Two years ago, Jean Magazzu began having trouble breathing. Her doctor thought it could be allergy-related — or perhaps a case of bronchitis.
But multiple rounds of antibiotics didn’t help, and her breathing worsened to the point where she couldn’t even walk across a room without sitting down.
An X-ray revealed the frightening truth: Her lungs were filled with blood clots and scar tissue, a symptom of pulmonary hypertension (PH). She was immediately transferred to Allegheny General Hospital, where cardiologist Dr. Raymond Benza diagnosed her. “Pulmonary hypertension is a rare disease, affecting only 15 people in 1 million,” says Dr. Benza, a nationally recognized leader in the treatment of PH. “It’s also rapidly progressing and deadly — probably as mortal as any cancer we treat.”
Jean’s form of PH — related to the chronic clots in her lungs — was even more rare.
The disease is characterized by high blood pressure in the lung’s pulmonary artery, which forces the heart to essentially work itself to death. “When she came to us, she was already in the throes of severe heart failure,” says Dr. Benza.
Along with his team, he determined that Jean needed to undergo an extremely delicate and difficult surgery to remove the clots and scar tissue. The procedure can take 12 hours, and requires stopping the heart and deep-chilling the body and brain; it’s performed in only a handful of U.S. institutions.
Fortunately, AGH recently joined that exclusive club. Dr. Benza had arranged for AGH cardiothoracic surgeon Dr. Robert Moraca to receive the months-long training necessary to perform the procedure, which took Dr. Moraca to hospitals in San Diego, Canada and Alabama.
Jean was the first person to undergo this procedure at AGH, but she says Dr. Benza’s manner helped lessen her fears. “He understood this was scary, but he explained things so I could understand them — and told me, matter-of-factly, what they were going to do,” she says. “I really wanted to know, so I appreciated that. His and Dr. Moraca’s confidence really helped.”
Jean’s surgery required a bypass machine to produce total circulatory arrest. “You can’t have any blood going through the lungs because that’s where we were operating,” Dr. Benza explains.
After her body and brain were chilled, lead surgeon Dr. Moraca opened up all the blood vessels in her lungs and carefully teased away the clots from the vessels. “Deciding what’s real tissue and abnormal tissue inside the vessels is one of the real challenges here,” explains Dr. Benza. “And if you make a mistake and puncture a blood vessel, that’s usually fatal.”
The positive results of Jean’s surgery were apparent immediately — the high pressure in her lungs dropped to normal, and her circulation responded in kind. “Although I was weak from the surgery, I remember sitting up and taking a breath and thinking, Wow, a real improvement!” Jean says. “I was only in the hospital a week, and back to normal quickly.”
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Wednesday, August 24, 2016
Medical Mystery
In June 2011, 23-year-old Lauren Cantalope, an X-ray technician in Hastings, Pa., began suffering from terrible headaches. It was the start of a medical mystery that would land her in a coma — and near death — until the tangled threads of her symptoms were unraveled by Pittsburgh neurologist Neil Busis.
Lauren’s headaches were soon accompanied by a high fever, facial numbness and severe nausea. “At first, my doctor thought it could be migraines or a stomach bug,” she recalls, “but I kept getting worse.” She had tests done at an Altoona hospital, but the results only muddied the waters, revealing that it might be meningitis or encephalitis.
A spinal tap showed inflammation and unusual cells, suggesting meningitis again — or even leukemia. By this point, Lauren had sunk into a state of severe fatigue and confusion. She was transferred to the Hillman Cancer Center, a division of UPMC Presbyterian Shadyside Hospital, for what doctors thought would be a cancer diagnosis. Dr. Busis, the hospital’s chief of neurology, was asked to consult on her case. “I didn’t think it was cancer,” he says. Instead, he suspected a benign ovarian tumor, called a teratoma, which he noticed on her CT scan might be the cause of her symptoms, albeit in a very unexpected way.
Meanwhile, Lauren’s condition became critical. “Within two days of arriving, she was comatose, on a ventilator and seizing,” says Dr. Busis. “Highly unusual for a healthy young woman.” As he was pondering the mystery of Lauren’s condition, Dr. Busis remembered a neurology lecture he’d attended a year earlier. “A light bulb went off,” he says. The speaker had briefly mentioned the very rare paraneoplastic syndrome, in which young women with tumors like Lauren’s suffered from brain disease when the tumors triggered the body’s autoimmune response.
Although only 150 cases of the disease had ever been documented, Dr. Busis realized that Lauren’s symptoms were almost identical. “The immune system fights off the tumor threat with antibodies,” he explains. “[The] problem is [that] the antibodies cross-react with normal brain tissue and make the brain sick.” Curing the syndrome called for tumor removal and treatment of the immune system. “So I called an obstetrician/gynecologist here at the hospital,” Dr. Busis says, “and told him, ‘I think the teratoma is the problem. I’d like you to take it out; it might save her life.’”
The simple operation went off without a hitch. Post-surgery treatment of Lauren’s immune system included intravenous immunoglobin to block bad antibodies and steroids. Lauren’s symptoms abated; Dr. Busis had made the right call. While she had only been in a coma for 24 hours, she did not wake up for 3 weeks after the surgery. Only then did she began to learn about all she’d been through and about the doctor who had saved her life. “Honestly, everything from that July to September is a blur to me,” she admits. “I don’t remember much about Altoona or Shadyside … or meeting Dr. Busis. But my family told me how great he was, along with everyone else at the hospital.” It took Lauren a long time to feel truly better, but given her grave illness, Dr. Busis calls it “a remarkable recovery;” she spent a month in the hospital and several more months in physical, speech and occupational therapy in Altoona. Today, she’s home in Hastings, working again and also engaged. She says “things are finally normal again.”
Lauren’s headaches were soon accompanied by a high fever, facial numbness and severe nausea. “At first, my doctor thought it could be migraines or a stomach bug,” she recalls, “but I kept getting worse.” She had tests done at an Altoona hospital, but the results only muddied the waters, revealing that it might be meningitis or encephalitis.
A spinal tap showed inflammation and unusual cells, suggesting meningitis again — or even leukemia. By this point, Lauren had sunk into a state of severe fatigue and confusion. She was transferred to the Hillman Cancer Center, a division of UPMC Presbyterian Shadyside Hospital, for what doctors thought would be a cancer diagnosis. Dr. Busis, the hospital’s chief of neurology, was asked to consult on her case. “I didn’t think it was cancer,” he says. Instead, he suspected a benign ovarian tumor, called a teratoma, which he noticed on her CT scan might be the cause of her symptoms, albeit in a very unexpected way.
Meanwhile, Lauren’s condition became critical. “Within two days of arriving, she was comatose, on a ventilator and seizing,” says Dr. Busis. “Highly unusual for a healthy young woman.” As he was pondering the mystery of Lauren’s condition, Dr. Busis remembered a neurology lecture he’d attended a year earlier. “A light bulb went off,” he says. The speaker had briefly mentioned the very rare paraneoplastic syndrome, in which young women with tumors like Lauren’s suffered from brain disease when the tumors triggered the body’s autoimmune response.
Although only 150 cases of the disease had ever been documented, Dr. Busis realized that Lauren’s symptoms were almost identical. “The immune system fights off the tumor threat with antibodies,” he explains. “[The] problem is [that] the antibodies cross-react with normal brain tissue and make the brain sick.” Curing the syndrome called for tumor removal and treatment of the immune system. “So I called an obstetrician/gynecologist here at the hospital,” Dr. Busis says, “and told him, ‘I think the teratoma is the problem. I’d like you to take it out; it might save her life.’”
The simple operation went off without a hitch. Post-surgery treatment of Lauren’s immune system included intravenous immunoglobin to block bad antibodies and steroids. Lauren’s symptoms abated; Dr. Busis had made the right call. While she had only been in a coma for 24 hours, she did not wake up for 3 weeks after the surgery. Only then did she began to learn about all she’d been through and about the doctor who had saved her life. “Honestly, everything from that July to September is a blur to me,” she admits. “I don’t remember much about Altoona or Shadyside … or meeting Dr. Busis. But my family told me how great he was, along with everyone else at the hospital.” It took Lauren a long time to feel truly better, but given her grave illness, Dr. Busis calls it “a remarkable recovery;” she spent a month in the hospital and several more months in physical, speech and occupational therapy in Altoona. Today, she’s home in Hastings, working again and also engaged. She says “things are finally normal again.”
When you have rearest Cancer in the world.
After experiencing serious food poisoning for several days, Susan Killmeyer knew she needed to visit UPMC Presbyterian Shadyside Hospital, where she works as a clinical director.
There in the ER, a CT scan of her abdomen revealed a danger much greater than a routine case of food poisoning: a cancerous tumor on her inferior vena cava, the vein that carries blood from the lower half of the body into the heart.
The food poisoning may have saved her life, says Dr. David Bartlett, a cancer surgery specialist at UPMC CancerCenter. “Her cancer is among the rarest — [it affects] about five in a million,” he says, “and it takes so long to develop symptoms that by the time it does, it’s usually beyond what we could treat.” Dr. Bartlett, along with a team that included Dr. Herbert Zeh, a cancer surgeon at UPMC CancerCenter, determined that Susan would have to undergo a long, complex operation to remove the tumor. “It wasn’t easy news to hear,” she says, “but I’m a take-charge person — I don’t feel sorry for myself. So from my hospital bed I compiled a list of physicians for my ‘team’ — and Dr. Bartlett was on it. I knew he was a good surgeon.” “Her tumor was in a tricky area and involved her liver, so to reach the tumor, we had to take out the vena cava,” Dr. Bartlett says.
This created complications, including problems with blood pressure and clotting as well as serious bleeding — all of which put Susan’s brain at risk. Throughout the nine-hour operation, continuous efforts to keep her alive were made — including massive transfusions of blood products, and both medication and intermittent clamping of the heart’s main blood vessel to maintain pressure to the brain. “It was a Herculean effort by a large team,” says Dr. Bartlett.
The surgery was a success, but Susan was back in the operating room several hours later because of bleeding. More surgeries followed, including one where she was so unstable that she couldn’t be moved from the ICU. “So we did the operation there,” Dr. Bartlett says. Multiple surgeries contributed to Susan’s kidneys failing, while the length of her sedation led, temporarily, to total body paralysis. “Her case was unique,” says Dr. Bartlett. “She seemed to have every problem you could have.” Susan was in the hospital for three months. “The day I left, I could barely sit on the side of the bed,” she recalls. “I had to learn to feed myself and to walk again.” Her recovery was arduous, but Susan was able to return home (and get back to work). “The psychological aspect of a recovery like Susan’s is huge,” Dr. Bartlett, says, “and she was very strong.
Even when paralyzed — her kidneys failing — she maintained good spirits.” Thinking about her adult twin daughters gave her strength, Susan says. “My husband died 17 years ago, and there was no way in hell I was leaving my kids without a parent.” Susan’s short-term goal was to see her girls get married. “One daughter’s wedding was last October and I was able to walk her down the aisle,” she says. “My other daughter is getting married this month, so I’ll be doing it again.” “It’s rewarding to take a challenging case like Susan’s and give her hope for the future,” says Dr. Bartlett. “What allows us to do it are all the people and resources at this hospital — from the anesthesiology department to the ICU nurses and many others. The whole system supports a case like this.
There in the ER, a CT scan of her abdomen revealed a danger much greater than a routine case of food poisoning: a cancerous tumor on her inferior vena cava, the vein that carries blood from the lower half of the body into the heart.
The food poisoning may have saved her life, says Dr. David Bartlett, a cancer surgery specialist at UPMC CancerCenter. “Her cancer is among the rarest — [it affects] about five in a million,” he says, “and it takes so long to develop symptoms that by the time it does, it’s usually beyond what we could treat.” Dr. Bartlett, along with a team that included Dr. Herbert Zeh, a cancer surgeon at UPMC CancerCenter, determined that Susan would have to undergo a long, complex operation to remove the tumor. “It wasn’t easy news to hear,” she says, “but I’m a take-charge person — I don’t feel sorry for myself. So from my hospital bed I compiled a list of physicians for my ‘team’ — and Dr. Bartlett was on it. I knew he was a good surgeon.” “Her tumor was in a tricky area and involved her liver, so to reach the tumor, we had to take out the vena cava,” Dr. Bartlett says.
This created complications, including problems with blood pressure and clotting as well as serious bleeding — all of which put Susan’s brain at risk. Throughout the nine-hour operation, continuous efforts to keep her alive were made — including massive transfusions of blood products, and both medication and intermittent clamping of the heart’s main blood vessel to maintain pressure to the brain. “It was a Herculean effort by a large team,” says Dr. Bartlett.
The surgery was a success, but Susan was back in the operating room several hours later because of bleeding. More surgeries followed, including one where she was so unstable that she couldn’t be moved from the ICU. “So we did the operation there,” Dr. Bartlett says. Multiple surgeries contributed to Susan’s kidneys failing, while the length of her sedation led, temporarily, to total body paralysis. “Her case was unique,” says Dr. Bartlett. “She seemed to have every problem you could have.” Susan was in the hospital for three months. “The day I left, I could barely sit on the side of the bed,” she recalls. “I had to learn to feed myself and to walk again.” Her recovery was arduous, but Susan was able to return home (and get back to work). “The psychological aspect of a recovery like Susan’s is huge,” Dr. Bartlett, says, “and she was very strong.
Even when paralyzed — her kidneys failing — she maintained good spirits.” Thinking about her adult twin daughters gave her strength, Susan says. “My husband died 17 years ago, and there was no way in hell I was leaving my kids without a parent.” Susan’s short-term goal was to see her girls get married. “One daughter’s wedding was last October and I was able to walk her down the aisle,” she says. “My other daughter is getting married this month, so I’ll be doing it again.” “It’s rewarding to take a challenging case like Susan’s and give her hope for the future,” says Dr. Bartlett. “What allows us to do it are all the people and resources at this hospital — from the anesthesiology department to the ICU nurses and many others. The whole system supports a case like this.
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