Living with cystic fibrosis

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When Merisa LePage wakes up in the morning, she smiles seeing her daughter Sasha on the monitor next to her, sleeping soundly. She enjoys the moment before she thinks about her daily duties: give Sasha 10 different medications, perform respiratory exercises, check daughter’s bowel movements and keep an eye for any other signs of illness.

Every morning LePage, along with her husband Joseph, wake up with this long to-do list because their 21-month-old daughter has cystic fibrosis.

“It’s absolutely the hardest thing that I’ve ever had to deal with in life,” says LePage. “To even think that I could outlive my daughter just seems something impossible that I cannot even fathom happening. So we do whatever we can.”

Cystic fibrosis is a genetic disorder which effects many parts of the body, but primarily the lungs and digestive system.

According to Cystic Fibrosis Canada, it can often lead to “destruction of lungs and loss of lung function” and is known as the “most common fatal genetic disease affecting Canadian children and young adults.”

LePage is all too familiar with the effects of the disease. She was the coordinator for the University of Windsor’s Shinerama campaign which raises funds for cystic fibrosis research. Although she had no connection with the disease, she volunteered with Shinerama and eventually applied to be an assistant coordinator. When the coordinator quit however, LePage was asked to fill the person’s shoes and in the end raised more than $20,000, one of the highest earning Shinerama campaigns for the university. She never dreamed the knowledge she learned as Shine coordinator would someday be important in her own home.

The first-time mother says when she learned of Sasha’s diagnosis she was in shock and devastated because she knew the health struggles her daughter would face.

LePage says her experience with the CF charity has helped her be better prepared to look after her daughter. She has also found dreaming of a future for her daughter has helped her stay strong when Sasha has health problems. However, she says her most recent trip to the hospital with Sasha made her question her strength.

“After finally getting her calm during some nights, I’d just sit in the rocking chair and watch her breathe and sleep. It was during those moments that I’d look around and realize that this is likely a place we would be frequently with Sasha,” says LePage.

Sasha had been admitted in September for 11 days due to her digestive system backing up, resulting in the now two-year-old’s bowel becoming blocked. Without being able to pass stool, Sasha was frequently in pain.

“Watching her with tubes in her nose and yelling in pain at times, it was a real low point knowing that I couldn’t keep just holding out that Sasha might have very few issues with CF . . . In those moments, you can’t help but think about all the horribleness that this disease can do and about how many lose their lives because of it.”

During that time, LePage said it was actually one of her nurses who helped her regain her confidence by reminding her she was Sasha’s advocate. It was this support and her husband constantly staying positive which allowed her to continue fighting for her daughter.

“It’s absolutely needed in order to get through the days knowing that your child has this disease and you know being positive that her future is going to be long, healthy and happy,” said LePage.

Having a positive outlook is what 19-year-old Mitch Hannigan says is important when living with cystic fibrosis. Hannigan also has CF and deals with problems each day, but says it always varies. While he has lung issues, the primary problem is his digestion. He says mucous builds up in his intestines and bowel and so as food is digested it moves through his body slower and can stop moving, resulting in severe pain at any time.

People living with CF and their families have also been able to interact with one another in Windsor and Essex County either in person or through Facebook and email. This communication often begins after meeting family members of those with CF through the Great Strides Walk, the Princess Ball or other fundraisers. Hannigan said he and his mother have been in touch with many families including the LePages. He said after dealing with the disease himself, he thought he could give other families advice based on his own experience on how they can live with the disease and fight it.

“We just tell her (Merisa) just stay positive and make sure to tell her make sure to ask the right questions to the doctors or nurses,” said Hannigan.

“Things are going to happen that are stressful or struggling but it’s the fact that you gotta help, you gotta do it and you gotta stay positive about it and we tell Merisa that always keep your head up high and just remember she (Sasha) does have CF, but she can fight it and CF won’t have her if she doesn’t think that.”

For Hannigan, being positive means believing he can live a long life

“I want to become the first CF person to make it to 75.”

In his opinion, he’s already lived longer than his doctors gave him when he was born, which was 18-years-old, so he can only get older from there.

But staying positive has not always been easy for the second-year St. Clair College student. He says in dealing with the disease, he reached his lowest periods of life in 2005 and then 2010. The first occasion saw Hannigan in hospital with a collapsed lung and appendix and this, along with the pain, brought on negativity. He hit an emotional low, wondering if he would reach his goal of 90 years old or past the hospitalization. Even though he did live through the experience, these same thoughts crossed his mind again in 2010 when he was admitted several times with a lung infection. He says with all the “annoying coughing” and double the treatments, it was a challenge to stay positive because it’s “something that nobody should feel, ever.”

But he says in addition to the support he received from his parents, it was his doctor Lenna Morgan and paediatric nurses who had him redirect his thoughts to positive ones. He adds his entire support group was “never hesitant to tell (him) the harsh truth.”

Even when his treatments or procedures prevent him from participating in team sports or he is unable to get together with friends because of how he is feeling, he says their understanding and positivity has not only been helpful, it’s something he has incorporated into his own outlook.

“I know I’m going to get through it, it’s just with a little struggle but whatever, I’ll get through it and basically once again keeping the positive attitude about growing with CF, knowing that the people around me are awesome,” says Hannigan.

Kristin Douglas of Cystic Fibrosis Canada said the support a person with CF receives can be crucial in helping a person live with the disease.

The Windsor-London-Sarnia chapter fund development manager for CF Canada said along with the physical difficulties a person faces, mental health can also be an issue.

“You can imagine even just being 25 years old and dealing with a life threatening disease,” said Douglas.

“It might be hard to meet friends, it might be hard to have a relationship, it might be hard to live a regular daily life when you have to take 30 pills a day and do four hours of therapy so there’s definitely a lot of things that come into play.”

Hannigan takes about 50 medications per day which has steadily increased with age. While Sasha, almost two years old, has about 10 to take daily, LePage acknowledges this will increase as she ages.

Sasha’s daily routine consists of “cupping” exercises in which LePage uses her palm to loosen mucous in the lungs. This is because when a person with CF contracts something, such as bacteria, in their lungs, they are unable to cough it out in the same way someone without CF can. It will more likely stay in the lungs because it gets caught in the mucous and could cause infection. LePage says people with CF die at an earlier age because lung function can steadily decrease from infections until they cannot breathe regularly.

The median survival rate of Canadians with CF has increased to 50 years old, based on numbers released by Canada’s CF registry in 2014.

Douglas says the thought of living to only 50 can weigh on people’s minds. However, by creating an almost familial relationship with other people living with CF in Windsor-Essex, it can make it easier to live with the disease.

“Everyone involved with the chapter has a huge family support system and also friends and friends of friends who hear about it and they hear about the stories. I think just from even Mitchell and Sasha having their stories told, gets other people involved too and gets even more support from even people they may not even know that well. So I think having family around you is huge,” says Douglas.

While communication has been key, Douglas stressed people with CF are unable to interact with one another in person so they’re using e-mail and Facebook. She said they need to avoid sharing bacteria, which could quickly multiply in the body of another person with CF.

LePage said she became involved in the community because of the emotional support it has provided her and Joseph as they raise Sasha. She has helped in organizing the Princess Ball, the Great Strides Walk and several other fundraising efforts, as well as being on the board of directors for Cystic Fibrosis Canada in Windsor-Essex. It has allowed her to have a more hands-on involvement with the disease.

“It’s great to see your friends, your family and people you don’t even know gather together donate what they can, come to the event, walk, show their support and that we’re raising awareness (with) more people than ever are learning about the disease at the same time,” said LePage.

For LePage, her hope is through these events and through raising awareness, the public can learn more about the disease and will in turn result in more people donating to Cystic Fibrosis Canada. This would allow more research to be done and a cure could be found sooner than later.

Until then, LePage says she is always hoping for the best for her daughter.

“I want her to go to school, I want her to hopefully get married one day, I want her to experience the joy of having children herself if that’s something that she wants down the road. Just for her to be happy more than anything, that’s all any parent wants for their child,” says LePage.