My family has been participating in the CF hike for years, so we have acquired quite a few Xtreme hike monogrammed items. I frequently wear the down coat, my dad has a small cooler, we got a pretty cool hat. Sometimes friends see the gear and ask about the hike and ask if we know someone with CF. That's when I tell them actually my husband has CF. They normally respond by saying "No way! David seems like such a normal healthy adult. I would have no clue." And that's when I normally go on my little spiel about CF and how it's no longer a childhood disease and the advancements in treatment has increased the lifespan of those affected by CF to now make it an adulthood disease!

When David was born his expected lifespan was 18 years old. David was able to receive Trikafta pretty early on back in 2020 and since then his lung function has plateaued, which means more tomorrows.

So what does a typical person with CF look like? It's different for each person with CF. Most have to take enzymes with every meal or snack (typically 5-6 capsules), there's some that need to take insulin, there's some that need frequent nebulizer treatments and chest percussion therapy.

You all have probably heard about all the pills and hospital stays a person with CF might have to encounter... Let's talk about what it is like being married to someone with CF. First of all, they are stubborn. Really really stubborn. And won't let anything slow them down. I'm a travel nurse, and David is able to work remotely. Not once in our 2.5 years of being on the road has David let his health slow us down. In fact, he has always been the one supporting and pushing for us to go explore the country. We move every 3-4 months. He is required to go to his CF specialized doctor every 3 months. Luckily his lung function is stable which makes his doctor comfortable enough to agree to in person visits 2x a year (THANK YOU TRIKAFTA), the rest can be tele health visits. These visits still require him to use a specialized PFT, pulmonary function test, that are able to report his lung function directly to his doctor. Prior to this past year, David had been having to fly cross country, stay the night in Chicago, take the train or uber out to the suburbs, wait around at the doctor and do a bunch of tests which typically takes 4-6 hours, leading to the doctor visit taking the entire day with commute time. The agreement to tele health visits has been life changing. Prior to Trikafta, David was doing both nebulizer treatments and chest percussion therapy multiple times a day. He was hospitalized a couple times for different infections throughout his childhood. He was unable to attend the same school as another child with CF- forcing him to switch elementary schools.

David is still affected by CF on a daily basis. A common cold can mean just a sore throat for me. For David, a common cold can last weeks often requiring a visit to his CF doctor for a medication cocktail as he calls it. Every time David eats, he needs to take 5 enzyme pills in order to properly digest the food. On average David takes about 30 pills a day.

This summer, I convinced David to move to Alaska. Planning for Alaska was a bit more stressful than other contracts. We were originally going to be in Sitka- only accessible by 10 hr ferry from Haines or couple hour plane ride from Anchorage. Delivery of packages takes months to arrive. If David were to run out of medications, it could mean he would have to fly home to St. Louis to collect them from our storage unit (my parents' house). But if he were to get sick.... He would have to go to a 36 bed hospital and wait to be lifeflighted (helicopter) to a bigger hospital in Anchorage and then potentially be lifeflighted out of there to the lower 48. Scary.

Luckily we were able to find a contract in Anchorage very last minute (literally 1 week before departure for Alaska!). This was better! Packages can arrive within two weeks and we are close enough to a big medical facility.

We started packing for Alaska and David packed away his medications like a squirrel getting ready for winter. He travels with (2) 16x24 tubs of medication along with getting deliveries to our place we are staying. For an average healthy person, they would be able to fly out to wherever and be able to not think about it months leading up to the move. For David, we have to make sure he has enough medication stored up, close enough on grid for more medication to be shipped, get his doctor's approval and also be within a close distance to a large reputable hospital. Each location takes months to years of planning. For Alaska, this took 3 years of planning.

These advancements in science have been life changing for David, but there's still more research and advancements to be made. There's still mutations of CF that have not found a life changing modulator that works for gene variation. These modulators are one step closer to finding a cure. This is where you all come in...most progress made to finding a cure and finding treatments such as Trikafta has come through donations. Your donation goes straight to the foundation to fund research and find a cure. With your help more people with CF can live "normal" lives and more people can say "oh my gosh I had no clue .... has cystic fibrosis!"