Living with cystic fibrosis in the family
On the weekend of January 4, Heather Strange was out doing what she has done so many times in the past, and will continue to do so many times in the future: fund-raise for cystic fibrosis.
Sometimes, the long-term goals can seem overwhelming.
Regarding a future, it’s not a luxury Heather and her husband Dave allow themselves. Their son Carter is afflicted with cystic fibrosis.
“I don’t think about it. Dave and I, we had to shut that down. We had to shut down the idea of high school graduation, we had to shut down the idea of university, marriage, kids, because the future is so…” she leaves that hanging. “It’s kind of overwhelming to think of that.” They know too many people with CF who have passed away in their mid-20’s.
Cystic fibrosis is a genetic disorder present at birth. In order for a child to be born with CF, both parents would have to have a mutation on the chromosome 7. Children with parents who both have this mutation, have a one in four chance of getting CF, a one in four chance of not being impacted in any way, and a one in two chance of being — as their parents were — a carrier.
Heather explains that CF is like having a defect in the pump that regulates salt in our bodies.
“Because this salt-exchange pump is completely out of whack, what happens then is the mucus in your body becomes extra thick.”
The disease, Heather elaborates, is a “head-to-toe” disease. Complications include sinus problems, lung infections, digestive system, pancreas function, breathing difficulties to name a few. The symptoms vary from person to person, making it very individual.
The diagnosis for her son was difficult to achieve. Heather knew in her gut that things weren’t right for Carter. Yet, it took almost three years — and a doctor with previous exposure to CF — to administer a simple sweat test. This test confirmed the suspicions that something was wrong, and turned their worlds upside-down.
There are still difficult parent-child conversations that they need to face, and as a couple with an overwhelming challenge, Heather and Dave move through each day step by step.
In the meanwhile, they are planning local trips — vacations — where they can, for a brief moment in time, have some semblance of normalcy. They are looking at locating a camping trailer that would afford them the mobility to come and go on their own schedule, without having to rely on flight schedules and permissions.
“We’re the abnormal ones at the moment,” said Heather, reflecting on the longevity of her relationship with her husband. “We are nine and a half years into this. We were struggling for a number of years.” As difficult as it is, thefir life has established a rhythm, one that a diagnosis really helped with. It gave them a concrete problem to manage. Sure, there are still challenges, but they are managing.
Heather’s coping method has been to compartmentalize. She became a gatherer of information, and a relayer of fact. She empowered herself through knowledge.
“I gave up my career, and my world. … Once we got diagnosed I dumped my brain of … any information that I learned prior to that and I filled it up with new information.” She strongly recommends that knowledge be obtained from reputable sites.
A recent break-through drug called Kalydeco, detailed in an earlier CBC article (“Cystic fibrosis ‘breakthrough’ pills have $300K price tag” printed at cbc.ca on December 6, 2019), has an astronomical price tag: That’s $300,000 per person per year. Further, this drug is only for those with a certain form of CF, known as G551D which according to the Canadian Cystic Fibrosis Registry (2011) affects 3.4 per cent of those with CF.
Carter, is afflicted with DeltaF508, which occurs in 91.5 per cent of CF mutations. The current explorations for this form is CF is a combination of Kalydeco and Lumacaftor. According to Heather Strange, doubling the drugs effectively doubles the price.
Managing the disease — outside of the costs of medicine — is expensive. First, there’s the lost income of one parent. Then, Heather and her husband Dave purchased a machine that administers multiple medicines to Carter through a face mask, which costed about $2,000. It costs an additional $1,000 a year to maintain this machine. And then there’s the medicine.
“One of the drugs that Carter takes on a regular basis is $1,200 a month. Occasionally, he has to go on another drug, and that’s $3,000 a month.”
A combination of medical insurance and Pharmacare helps them manage these costs, but the deductibles before coverage kicks in do add up.
In the meanwhile, Heather does what she can to raise funds for research for CF. She has cycled for CF, done Jazzercise fund-raisers, and committed to a number of other fund raising events around town. Just like the weekend past, where she raised about $800 in a bottle drive. Right now is what they have. And right now, it’s all about putting one foot in front of the other.