he might not have survived. The Natrona Heights resident felt the first signs of heart trouble one day last October. “I started feeling bad, so I took my blood pressure,” he says. “It was 200 over 100-something. I started sweating like crazy — my heart was pounding. My wife asked if she should call an ambulance, but I said, ‘I don’t think we have time.’ So she helped me to our car.”
Allen, 53, was having a massive heart attack, commonly known as “The Widow Maker” because it is a fatal injury in most cases. Fortunately, Allen lived only a half-mile from Allegheny Valley Hospital. By the time his wife pulled up to the emergency room, he was in full cardiac arrest. The AVH emergency team began intensive efforts to resuscitate and stabilize him, including CPR, clot-dissolving agents and defibrillation. Their relentless efforts to save him paid off, and a stable heart rhythm was restored.
Because Allen was still in grave danger, he was immediately flown to Allegheny General Hospital, where interventional cardiologist Dr. David Lasorda and his team were waiting.
“Allen looked awful. He was in shock, on a ventilator and bleeding,” Dr. Lasorda recalls. “He was rushed to the cardiac catheterization lab where the angiogram of his heart showed a total blockage of the left anterior descending artery — the big artery down the front of the heart. We were able to pass a wire across the blockage, then open the blockage with a coronary stent. ”
But Allen remained in shock, and his heart was still failing. So Dr. Lasorda and AGH cardiac surgeon Dr. Stephen Bailey placed him on ECMO (extracorpeal membrane oxygenation) to give cardiac and respiratory support. “This gave us time and enabled us to switch him to the Tandemheart left ventricular assist device,” Dr. Lasorda said.
Tandemheart is a new generation heart assist device that does the work of the heart’s failing left ventricle by drawing the oxygenated blood from the left atrium, running it through a small external pump and then back into the aorta(bypassing the failing left ventricle) so that the vital organs of the body can be perfused. The Tandemheart is connected to the heart’s left atrium by way of cannulas (or tubes), which are inserted through the leg.
“It’s an amazing device that gives the heart time to recover,” Dr. Lasorda says. “And it’s made by a company headquartered in Pittsburgh called CardiacAssist.”
Allen was placed in a medically induced coma for 10 days to allow his heart to recover. Many patients in his situation may survive the heart attack but end up with brain damage due to oxygen deprivation.
“Allen’s recovery was simply remarkable,” says Dr. Lasorda. “There was no brain damage, and after about 48 hours on the Tandemheart, we could see his heart-muscle function was improving, so we weaned him off it. When he left the hospital, I’d say his heart function was near normal. It’s exhilarating to see a patient recover like that.”
Today, Allen still marvels at how quickly he went home. “I hear ‘miraculous recovery,’ but I’ve never felt that way,” he says. “Then again, I slept through most of it! I’m just glad they didn’t give up on me. I now know how bad it was, and I know how hard they worked to save me.”
— Joshua Mooney
Dr. Regina Jakacki can’t stifle a smile when she talks about Leah Koller. Her smile often becomes an out-and-out grin. That’s because of how Leah’s doing. At 12, eight years after she was diagnosed with a glioma — a complicated form of brain tumor — the Waynesburg girl is thriving, thanks to an experimental treatment spearheaded by the Brain Care Institute at Children’s Hospital of Pittsburgh of UPMC.
The team included Dr. Jakacki, director of the neuro-oncology program, and her colleague, pediatric neurosurgery chief Dr. Ian Pollack. The treatment that has been working for Leah is, in essence, a vaccine — not entirely unlike a vaccination against mumps or measles — that attacks certain proteins in the tumor. A team at the University of Pittsburgh and Children’s developed the vaccine, which is the first of its kind in the country.
Because of the tumor’s location, doctors knew that removing it would cause her to be paralyzed on her right side. And it also appeared that Leah’s tumor was spreading throughout the brain, which made surgery an even less viable option. Radiation therapy to the brain can cause severe developmental damage in young patients. So last year, with the Kollers’ blessing, they turned to the experimental vaccine.
The idea of using vaccines came out of a longstanding program that examined how they’d affect malignant brain tumors that weren’t responding to standard therapies like surgery, radiation and chemo, which aren’t curative. Doctors at Children’s suspected that immunotherapy might work better. So they looked at the antigens on the tumor cells and identified several targets — what Dr. Pollack calls “little snippets of protein” that could be used as a vaccine. An early study in adults was encouraging, so Drs. Pollack and Jakacki moved on to children, who tend to have more robust immune systems.
At first, in 2009, even the doctors were skeptical about the vaccine. “I wasn’t a complete believer,” Dr. Jakacki recalls. “I wasn’t even sure people were going to come for it.” For starters, a patient needed to have a certain tissue type to be eligible — a type that is found in only about 40 to 45 percent of the general population. “Initially, it sounded like a pie-in-the-sky kind of thing,” Dr. Jakacki says.
Not much happened at first, and few volunteers came forward. Then enrollment peaked, and progress surfaced in a few patients. One child got much worse during the treatment, and the tumor looked much bigger. However, throughout the next several weeks, the child improved, and an MRI showed that the tumor had shrunk to less than half the size it had been, indicating that the tumor had swelled as the vaccine attacked the tumor cells — but the vaccine was indeed working. Says Dr. Jakacki, “We kind of looked at each other and said, ‘Really?!’”
Leah’s tumor was low-grade and slow-growing yet complicated; it started expanding into the coating of the brain. Leah was put on chemo, but the radiation that could have really tackled the tumor would have also affected her cognition. When she started the vaccine, she had few side effects. But an MRI taken after nine weeks showed the tumor actually seemed worse. They kept going. Then it looked stable. Then, slowly, it shrank — enough that its shrinkage finally became obvious.
Now, Leah’s tumor is about 50 percent smaller — 75 percent, Dr. Jakacki says, if you take into account dramatic shrinkage in the areas of metastasis. Twenty-seven children have tried the treatment, and a dozen remain on it. And Drs. Pollack and Jakacki are looking at ways the vaccine usage might be expanded. That, too, makes Dr. Jakacki grin.
Meanwhile, Leah is starting to flourish.And there’s a new member of the Koller clan these days, not related by blood but rather sweat and tears: Dr. Jakacki, who finds herself an honorary Koller for life.
“She’s just part of our family, whether she wants to be or not,” says Leah’s mother, Raelene. “She’s part of us now.”
— Ted Anthony
Three years ago, Reid Sigal was an ambitious 24-year-old starting his accounting career at a Pittsburgh firm — so he was willing to shrug off his serious stomach pains as “a real nuisance.” But the pains became relentless and kept him up at night. In time, they almost killed him. Reid was eventually diagnosed with Crohn’s disease, a chronic inflammatory bowel disease that usually attacks the intestines. He was treated by Dr. Miguel Regueiro, Head of UPMC’s Clinical Inflammatory Bowel Disease (IBD) Center, which provides a healthy future for patients with its novel treatment protocol for postoperative Crohn’s.
“When I first met Reid, we put him on medications, and he was doing well,” recalls Dr. Regueiro. Typical Crohn’s medications include anti-inflammatories, steroids or immune-suppressants to control the inflammation. “We were testing him regularly and the Crohn’s seemed under good control. He didn’t have any apparent inflammation or scar tissue that Crohn’s disease can cause. So what happened next was a surprise — and very humbling to us as physicians.”
One night in fall 2009, less than a year into treatment, Reid’s stomach pains came back, worse than ever. “I just started throwing up everywhere,” he says. “I’ve played sports and I’ve broken bones before — but this pain was much worse.”
“He came to the hospital that night with complete bowel perforation,” recalls Dr. Regueiro, “and was sick enough that we thought he was going to die. He was triaged straight to the operating room.”
Reid was enduring what Dr. Regueiro calls “a perfect storm” of Crohn’s complications: intestinal scar tissue (common in Crohn’s patients) as well as a fistula. “The scarred tissue caused a bowel blockage and then Crohn’s disease burrowed through the weakened intestine wall. It ruptured more suddenly than we usually see.”
“Reid’s situation was as bad as it gets,” remembers Beth Rothert, who, as Dr. Regueiro’s nurse-coordinator, is the primary liaison between him, his patients and their families. “Reid’s family couldn’t help but be devastated in a situation like that.”
The Crohn’s-affected section of Reid’s bowel was removed by Dr. Wolfgang Scrhaut, but he wasn’t out of danger and spent a long time in the intensive care unit. “He had a bad infection, due to the ruptured bowel,” says Dr. Regueiro. “That can send people into shock, and they may not recover. Well, not only did Reid recover, but we were also able to initiate a novel treatment plan so that he’s had no recurrence of Crohn’s. Today, you wouldn’t know that he’s ever been sick. That’s very rare.”
Dr. Regueiro’s innovative treatment approach is based on his study, which found that when a biologic medication (known as an anti-TNF) is given after surgery, the recurrence of Crohn’s disease is dramatically reduced. This means that many patients are living “Crohn’s-free” lives with this new treatment plan.
Reid currently feels better than he has in years. “I’m basically in remission,” he says. “Dr. Regueiro’s treatment plan has worked great.” Since leaving his job, Reid is now working full-time toward an MBA at Carnegie Mellon University.
“After the surgery, I was inspired to do something I’d always only talked about,” he says. “The near-death experience spurred me on, changed my perspective and gave me motivation. And now I’ve got the energy to do it.”
“Reid is a very strong person,” says Nurse Rothert. “Even after his rupture, he never fell into that ‘sick’ role that some patients do. Drawing on the loving support of his family, he physically and mentally fought back to survive.”
— Joshua Mooney
In 2005, Rita Spontak was diagnosed with colon cancer. She had an operation, followed by chemotherapy, and fervently hoped her troubles were over. But just months later, she learned that the cancer had spread to her liver, so she would need to undergo more surgery. “It didn’t register at first,” Rita recalls. “It was depressing to learn I’d have to do this all over again.”
Today, Rita is 61, cancer-free and enjoying her retirement in Economy Borough, thanks to UPMC’s multidisciplinary treatment of liver cancer.
Colon cancer is one of the top four cancers in the country — along with lung, breast and prostate cancers — says Dr. David Geller, co-director of the UPMC Liver Cancer Center.
“There are 160,000 new cases of colorectal cancer every year,” he says. “And about 60 percent of patients will have it spread to their liver, as [it did] in Rita’s case.”
Rita had stage IV cancer, with a softball-sized cancerous tumor filling most of her liver’s right lobe and touching important blood vessels. Three-quarters of the patients in her condition are inoperable.
“That’s why she could be described as a relative medical miracle,” says Dr. Geller. A team of UPMC doctors worked together to give Rita her multidisciplinary treatment. Surgeon Dr. Garth Elias initially removed the part of her colon in which the cancer had started. Then oncologist Dr. Kiran Rajasenan put her on a clinical trial of chemotherapy drugs, which reduced the size of the tumor and allowed Dr. Geller to proceed with her liver resection (surgical removal).
“The only cure for liver cancer is to cut it out,” Dr. Geller says. During the operation, he removed almost two-thirds of her liver.
Dr. Geller has pioneered minimally invasive laparoscopic liver resection surgery, which allows most patients to go home on the second post-operative day with four or five Band Aid-sized incisions. It involves lower amounts of blood loss, faster recovery and less pain and scarring. Since 2000, his team has performed almost 500 of these operations, the largest series in the nation. He even taught the country’s first course on the procedure.
In Rita’s case, Dr. Geller needed to do a traditional resection because her tumor was too large and too close to the blood vessels to treat laparoscopically. Her operation at UPMC Passavant was the first of its kind performed in the North Hills. Dr. Geller started the program there, close to her home, because “people wanted cutting-edge cancer care in their backyards. They don’t want to have to go into the city.”
Rita went home five days after the operation. She didn’t need blood transfusions or recovery time in the ICU. “I really think we’re done with it,” she says confidently of cancer. “I don’t think we’re going to see it again.”
According to Dr. Geller, the surgical and medical management of cancer is significantly better than it was even a decade ago, as is the patient’s quality of life during and after treatment.
“We can’t cure them all, but many we can,” he says. “For others, we can buy a few years of life. That’s tremendously gratifying, especially when you can do it in a minimally invasive way.”
Rita says there’s another factor that contributed to her successful outcome: “Everybody knows Dr. Geller’s expertise, but his patients know he’s extremely kind. After my surgery, he was so concerned about getting me home for Christmas — and he did,” she recalls. “Going through all that, it’s so important to have someone you can trust.”
— Joshua Mooney
Jim Harris, a West Virginia auto mechanic and musician, recalls the day six years ago when he first knew something was wrong. “I was changing the oil on a pickup truck, but I couldn’t get my thumb and index finger to screw the oil plug back in,” Jim, 53, remembers. “It was strange — this is something I’d do every day. But then it happened again, and again.” The problem, he would learn, was Parkinson’s disease, a brain disorder that affects the muscles. It leads to tremors and trouble walking, and it can impact the patient’s ability to function normally on almost every level.
The damages only increase with time. Eventually, Jim reached the point where he couldn’t play his music or drive — and he had to go on disability.
Today — thanks to Dr. Donald Whiting, a leading specialist in the use of deep brain stimulation (DBS) therapy at Allegheny General Hospital — Jim is basically symptom-free. He’s currently off all conventional medications that, for decades, were the only treatment for this progressive, debilitating disease.
Parkinson’s involves the loss of brain cells that supply the neurotransmitter dopamine, which leads to muscle rigidity and tremors. Medication can help for a while, but Dr. Whiting says, “it has a finite life expectancy as far as usage, and you start needing more and more medication with less benefit. Plus, there are side effects.”
DBS, by contrast, involves surgery, in which electrodes are implanted in the brain to deliver a continuous low-level electric current to stimulate specific areas. “DBS doesn’t cure Parkinson’s — nothing can — but it can give patients their lives back,” says Dr. Whiting. “It’s more effective than medication because the brain stimulation is constant.”
The DBS current, supplied by a pacemaker-like device implanted under the skin, can be finely tuned and controlled in terms of voltage, pulse-width and frequency, explains Dr. Whiting. “So in a very organized, precise way, you can impact the exact parts of the brain you need to.”
Originally, Jim says the idea of brain surgery was intimidating. His neurologist, Dr. Susan Baser at Allegheny General, had been treating him with medications and told him about the option of DBS with Dr. Whiting.
“My wife and I discussed it for about a year,” Jim says. “But we put it on the back burner because the idea of brain surgery was scary. But it got to the point where the shots I was taking to control the Parkinson’s were only working for 45 minutes. So I told Dr. Baser I was ready.”
Dr. Baser agrees that the surgery is a tough sell. “Many people would be good candidates for DBS,” she says, “but they have to get over that hump.”
And Dr. Whiting understands the concerns because “it’s brain surgery, after all. But it’s minimally invasive — not like removing a tumor — with a very small percentage of risk.”
For Jim, like most DBS patients, the positive results were instantaneous. “They filmed my operation, and you can see how, at first, as I was lying on the table, my right-hand tremors were tremendous,” he says. “I was shaking like a leaf. But as soon as Dr. Whiting turned the power on the probe, those tremors came to a dead standstill. It was amazing. And still is.”
Nowadays, Jim says, “I can walk into my music room any time, pick up my bass and play it. It’s almost like I don’t have Parkinson’s at all. And that’s made a complete difference in my life.”
— Joshua Mooney
Kelly Schwabenbauer, 19, is talking on the phone and babysitting — two typical activities for a teenage girl. But for most of her life, Kelly says she “missed out on a lot,” due to a viral infection at age 2 that caused severe hearing loss in both ears. Now, thanks to Dr. Douglas Chen and a revolutionary hearing device called an “active middle-ear implant,” she’s interacting with her world in an exciting, new way.
Dr. Chen, co-director of Allegheny General Hospital’s Hearing and Balance Center, is a pioneer in the use of a new generation of hearing devices that can be partially or fully implanted. “They provide much better hearing than conventional in-ear aids,” he says. “And they’re also more cosmetically appealing, as they don’t sit in the ear canal itself.”
Kelly agrees: “I started wearing an over-the-ear hearing aid at age 3. I had challenges in school because I was slightly different.”
Those devices could be painful — but even worse, Kelly says, “I always knew I wasn’t hearing everything. And with the old hearing aids, if something got too close to them, like a phone, there was a constant, annoying whistling sound.”
Conventional aids merely amplify sound, and because they rest in the ear canal, distortion is inevitable, says Dr. Chen. “If you place your finger over your ear and talk, you’ll hear an echo we call an ‘occlusion effect.’ Rather than just amplify sound, these implants create a direct mechanical connection to the little ear bones called ossicles and vibrate them directly,” he says. “The vibrations are interpreted by the brain as sound. So you not only lessen the distortion, but you also get better amplification in the high frequencies.”
Last summer, Kelly’s first partially implantable device, called the Vibrant Soundbridge, was surgically placed in one ear. Dr. Chen performed the outpatient surgery, which required a 3-inch incision behind her ear. The device consists of two parts: One placed underneath the skin behind the ear, and the other, about the size of two stacked quarters, sticks to the skin with magnets. In Kelly’s case, the Soundbridge is situated in her hairline and is essentially invisible.
A few months later, Dr. Chen finally activated Kelly’s Vibrant Soundbridge for the first time. “She was ecstatic,” he recalls. “It was like a lightbulb had been switched on. That’s the kind of emotional response we often see from our patients.”
“I could hear everything,” Kelly says. “As we were leaving the doctor’s office, my dad was standing behind me in the elevator and whispered, ‘Kelly is awesome.’ And I could hear it! I could never hear anyone behind me before — they had to be in front of me so I could see their face and read their lips.”
Kelly’s hearing is now in the normal ranges, Dr. Chen says. While she’s considering whether to get a second device for her other ear, she says that she hears much better with one Vibrant Soundbridge than she did before with two hearing aids.
“With this new generation of devices, we can provide life-changing events for patients, many of whom were previously told that nothing more could be done for them,” says Dr. Chen. “And in my field of hearing restoration, that’s what it’s all about.”
— Joshua Mooney
Point Breeze native Josh Mooney spent the last 25 years as a journalist in Hollywood and New York, and has now come home.
Ted Anthony, a veteran writer and editor, has reported from more than 20 countries and 47 U.S. states. He lives in Allison Park.