Cystic Fibrosis: A Day in the Life of Dallas
If you're anything like me, you've heard of this allusive disease called Cystic Fibrosis (CF) but you don't really know much about it. I was afforded the opportunity to learn more by spending the afternoon with Dallas and his family a few weeks ago. Dallas is a very sweet, spunky, ACTIVE, almost two year old, who was diagnosed with Cystic Fibrosis (CF) at the ripe age of five weeks old. Unlike some of the more common childhood diseases, CF isn't the type disease that you can "see on the outside." It attacks internal organs such as your lungs and pancreas. You could walk by Dallas on the street and have no idea he is suffering from Cystic Fibrosis.
"Cystic Fibrosis is a progressive, genetic disease that causes persistent lung infections and limits the ability to breath over time. In people with CF, a defective gene causes a thick, sticky buildup of mucus in the lungs, pancreas, and other organs. In the lungs, the mucus clogs the airways and traps bacteria leading to infections, extensive lung damage, and eventually, respiratory failure. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to break down food and absorb vital nutrients. " ~ Cystic Fibrosis Foundation
The reason I'm writing this blog is awareness. In visiting with Dallas, I learned that there is zero government funding for Cystic Fibrosis. That means for those 30,000 people in the U.S. living with CF, the government is providing absolutely nothing in the way of support for research. There are 1,000 cases of CF diagnosed each year with 75% of those cases being children less than two years old. The research for this progressive disease is funded completely out of the pockets of people just like you and I.
CF is Expensive!
I looked into the top three treatment options for Cystic Fibrosis along with the costs associated, and was absolutely shocked. *Notice I used the term "treatment" and not "cure."
To prevent the sticky build up in Dallas' lungs from blocking his airways, he wears this fancy inflatable vest that vibrates his chest at a high frequency to help loosen and thin mucus. Dallas sits like a champ twice a day for 30 minutes while this vest goes to work on him...an incredible feat for a toddler! This vest and the device that power it, cost between $15,000 and $20,000 and depending on your insurance, may not be covered.
The second most popular treatment for CF patients include inhaled medicines. These are liquid medicines made into a mist or aerosol then inhaled through a nebulizer. A nebulizer costs anywhere from $50 to $2,000 depending on the features you need. Don't forget you have to buy the medicines that go in the nebulizer! These medicines include antibiotics to fight lung infections along with therapies to help keep the airways clear.
The third most popular treatment for CF includes Pancreatic Enzyme Replacement Therapy (PERT). Most CF patients have blocked pancreatic ducts so they can't absorb fat and proteins properly. PERT is used to replace natural enzymes so that fat and proteins can be absorbed properly, which improves nutrition. The average cost of PERT ranges from $300 to $5,000 per month. For Dallas, this means planning every meal and every snack to a timely tee. Dallas must take his digestive enzyme supplements before anything goes in that tiny mouth, or his body will just flush it right out.
As I mentioned before, these are only treatments, not a cure. In 2015, the United States Food and Drug Administration approved the second drug to treat the root cause of Cystic Fibrosis. The arrival of this group of drugs called CFTR modulators, is a historic breakthrough in how CF is treated. It is expected that CFTR modulators could add decades of life for some people with CF. What's the price tag on this group of drugs you ask? With a discount drug coupon I found for CVS Pharmacy, you're looking at $21,849.70 per month. Ouch!
It goes without saying that if you have a progressive disease, you probably have some restrictions on what you should and shouldn't do. Dallas is so susceptible to germs and infection that he has some pretty hefty restrictions. Flu season could be detrimental to Dallas so from October to April, Dallas' family do a lot of staying home. Even a simple cold could land Dallas in the hospital for weeks.
One very interesting restriction Dallas has is shoes. Who would have thought? Certain shoes are known for holding moisture, therefore creating a breeding ground for bacteria. For the average Joe, that bacteria is harmless, but for someone with CF, that bacteria could be deadly.
Stagnant water is another breeding ground for bacteria making it a no-no. Dallas has an incredible creek that flows through his family's 11 acres of land and Dallas will never get to dip his toe in the wading pool area of it because the bacteria that lives in stagnant and slow moving water.
The most shocking restriction to me, is the six foot rule. Those living with CF cannot be any closer than six feet to another person with CF. Cross-infection is very high among CF sufferers so a simple sneeze from one person with CF around another person with CF could be fatal. We recently participated in the Great Strides fundraising walk put on by the Cystic Fibrosis Foundation in Winston Salem and I think the quote below from Dallas' mom says it all.
"Today we walk in honor of these two CF warriors so that one day they can be closer than 6 feet apart! We have hope that one day a cure will be found and oh what a day that will be." ~Megan Hulen