Part two of a two-part series
Many people, if given the chance to choreograph their death, would probably envision going gently into that good night by drifting off peacefully in their sleep, with no cares, regrets or pain, after having lived for several decades, even a century or so.
That option fades away for those with diseases and conditions doctors have given them little or no chance of surviving stage 4 ovarian cancer, tuberculosis, congenital diaphragmatic hernia, an omphalocele, stage 4 leiomyosarcoma.
Newsday asked readers given these and other diagnoses to share their tales of long-term survival, which doctors and surgeons didn't think possible for most of them.
"I was told I had three months to live," wrote Janet Shavel of Seaford, diagnosed in 2008 with stage 4 leiomyosarcoma, or soft-tissue cancer. She had 22 tumors, including one on her uterus that was the size of a basketball. "I was in shock .?.?. my sons, my mother, we felt like someone just stopped the merry-go-round of life and I was told to get off."
But she survived, as did Ali Bie, who was born in 1992 with her abdominal organs outside her body. An omphalocele meant surgery when she was just 2 days old to correct the condition. Bie weighed less than 3 pounds at birth, and, like up to 40 percent of afflicted babies, she had other birth defects — a large hole in her heart that required surgery when she reached 5 pounds.
In the two-part series "Living Proof," Newsday profiles 14 Long Islanders, "walking miracles" who faced down death, fought for their lives and won.
Anna Kaplan, 60
Diagnosis: Ovarian cancer
Prognosis: 50-50 chance of survival
Outcome: 10-year survivor
Turning 60 recently was something of a cakewalk for Kaplan — she spent her 50th birthday in the hospital being treated for ovarian cancer, wondering if she'd live to see another birthday.
This month, Kaplan, a professor at Five Towns College in Dix Hills, is celebrating her 10-year anniversary of being cancer-free. "I'll take it," she said. "Another year above ground and with your health."
The Merrick resident credits her survival to a certain amount of luck, support and to being aware of possible symptoms that sent her in for an early checkup that caught the cancer before it progressed too far.
"I don't know if I'd be here if I didn't," Kaplan said. "My mother had her head in the sand. She kept it quiet, she didn't tell anybody when she found a lump. My mom was 58 when she died of breast cancer. My radar was up after that."
Kaplan, who is of Ashkenazi Jewish descent, said she didn't test positive for the BRCA1 genetic variation that can indicate an increased risk of cancer.
"I was so alert to the symptoms because of Gilda Radner and other women who died of it," she said, referring to the "Saturday Night Live" comedian who died of ovarian cancer in 1989. So when her abdomen was bloated and distended two months running around the time of her monthly period, but then fine as soon as it was over, Kaplan went to the doctor. Within a week, she was scheduled for surgery to remove a hormone-charged tumor.
During six rounds of chemotherapy, she said her golden retriever, Normandy, wouldn't leave her side for three days after each treatment until she started feeling better.
Her attitude wasn't good the first year, Kaplan said, but she worked at improving it for the sake of her children, Jaine, who was then 24, and David, who was 17. "My husband, Dennis, helped me to see the glass as half-full instead of half-empty," she said. "You have a 50-50 chance. It was hard to see that going through it."
Kaplan was lucky and came out on the positive side. "I'm not paralyzed by it now," she said. "When you finish chemo then you're afraid, because the chemo was your safety net. But the further out I get, I can say, 'Maybe I am on the other side of that 50 percent.' "
To help others, she wraps knitted and crocheted blankets that the group We Care Blankets distributes to children undergoing chemo. Recently she donated about 9 inches of her hair to make wigs for other patients undergoing chemo. She now goes for what she refers to as "necessary evils" at regular intervals — mammograms and colonoscopies — as well as CAT and PET scans and blood tests to check her CA-125 level, a tumor marker.
Kaplan exercises and tries to watch her weight. "But life is short," she said. "I leave room for dessert."
Janet Shavel, 64
Diagnosis: Stage 4 leiomyosarcoma
Prognosis: 3 to 6 months to live
Outcome: 5-year survivor
January will mark five years since Shavel was diagnosed with stage 4 leiomyosarcoma and had surgery to remove a basketball-sized uterine tumor and an unrelated fibroid, as well as tumors on her intestines. But the cancer didn't stop there; it invaded her lungs and heart cavity with 22 tumors.
"We expected uterine cancer, but I don't think the doctor expected it to be in my intestines and all through my lungs," she said. When the doctor told her there was little Shavel could do and hospice was called in, Shavel, now 64, decided she had to get a second opinion.
She asked Dr. Stuart Lichtman at Memorial Sloan-Kettering Cancer Center in Commack to try to find a chemotherapy that would work. The odds were not in her favor. Lichtman told her only 15 percent of patients survive her diagnosis. With help from the cancer center's researchers in Manhattan, they came up with a combination of three drugs. "I told him, 'I'm going to be your first miracle. I am going to live,' " she said.
"The chemo was brutal, like the worst flu you can imagine," Shavel recalled. When she was over the worst of it six months later, the first place she went was to church. "I have very strong faith," she said. "I was in prayer a lot."
She also credits prayers from congregants at St. Rose of Lima in Massapequa, where she was on the prayer list for six months.
The cancer was no match for some quality care, a particularly inspiring oncology nurse, Shavel's faith and what she calls nothing short of a miracle. CT scans show there is no active cancer, and Shavel considers herself the one-in-a-million who has beaten the diagnosis.
Shavel, mother to three sons — one of whom died the same week she buried her mother — shares her story with others, including cancer patients whom she urges not to give up. She is aware the cancer could return, and that she would have to fight her way through it again.
"Cancer is a disease, not a death sentence," she said. "It's all about the fight and the mind. Now it's time for me to pick up my life and dust myself off."
As more people survive cancer, those who work with cancer patients should redefine recovery, Shavel advises. "We need rehab and recovery that covers body plus brain and motor skills," she said. She still has some short-term memory loss from the chemo and said she needs "a little more time to think." She also needs help with heavy lifting and time to catch her breath.
"I haven't had my fun yet," she said. "I am so happy to be alive."
Taylor Zoitos, 10
Diagnosis: Congenital diaphragmatic hernia
Prognosis: 50 percent survival rate
Outcome: 10-year survivor
Taylor is a bubbly fifth-grader who likes playing with her dogs, Dexter and Penelope, and making bracelets for others.
She also knows a thing or two about medical conditions. When asked, she succinctly describes the birth defect she survived but is fatal for half of children born with it.
"When I was born there was a giant hole in my diaphragm, and my organs went into my lungs, my intestines and my spleen. My kidneys stayed where they belong."
Taylor, of Hicksville, had a congenital diaphragmatic hernia. It occurs when the diaphragm, the muscle that separates the chest and abdominal cavities, doesn't close properly and abdominal organs go into the chest cavity and prevent the lungs from developing as they should.
Her mother, Tara Zoitos, then 22, was told her baby's prognosis was "very poor."
"They told me that 50 percent die," she said. "I felt like nobody has a guarantee, even healthy babies, so I did more research and got more information."
Nowadays, 10-year-old Taylor, an only child, tells people she has asthma and needs a nebulizer and other treatments, said her mother, who went to school when Taylor was 4 and is now a registered pediatric nurse.
Taylor's left lung didn't grow, so her right lung does a lot of work. For most people, Tara Zoitos said, taking a breath is like breathing through a regular straw. "Taylor breathes through a coffee stirrer straw" by way of comparison, she said. Taylor has some issues with stamina in the summer when it's humid and has some limitations, her mother said, but she takes gym and is in kickline.
Since Taylor has never known anything different, that's just how it is. At Woodland Elementary in Hicksville, she likes science courses -- "Life science is my thing, plants and animals," she said — and takes saxophone lessons in the school music program.
"The saxophone gives my lungs a little exercise," Taylor said. She takes 10 medicines a day and "a little machine helps me breathe sometimes," she added. "When I grow up I'm going to be a doctor. I love helping others."
In the meantime, she's helping raise money for the Association of Congenital Diaphragmatic Hernia Research, Awareness and Support, or Cherubs, a nonprofit that offers support and lobbies for more study of the condition. She makes and sells bead bracelets, and takes orders at saveacherub@ yahoo.com. This year she raised $5,000 for the charity through a grant, plus $240 selling bracelets and $500 in raffle ticket sales.
Taylor and her mom, who calls her daughter an "old soul," work hard not to let the disease define her. But it does have another effect, according to Taylor's mother.
"It empowers her."
Mary Taylor Joyce, 75
Diagnosis: Pulmonary tuberculosis
Prognosis: 3 months to live
Outcome: 58-year survivor
In 1954, Joyce was a busy high school junior, traveling across the city from Brooklyn to attend a school in Queens on a scholarship.
She was hospitalized briefly for removal of a cyst on her neck, then returned full-tilt to her hectic academic and social schedule. But within a few months she began to tire easily and was coughing up blood. She needed a nap before dinner. By June, the family doctor ordered X-rays.
The diagnosis was pulmonary tuberculosis in all the lobes of her lungs. Dr. Ralph Romano, chief of pulmonology at Kings County Hospital Center in Brooklyn, told Joyce she also had a crater the size of a 50-cent piece that could cause her to bleed to death with any coughing fit.
"I had no idea I was that sick," said Joyce, who lives in Garden City.
She rebelled at being sent to a sanatorium (a facility for the chronically ill) in the Adirondacks, so a compromise was reached: Three times a week Romano journeyed across Brooklyn to give Joyce injections of streptomycin, the first antibiotic to be used against tuberculosis. After three weeks of injections, she had an allergic reaction. "I remember saying, 'Doctor, I'm leaving,' " she said. Romano ran to his car and came back with adrenaline to open her airways.
"I felt I had watched all this action from somewhere up on the ceiling — and nothing hurt," Joyce recalled. She remembers her mother crying at the foot of the bed. "Then boom! I was in my bed again and talking to the doctor. Who can explain it? I can't."
Romano recommended a new drug that had just been approved, INH, and she took it three times a day along with para-aminosalicylic acid tablets that helped inhibit the growth of tuberculosis bacteria. "In three weeks I went from a 95-pound, 5-foot-7 girl who couldn't get comfortable and couldn't breathe to no pain, no discomfort, no cough and sleeping like a baby all night," she said.
Standard treatment then included bed rest for a year. After she responded to antibiotics, Joyce's friends were allowed to visit. "They kept me up on all the news. They're still friends to this day," she said.
Joyce said she ignored her doctor's orders to stay away from boys for a while and was married and the mother of three by the time she was 25.
"Each baby made me stronger," she said.
She went to college when her fourth child entered kindergarten, and she became a registered nurse at 39. Between semesters in nursing school at Nassau Community College, she had her fifth child.
"When I was diagnosed with TB, nobody called it that," Joyce said. "They said, 'You have a spot on your lungs.' I never talked about it. It's time to talk about it now."
Joyce, now 75, said she is thankful for her full life, family, nine grandchildren and "the world's best husband for 54 years. I'm really very, very fortunate."
Flo Puff, 70
Diagnosis: Acute lymphoblastic leukemia
Prognosis: 50-50 chance of survival
Outcome: 36-year survivor
Puff enjoys taking cruises, never missing a Yankees game, and going to hear country singer Willie Nelson when he plays a concert nearby.
A new outfit each Easter used to be a must for her. But cancer changed all that.
"I haven't had a new Easter outfit in 36 years," she said. "Who cares? After you survive a cancer diagnosis, it's not important. You enjoy today, get the most out of it."
Puff, of Merrick, retired from teaching 10 years ago. She works part time for the Nursing Sisters of the Sick Poor in Rockville Centre.
Her fourth-grade students at the former St. Kilian's Catholic elementary school in Farmingdale helped keep her focused when she was undergoing 3 1/2 years of treatments starting in 1976, she said. She also credits prayer and the support of her mother and brother, co-workers and students' parents, and a medical team that combined doctors from Memorial Sloan-Kettering Cancer Center in Manhattan and from North Shore University Hospital in Manhasset. The cancer center is where she began treatment after she got a third opinion and the diagnosis of the type of leukemia she had; doctors at North Shore worked with the established treatment protocol since Puff couldn't make the trip into the city while she was working.
Puff, now 70, said she believes being anointed each month by her school's pastor played a big role as well. "How you persevere, the resilience you have — it comes from those around you," she said. "I was in constant prayer myself."
She had some side effects from the weekly chemo treatments, which included 60 spinal taps to deliver the medication, but missed relatively few days of school; the most was 11 days one year. "I was able to take the medication, I never got an infection and I never [had] pneumonia," she said. "I was very fortunate."
Puff would leave school and have chemo at 3:30 p.m., get home by 5:30 p.m., where she would be ill every 15 minutes until about 12:30 a.m., then be up at 6:30 a.m. to get ready for school. The day after a treatment she could stomach only tea and toast, she said, but by the second day she could eat regular food. She lost her hair, had sores in her mouth and had difficulty walking in the beginning, because the chemo affected her reflexes.
"My mother and my brother were my strength," she said. "Never did they treat me as a sick person."
She hopes hearing her story of long-term survival will help someone else. She remembers well reading a survivor's story in 1976, when she was diagnosed, about a girl with the same kind of cancer who had been in remission for eight years.
"The article brought me out of depression and gave me hope and determination," Puff said. "It turned me around. I thought, 'If she can do it, I can do it.' "
Gary Klausner, 47
Diagnosis: Cystic fibrosis
Prognosis: 6 months when added to lung transplant list
Outcome: 14-year post-transplant survivor
Klausner ran a 5-kilometer race and a few weeks later did a 5-mile race in the summer of 1999. Not remarkable, perhaps, but Klausner did the runs after returning home from a double lung transplant at Duke University Hospital in North Carolina.
The 47-year-old Merrick resident said life is good. He and his family rented an RV for a vacation out West this past summer and visited the Grand Canyon, Hoover Dam and other sites. He's active playing basketball and tennis and going to the gym, and coaches the sports teams of his twins, Matthew and Steven, who just turned 14.
Klausner makes as many memories as he can with his wife, Robin, and sons, to go with the tape he made the boys before he left to get the transplant, hours after their bris, when he didn't know whether they would get to know him. And he has written a memoir, "Never Say Never: A Life of Challenges," released this year.
The lung transplant has enabled Klausner to beat what the Cystic Fibrosis Foundation website says is a median survival age of the late 30s for those with the disease. Klausner was diagnosed when he was 10; at that time the median survival age was 16.
The disease causes the body to produce thick, sticky mucus that can build up and block the lungs and the pancreas, the organ that secretes digestive enzymes. He takes pride in helping forge a new medical path.
"I'm the guinea pig — here's a 14-year postoperative experience," he said. "I've been blessed, and I try to share my story."
Not a lot was known about cystic fibrosis when Klausner was diagnosed. Everyone thought he had severe allergies, he said. He went to school, even played tennis and basketball in high school and college, married and lived in Manhattan. Then he became increasingly ill, his blood oxygen levels dropped and he was hospitalized for more than a month. He was told he needed a double lung transplant to survive.
Duke was one of the few hospitals that would evaluate him for the operation because he was infected with a bacterium, B. cepacia, that is resistant to many antibiotics and can worsen lung diseases. According to Cystic Fibrosis Foundation estimates, about 2.7 percent of those with the disease have the bacteria.
Klausner used to attend cystic fibrosis events and share his story, but the foundation no longer permits those infected with the bacteria to participate in live events, to prevent
spreading the infection.
He exercises, washes his hands frequently to prevent infections, takes all his medications — including insulin four times a day — and returns to Duke three or four times a year for routine checkups, but he doesn't live in a bubble.
"I've had a very blessed life," Klausner said.
Kimberly Holland, 42
Diagnosis: Stage IIB Hodgkin lymphoma
Outcome: 18-year survivor
Holland is a poster child of sorts, not necessarily for a cause she ever imagined she'd support, but one that she's happy to still be part of.
Holland, 42, of Massapequa, is being treated for Hodgkin lymphoma, a cancer of the lymph tissues. She was diagnosed in 1994. About 88 percent of patients are cured after the initial treatment. Holland is part of an even smaller subset of the 12 percent who aren't cured; she is among the 25 to 30 percent of that 12 percent for whom the disease does not respond to treatment.
"You have to keep getting chemo," she said. "It's going to just keep coming back."
Holland participates in clinical trials so she can keep being treated. She has had two autologous bone marrow transplants, using her own bone marrow cells to try to rebuild her immune system. Doctors wanted to do an allogeneic transplant, using cells from a donor, but after evaluating the options Holland chose not to do that type of transplant because she would become ineligible for many clinical trials.
"It's not a risk I want to take," she said. "I'd rather just roll the dice."
Attitude is key, she said. "If you get down when you have cancer, you're finished. It's crazy that I'm still here. I think 75 percent of it is the attitude."
She never goes for a treatment without making sure she looks as good as she can. "You make the effort," Holland said. "You have to."
Her mother and sister support her in her fight, along with her husband, Paul Falbo, who she notes married her knowing she had cancer. "It's a huge commitment when you're marrying someone with cancer," Holland said. "I married into a huge Italian family. They're absolutely amazing."
Her medical decisions are hers alone, though. Right now, Holland is being treated for a persistent cough she developed in September. "They've tested me for whooping cough and Legionnaires' disease," she said. "They tell me, 'You could be having this cough from something you took 10 years ago.' They don't know."
Holland has a deep understanding of clinical trials, where experimental drugs and treatment plans are tested, and their different phases. "That's why I started my foundation. I'm only kept alive on trials."
She looks for sponsors for the Kimberly D. Holland Hodgkin's disease Foundation, hodgkinsfoundation.org, and holds fundraisers when she's feeling well enough to organize them, then donates to the research and clinical trial team at Memorial Sloan-Kettering Cancer Center in Manhattan. Since 2001 she has been able to donate $50,000.
In 2009, after nine treatments with SGN-35, a drug the Food and Drug Administration approved in August 2011 and one that she responded well to during a clinical trial, she's scheduled for a PET scan, after which doctors will discuss her options.
"It [the drug trial] gave me one year of not having to do anything before my tumors became too big where I had to do something again," Holland said.