For two decades, cystic fibrosis placed a time limit on the Turcotte family. A lung transplant has allowed them to contemplate a future.
Veronica Turcotte asked her son Eugene only once if he was prepared to die.
She posed the question in 2010, during a particularly dark skirmish in his long battle with cystic fibrosis, inefficient but rapacious killer of children and young adults, when the last weapon remaining in the medical arsenal was a lung transplant that might or might not be available while he slowly slipped into submission.
He was 19, an age when his mates were contemplating life, not death. But the latter was a topic never far from the Turcotte family’s thoughts ever since his older brother Maurice was diagnosed with CF at nine months old and Eugene still in his mother’s womb.
With his birth in 1991, Veronica, then in her mid 20s, was suddenly a mother of three children: a healthy girl of 2½, and two sons not expected to reach their teens.
How do you measure strength of heart, the will to persevere against adversity? Is courage benchmarked in the Utah desert when an arm must be severed to survive? Is it metered by those final few steps approaching exhaustion on the peak of Everest? Or is it something much closer to home; an ability to accept what you cannot change and keep on throwing punches against the challenge?
For the Turcotte family, the measurement is not so much one of fortitude, although when you’ve finished this story, you’ll realize a vast amount of it was required. It is rather one of solidarity. It’s a story of a family that relied on each other to stay afloat on a river of medical misfortune. And, while the story isn’t over, they are approaching higher ground than they’ve enjoyed for many years.
Veronica Gregoire, mother, Saskatchewan farm girl, Lethbridge College alumna (Office Administration, ’08) and now a fixture in the college’s Advancement Office, married John Turcotte in 1987. The two settled in Taber where all three children were born. Suzie came along on July 6, 1988, healthy, beautiful and destined, at 20, to become a fellow Lethbridge College grad and, after completing her education at the University of Lethbridge a year ago, a registered nurse.
There was little reason to suspect a second child, Maurice, would be anything but perfect, and he was born just that way Sept. 16, 1989. But by nine months, he had been diagnosed as a “failure-to-thrive” baby, unable to gain weight. Shortly after, during a visit to a pediatrician as the family searched for answers, the doctor asked Veronica if she could “lick” her son.
“It was a strange request, but I said sure,” says Veronica. “What she was looking for was a salty taste on his skin, one of the symptoms of CF. She said ‘I think your baby has cystic fibrosis.’”
The diagnosis couldn’t have come at a more frightening time: she was three months pregnant with Eugene. Could there be a chance he, too, could have cystic fibrosis? It’s a question many parents would ponder long into the night, but Veronica and John knew they would have their third child regardless of the risk.
“I wouldn’t wish CF on anyone,” says Veronica. “But I’d make the same decisions again, based on my faith and who I am.”
When Eugene was born at 4:30 a.m., Jan. 6, 1991, he appeared normal and healthy, but within two hours he was suffocating and was taken from Taber to Lethbridge and, ultimately, to Alberta Children’s Hospital in Calgary. At 36 hours old, he was operated on to remove half his small intestine, the first of a multitude of hospital stays in his future.
“It was pretty grim,” says Veronica. “The surgeon told us Eugene’s intestine was falling apart in his fingers. It was knotted, stopping the blood flow, one of the leading indicators of cystic fibrosis.” So, you’ve got a toddler and an infant, the latter with cystic fibrosis, and now you’ve discovered your newborn has CF, too. Oh, and you’re only 27.
“It’s hard to have a normal life when you know your children are going to die,” says Veronica. “It’s always in the back of our heads. This was 20 years ago, when CF almost always led to an early death, and not a particularly nice one, either.”
Beyond the drugs (enzymes and vitamins) taken in handfuls each day to aid digestion, and inhalers to keep the lungs clear, the only other thing the family could help with was “postural drainage,” a procedure which involves thumping your child on the front and back to knock the fluid loose in the lungs, like whacking the sediment free in a water heater. It wasn’t what one might call a warm, fuzzy time.
“John really struggled with it,” says Veronica. “Here he had a lovely, healthy son and then suddenly he became a son with a death sentence hanging over him. For myself, I became overly protective. Any decisions I made in my life were made with cystic fibrosis as a component.”
Cystic fibrosis is one of those double-word, monsters-under-the-bed conditions like multiple sclerosis, muscular dystrophy or cerebral palsy. It required an encyclopedia to get a grip on it, and that hold wasn’t particularly reassuring.
“You think ‘what is this?’ You know it’s deadly, but you don’t know how bad it is until you look it up and read that it’s the most common fatal genetic disease in Caucasian males.”
The perfect CF storm in the Turcotte family was caused by both parents being carriers of the CF gene, almost unknown at that time, but now better understood. One parent a carrier: no harm, no foul; both parents carriers: a one-in-four chance of a CF baby.
Still, there wasn’t much time for whining, even if she had wanted to give into it anyway, because young Eugene was going to spend the first four months of his sure-to-be-abbreviated life in the Alberta Children’s Hospital in Calgary. Veronica needed to be with him. Fed through a nose tube, pumped with drugs, Eugene was ultimately able to return to Taber, and Veronica settled into the routine of raising her CF sons and a vibrant daughter. Time passed. The family moved to Wrentham in 1999, with Veronica home-schooling her children, partly because Suzie was diagnosed as dyslexic and required extra help reading, and because of the threat to the boys of school-bred diseases.
Cystic fibrosis is a degenerative condition, manifested by a steady decline in health; all treatment does is manage the decline. Eugene and Maurice have different CF gene “modifiers,” meaning their conditions affect them at different rates. Maurice has been in hospital just once, though he is required to follow the same regimen of drugs as his younger brother.
Still, in Wrentham, both boys experienced a relatively normal childhood. Eugene hunted, fished, took tae kwon do, tore around on a quad and swam; a rather normal slate of activities for a young, rural teenage boy. Sure, he required pills and inhalers to aid digestion and keep his lungs from clogging into sludge, but at least, until he hit 16, he could keep up a fairly good performance as a normal kid.
Then came puberty, rough enough on any 16-year-old, but devastating on a boy who, while his friends were pondering their first cars, spent long hours wondering about death and dying. Because both were in his immediate future and it’s hard to get hyped about the “rest of your life” when it might only be a few months long. Dark days set in, adding depression to all the medical malevolence.
The family moved to Lethbridge in 2007 and Eugene attended Winston Churchill High School. But he remained a frequent guest at Children’s in Calgary all through high school, requiring hospitalization every six months or so at the start, then with increasing frequency. When most Grade 12 grads were being measured for tuxedos in 2009, Eugene was dealing with a collapsed lung, followed by a bout of the H1N1 flu.
He was vulnerable to almost anything microbial. He couldn’t see the threats, like one might spot grizzlies or drunk drivers or falling pianos, but he knew they were there and were as unpredictable.
“I was extremely gaunt,” Eugene says now. “My ribs showed; my face was sunken.”
He was, to be blunt, running out of hope and low on desire to continue punching back. When he required a G-tube, required for feeding directly into the abdomen, Veronica knew he was on the short track.
On Aug. 28, 2009 one of those many Turcotte “days of infamy,” doctors at Foothills Hospital called the family together for an intervention. Eugene, they said, was deteriorating quickly. Time was drawing near where he would either have to step back into the ring or toss in the towel and prepare for the end.
“It was an emotional, terrible day,” says Veronica, who, on the drive to Calgary, told her family her cancer symptoms she once thought were behind her, might be returning. “I had told them I would never lie to them. It was a solemn family journey. Later that day we found out that Maurice had CF-related diabetes and later that month we found out that Terry, Suzie’s fiancé was also a carrier.”
By February, the only answer was a transplant. Eugene accepted the offer.
Being a candidate for a lung transplant means being stranded somewhere between a rock and a hard place. First, you must be physically capable of surviving the operation, which can take eight hours, stops the heart and generally messes with the body. However, you must also be within 18 months of death to qualify. It’s like blowing your life savings on a lottery ticket with no job and the rent due at the end of the week: hey, might work out, chances slim.
“When they counsel you on an organ transplant, there’s no pussy-footing around,” says Veronica. “You have to be psychologically prepared for it, as well as physically.”
In June 2010, Eugene began a series of tests to determine if the venture was possible. His heart was examined, his bone density scanned and his head was read to help doctors decide if he was a good candidate for having his lungs ripped out and replaced.
A month later, he attended sister Suzie’s wedding. Two days after the July 17 nuptials, he entered a six-week physical training program in Edmonton to reach a required biomass index of 18 per cent (he was at 16) and a weight of 110 pounds. Because muscle weighs more than fat, the pasta was passed over for push-ups. After Edmonton, Eugene worked out daily at the Lethbridge College Physical Education facility. With his body on the build, he was now in a chess match with time. “I knew this past winter would be it,” says Veronica, looking back. “I knew he’d either have a new set of lungs or he’d be dead.”
At the time, Eugene was one of 60 Albertans on the registry for new lungs; in the six months leading up to his discharge, six of those died waiting. Only 40 to 45 are done annually in the province, and summer is a busy time, what with all that vehicular traffic providing donors. And long weekends and, especially, New Year’s Eve.
“From May to July and on long weekends, transplant surgeons don’t take holidays,” says Veronica.
There are four such surgeons at the University of Alberta Hospital, a facility that has become the transplant centre for Western Canada.
It’s easy for the Turcottes, of course, to remember the day the call came; for anyone else, too: 10/10/10, Thanksgiving Sunday. Veronica was about to put the turkey in the oven when the phone rang, Edmonton on the line. By 1 p.m., mother and son were on a Medivac flight; three hours after that, Eugene was in surgery at the University of Alberta Hospital.
“I had no second thoughts,” says Eugene. “I knew I would need this operation since I was 17.”
It would be somewhere east of 1 a.m. before Veronica would see her son again, swaddled in the ICU with enough IV poles, monitors and respirators to run a MASH unit. “I wish I had broken the rules and taken a photo,” she says now. “He looked like this little mummy with a mask on.”
She had spent the 8½ hours of his surgery mostly alone, although one relative dropped by to sit with her. “I actually wanted to be alone. If things went well, I’d be able to let them know back home as soon as he was out of surgery. If he died, I’d have time to collect my thoughts before giving them the news.”
What she didn’t know, as she waited through the long afternoon and night, was that the surgeon was also watching the clock. Eugene’s lymph nodes, normally thumb-sized, were the size of walnuts due to long-term infection, making it difficult to remove the damaged lungs behind them. As he told Veronica later, he was afraid he’d run out of time making the switch. To beat the clock, he simply cut through the lymph nodes and finished his work.
At about 1:30 a.m. Oct. 11, she was able to text the message home that all was well.
On a respirator and resembling an Egyptian artefact, Eugene was starting a new chapter in his life. He remained in hospital for 19 days on the transplant ward and then another eight weeks in the Outpatient Residence, (long enough to learn from a Hutterite woman how to knit, which he did while spending time on an exercise bicycle). Once discharged, however, he soon found his second wind.
Transplant surgeons use organs harvested from across North America. Many western Canadian recipients find themselves with replacement parts from sunny California, with its large population, proximity to the west and penchant for automotive derring-do. After receiving a letter from the donor family, Eugene found out the lungs were from a teenaged girl. She would have graduated high school this June.
“I felt sorry for Gene,” says Veronica. “Finding out put a lot of pressure on him at a stressful time. Of course, I think about that family and what they lost, and I’m grateful.”
As the numbers would have it, the average age of CF male lung transplant recipients in Alberta is above 30; according to the clinic, women with CF tend to require transplants earlier because they develop less muscle mass through puberty, and often have accompanying psychological factors and poorer diets.
Upon return to Lethbridge, free from the 12-hour days of intravenous, morning and evening, to kill off infection and give the new lungs a chance to settle in, Eugene began to enjoy the simple pleasures of being able to walk a block without wheezing, and eating. Oh my, yes, the eating.
“Like a horse,” his mother says, and not unlike Maurice, an observer might suggest. “He wolfed down an entire sub in his hospital bed. I’ve never seen him eat like that.”
Food, for Eugene and Maurice (21 and 115 pounds) is a lifeline. They require 3,000 calories a day (5,000 is preferred, more than twice the recommended amount for a 20-year-old of Eugene’s weight and height.)
Eugene is still heavily into drugs (three kinds of anti-rejection medication, plus antibiotics and calcium, all laid out in a tray a fisherman might use for favourite flies. He is checked out monthly, although if his progress continues unabated, the frequency of the visits will decrease sharply. He measures his new lungs’ ability to exhale; a low rate four days in a row is a signal of possible rejection. He also self-measures his blood pressure, temperature and weight. No one is allowed to touch the equipment for fear of contamination. There remain, says his mother, a lot of ‘don’t’s in his life.
He sports a scar from sternum to belly and several drainage holes for additional adornment. But he has new-found energy and has become, says Veronica, a poster boy for transplants. He’s even added three centimetres in height and left his physiotherapy program more than two weeks early. He has the vitality to keep up with Pixie, his Yorkshire terrier, introduced to the family originally to help Eugene deal with his depression.
A small coterie of high school mates remain among his closest friends. They have, says Veronica, surrounded and protected him. Other acquaintances, sadly, had to be jettisoned because Eugene grew tired of sating their curiosity about his imminent death. Whether morbid teenage curiosity or heartfelt concern, it was all getting a little too heavy to bear.
He is holding his cards close for the first year, but would eventually like to travel; Australia is a first choice: the beaches, the girls and the first-rate medical care should he require it.
While Suzie admits to feeling some guilt for being the one healthy Turcotte child, she did not escape the stress of the family’s situation. She took a year off from working and just concentrated on her studies to deal with the pressure. “It’s a ‘family’ disease,” she says of CF. “It hasn’t identified who we are, but it has played a role in defining us. But we’re survivors; we’re a close family.”
“Something like this makes you a tight-knit family,” says Veronica, who considers Suzie her best friend, someone who has been at the epicentre since the beginning. “It’s always been the five of us.”
Yes, cystic fibrosis has taken a toll on their marriage. But Veronica and John have survived together, even if they took different routes. The divorce rate is five times higher in couples with a terminally ill child.
Would she do it all over again if she could wind back time? Would she run the risk of watching her babies die as teens or in early adulthood, living with the knowledge their lives would be cut short?
“No,” says Veronica. “But I wouldn’t have changed what happened.”
Still, the stress has been, as she says, ‘out of this world.” She maintained a full-time job at the college until the phone rang that day last October, and returned to work early in the new year. Those closest to her are amazed at her strength, much of which comes from her faith, but also her pragmatism. And it hasn’t been tear-free.
“I do my crying at night,” she says. “But I grew up on a farm and saw death often. I held the chickens while my father chopped their heads off. It was a part of farm life, just as death is part of life. The boys have come to that realization. “We do everything we can in our society to escape it, but death is death.”
Unfortunately, cystic fibrosis continues to stalk the Turcottes into the next generation. Suzie Turcotte Smith, while dodging the CF bullet herself, is a carrier, as is her husband Terry Smith. The couple rolled the dice and took their chances. Suzie is expecting in July; an amniotic fluid test has determined her daughter will be born with CF.
Suzie, now a nurse in the Claresholm Willow Creek Continuing Care Centre, says the decision to have a child was a joint decision. And, as an expectant grandmother, Veronica notes the advancements made in the treatment of CF in the last two decades gives the family hope for what lies ahead.
For the next long while, though, life is life and Eugene has his stretching out ahead. With Maurice holding his own, the Turcottes can finally escort death to the door.
For two decades, cystic fibrosis placed a time limit on the Turcotte family. A lung transplant has allowed them to contemplate a future.