GREENE — For years, hardly anyone knew Pam Sirois had cystic fibrosis. At first it was easy; she didn’t look sick.

Her treatments — 30 pills a day, four inhaled medications, a compression vest to clear her lungs — took hours but happened behind the scenes. She kept up with her nursing job, her husband and her three kids. Only family and close friends knew her lungs were functioning at 65 percent of what theirs were. Then 50 percent. Then 30 percent.

“I didn’t really want it to define me,” she said. “I didn’t want people to feel bad for me. I don’t feel bad for myself. I didn’t want people to associate me with a disease.”

Sirois has been dealing with CF — a genetic, life-threatening condition — her whole life. When she was a baby, doctors said she probably wouldn’t live past age 10. Last week she turned 43. But while she’s religiously adhered to drug regimens and breathing therapies, there was always one treatment Sirois said she’d never do: a lung transplant. 

“I just felt like I had been very successful,” she said. “I never expected to have a career, I never expected to have a husband or three children. I just felt like it was a huge gamble. You know, what’s worse, dying of CF or dying from complications of a lung transplant?”

Her children changed her mind.

“My son’s 11. I’d like to see him graduate high school. I would really like to see him graduate from college. He would like me see him get married. He said to me one night, ‘You know at weddings when the mom and the son dance together? Are you going to be there for that?’ And that just kills me that he’s thinking about that,'” she said. “I said, ‘Ben, I’m going to try my hardest.'”

Today, Sirois’ lung function is down to about 20 percent and she relies on oxygen to get through the day. She had to stop working in January. She’s waiting for a double lung transplant. 

The nurse who never wanted anyone to know she was sick is now starting to talk about her illness. And encouraging others to talk about organ donation.

“CF is an invisible disease,” she said. “When you see me even now, and I’m on a transplant list, when I do my hair and put on makeup, I look pretty good. But not inside.”

Rapid decline

Cystic fibrosis causes the body to produce mucus that is thick and sticky rather than thin and slippery. That mucus affects the digestive system and clogs the lungs.

In the United States, about 30,000 people have the condition, according to the Cystic Fibrosis Foundation. 

Like many people with CF, Sirois was diagnosed as a baby. She was colicky and fussy, wouldn’t eat, couldn’t gain weight. Her parents, who had two children before her, knew something wasn’t right.

“There was a lot of back and forth to the doctor,” Sirois said. “At the time, luckily, our pediatrician, one of the only pediatricians in town, happened to be the CF doctor as well.”

A sweat test confirmed it. She had CF.  

It was 1972, and children with CF weren’t expected to live much past age 10. But while Sirois had treatments and medications to deal with, her childhood in Greene was pretty normal.

“I was encouraged to go to school and have friends and play sports,” she said. “I grew up on a dairy farm, so I was quite active.”

Sirois blew past the first life expectancy doctors set for her, then the second. She didn’t talk about it much. Or make it widely known.

“Each time I outlived my life expectancy it was kind of a huge deal. But you also don’t want your friends or your friends’ families to feel like you’re a ticking time bomb,” she said.

One person who did know about her CF: her high school sweetheart, Wes Sirois. Even if he didn’t realize what it meant at the time, or what it would mean for them both.

“I was a 16-year-old kid, so I didn’t really know what it was at that point,” he said. “It was kind of a part of her life, but I never treated her like someone that had a chronic illness.”

Sirois graduated from high school, enrolled in college. She planned to become a doctor — “I knew what it was to be a patient,” she said — but the cost and time commitment were daunting. She became a nurse instead, then earned her master’s degree and became a nurse practitioner. 

She and Wes Sirois married in 1995. They had a son, then twin daughters, all three free of CF.

Sirois worked as an oncology nurse for Central Maine Medical Center in Lewiston, then for St. Mary’s Regional Medical Center in Lewiston. She later switched to an endocrinology practice, where she cared for St. Mary’s patients with diabetes.

Although Sirois’ CF treatments could take hours a day and her illness sometimes landed her in the hospital for a week at a time and she had three young children to care for through it all, she still rarely talked about it. 

“She’s one of those people that has never felt sorry for herself,” Shannon Deschenes, a family friend and local doctor, said. “Most of the people involved in her life, that she would come in contact with, probably never knew that she had CF.”

And generally they didn’t need to. Despite the treatments and hospital stays, the trouble breathing and digestive problems, Sirois generally felt pretty good. 

Then, over the past couple years, her struggles became dramatically harder.

“People with CF can be doing pretty well, then it just drops. That’s kind of what happened. Her lung function went from 65 percent down to 50, then below 50, then she couldn’t get it back to 50, then the 30s, then she couldn’t get it above 40,” Wes Sirois said. “It was a faster decline than either one of us was expecting.”

Her hospital stays grew more frequent, going from once every two years, to once every few months, to once every six weeks.

“Usually, I just lay there for a week and then try to get back on my feet. But that’s not happening anymore. I don’t feel any better,” she said. “It’s kind of frustrating.”

With her lung function around 20 percent, Sirois went on oxygen. She had trouble maintaining her weight as her body burned through calories working to breathe.

Doctors said she likely had more than a year to live, but not more than five. 

“But it’s also really hard for them to see me this way because I can’t do anything for them anymore. And that’s really hard. And I don’t want them to have to take care of me or to have a sick mother or all those labels that when they grow up and get older they’ll have to get therapy for.”

With that in mind, Sirois considered the one treatment that she had always shunned: a double lung transplant.

“We never really talked about it until a year, a little over a year ago,” Wes Sirois said. “I just said ‘You know, I don’t think you would live with yourself to tell your kids that you didn’t do everything you could to stay around for them.’ When she started to think about it, I think that’s what hit her.”

Particularly when it came to her 11-year-old son, Ben.

“He really wants me to get a transplant,” she said. “I figure at this point if I didn’t, he would have a lot of resentment that I didn’t take that chance to be here.”

Recently, Ben, a fifth-grader at Greene Central School, wrote a pair of school essays about his mom. Both focused on her battle with CF.

“My mom inspires me every day because she is a fighter; she tries to be kind all the time,” he wrote in one essay titled “What Shapes You To Who You Are.” “I love my mom and she loves me.”

Raising money, raising attention

Sirois is now on the transplant list at Brigham and Women’s Hospital in Boston. She is going through the process to be added to the transplant list at the Cleveland Clinic in Ohio.

Each hospital has its pros and cons. Brigham and Women’s is closer, but the Cleveland Clinic does more lung transplants each year.

If she’s accepted by the Cleveland Clinic and matched with a donor, she’ll have to take a private jet to get to the hospital in time. It will cost $4,000, and insurance doesn’t pay.

Insurance won’t pay for travel to Boston, either. Nor will it pay for hotel stays, time missed from work and some after care.

“It’s been hard to accept help. Especially financial help. My husband and I have been very, very fortunate in our lives monetarily,” Sirois said. “But with me not working, obviously, it’s been difficult. And the cost of transplant, and after transplant, and having to live away from home for 8 to 12 weeks. . . all that goes into it. It’s a lot of money.”

Family and friends created a GoFundMe campaign on Sirois’ behalf in November and recently set up the website HelpPamBreathe.com. So far, they’ve raised a little over $18,000.

They will also host a combination birthday celebration/fundraiser on Saturday, April 18, at the Boofy Quimby Memorial Center in Turner. The event will include dinner, music, a silent auction and a raffle.

Tickets will cost $10 for dinner and $10 for an evening concert. They will be sold at the door and online at HelpPamBreathe.com. 

Event organizers have two goals: raise money for Sirois and raise awareness about CF and organ donation.

“We’re really putting out as many ways as we can about this event but also how you can continue to help by becoming an organ donor, learning more about that, or going to the GoFundMe site and learning more about CF,” said Deschenes, who is helping with the fundraiser.

It’s attention that makes Sirois and her husband a bit uncomfortable.

“We never really ask anyone to help. We just kind of do things. That’s just the way we are. That’s not been easy,” Wes Sirois said.

But at the same time, the couple is grateful. They’re happy to be raising awareness about CF and organ donation, which is low in northern New England. They’re happy to have friends and family so willing to help, whether that means picking up one of their kids from school or setting up a fundraiser.

“Having that safety net, we’re thankful for that,” Wes Sirois said.

For now, Sirois waits for an organ match from Boston and waits to hear whether the Cleveland Clinic will accept her for its transplant list. It could take months to be matched. And even then, it’s not a sure thing. Sirois will still have to contend with the possibility of rejection and complications.

But it’s a risk she’s come to accept. For her kids.

“I’m teaching them that life is precious and life is short and no matter what you have or what you have to deal with, you have to persevere. You have to fight. And you have to keep living,” she said. “I hope that’s what I’m doing for them.” 

ltice@sunjournal.com

“I didn’t really want it to define me. I didn’t want people to feel bad for me. I don’t feel bad for myself. I didn’t want people to associate me with a disease.” — Pam Sirois

“My son’s 11. I’d like to see him graduate high school. I would really like to see him graduate from college. He would like me to see him get married. He said to me one night, ‘You know at weddings when the mom and the son dance together? Are you going to be there for that?’ And that just kills me that he’s thinking about that. I said, ‘Ben, I’m going to try my hardest.'”

— Pam Sirois

“People with CF can be doing pretty well, then it just drops … It was a faster decline than either one of us was expecting.”  — Wes Sirois

“My mom inspires me every day because she is a fighter; she tries to be kind all the time.”

— Excerpt from “What Shapes You To Who You Are,” an essay by Ben Sirois, age 11.