DerekChrstnsn

I also have CF, and will try to answer from my perspective as well.

One way to 'cope' is to just do treatments. I'm 24, and average 1-1.5 hours of treatment per day. Over my lifetime, that is 1.5 years worth of time that I've been attached to a machine whose purpose is to make me healthier. I know that those machines have lengthened my life by factors more than 1.5 years. So for me, whenever I do a 30 minute therapy treatment, I imagine me living another few days because of it.

Another big way is to just do as many things as you can. We are 'blessed' with knowing we may not live long, so we want to put as much stuff in our lives as possible. I chose to get a pilot's license before I even entered college. I even did skydiving in Hawaii (thanks MakeAWish). Knowing I might not live as long makes me want to 'fit in' as much life as I can.

Last I checked, it was 38 years. And an article recently came out stating how CF patient's life expectancies are getting higher at a faster rate than the average person. From looking at it previously, it seems like every 2-3 years, the average life expectancy goes up 1 year.

I personally know a guy with CF that is in his 70s, so it's not crazy to think that people with CF will live a long time.

I also have CF, so I'll answer this as well.

I'm personally excited about Ivacaftor (also known as Kalydeco). As well, Orkambi (which contains Ivacaftor) is another promising drug that came out. These drugs actually help fix the source of the problems of CF instead of the symptoms. I'll try to explain what they do, as I understand it.

There is a pump on the cell wall that typically transports sodium into the cell, in normal people. For CF patients, this pump can either be broken at the cell wall, inside the cell instead of at the wall, or broken and inside the cell instead of at the wall. These new drugs try to either fix the pump, bring it to the wall, or both. Right now, they kinda/sorta work, and they have been showing improvements for CF patient's lung functions. Eventually, the hope is to have a 1-pill cure that completely reverses the effects of CF by treating cells at the source and fixing them.

The Cystic Fibrosis Foundation sold their drug rolayties for some of these drugs for $3.3 billion. My doctor has told me that this sale has allowed for further investment into research for a cure, and has moved the timeline up for a potential cure by 10-20 years (because there are drugs they want to create/test, but don't have the money for). It's truly exciting times for anyone with CF of a CF friend/family member.

If she is only a carrier, there isn't too much concern. Maybe talk to her once she's 15 or 16, and just let her know that she is a carrier for CF. There isn't too much concern, as in the U.S., only 1 in 30 people (on average) are carriers of CF. So there is only a 3% chance that she will marry a carrier of CF, and even if she does, only a 25% chance that her child would have CF.

(CF patient here). Be as understanding as you can. Know that sometimes he may not be physically up for something (depending on his CF severity). As well, since treatments are a large time commitment, be understanding there as well. Try to support him in taking his pills and doing his treatments, as getting into a good habit of doing those will set him up for a healthier life.