Alex Nickel is a 9-year-old boy from Rosetown who was diagnosed with Cystic Fibrosis (CF) in 2009.
Cystic Fibrosis is a hereditary disease the causes severe damage to the lungs, digestive system and other organs of the body. According to Cystic Fibrosis Canada, CF is the most common fatal genetic disease affecting Canadian youth and young adults. There is currently no cure.
Alex's lung capacity over the past year has been deteriorating faster than the average person with CF. In May this year, despite receiving a high dosage of antibiotics during one of his regular hospital visits, Alex's lung function was at an all-time low, at 59 percent.
"Unfortunately Alex has not been responsive to any of the antibiotics that they have used," Alex's mother, Jenae Nickel said. "His baseline lung function from last September has dropped 8 percent, and the average person with Cystic Fibrosis would lose 1 to 2 percent a year."
The genetic disease occurs when a child inherits two defective copies of the gene responsible for CF, one from each parent. Approximately one in 25 Canadians carry one defective copy of the CF gene, though carriers do not have CF, nor do they exhibit any of the related symptoms. When two CF gene carriers have a child, there is a 25 percent chance that the child will be born with Cystic Fibrosis.
"The average in Canada is about one in every 3,200 children born will have Cystic Fibrosis," Janae said. "CF is a very personalized disease. Somebody with Cystic Fibrosis might not look anything like the next person with Cystic Fibrosis. There are adults in their young 20's who are waiting for a (lung) transplant and there are people in the 50's who have never been on a transplant list. So it varies person-to-person and unfortunately, Alex seems to be one that his disease is going in a way that we hoped that it wouldn't."
For Alex, the disease means he can't enjoy all of the activities he normally would as a kid.
"Having such a complex medical life does take a toll on him," Jenae said. "He can't do things like a normal child would. It's harder for him to keep up with his peers, we have to be very careful that he doesn't catch infections, but the hardest thing for Alex is hearing what the future is going to be like. He's getting to the age where he's beginning to understand what Cystic Fibrosis as an adult is going to be like and he understands that his disease is not going the way that his doctors would like it to. So it takes a toll on him that we can see as a family, but everyone else might not see at the swimming pool or at the soccer pitches."
Somehow through all of this, Alex has managed to keep a positive outlook.
"Alex is extremely resilient and very hopeful," Janae said. "he's always looking at the positive side of things."
There is a fairly new medication available that helps slow down the effects of Cystic Fibrosis, though the only problem is the cost. Orkambi is a medication that works by correcting a mutated gene in Cystic Fibrosis patients. It essentially presses pause on the disease and allows for relief of deterioration in the lungs and other organs. It was approved for use by Health Canada two years ago, though the government of Saskatchewan does not cover the cost of the drug. The estimated cost to receive the drug is $250,000 a year.
After his last visit in the hospital, Alex's doctor wrote a letter to the government of Saskatchewan asking them to consider funding the medication for Alex. Last Thursday, Janae got the letter back from the government of Saskatchewan saying the funding was yet again denied.
"So regardless of how sick he's getting," Janae said. "the government isn't wanting to make an exception for him or for any other people with CF. The clock is ticking for them, each month Alex waits without this medication, his lung function is getting worse and that may not be reversible."
Janae said there is still hope the province will cover the medication. She said there will be a review by the Canadian Agency for Drugs and Technologies in Health (CADTH) and the provinces to negotiate a price for the medication.
"Cystic Fibrosis Canada has released information that they're hoping a recommendation would be made by early August," Janae said. "It's not a cure, but it's the closest thing we have to dealing with the medication at the cause, instead of just trying to treat the symptoms of it."
She noted that the medication would be very expensive for the province, but so is the treatment Alex currently receives every year just to try and maintain his current condition.
"What we really need," Janae said. "Is for the provinces to put the health of our children first and negotiate a price and get this medication into the hand of Canadians that need it."