Thursday, March 1, 2012

End-Stage Cystic Fibrosis

I debated about whether I wanted to write this blog for a little while this morning.  I decided, ultimately, I want to share my thoughts about this topic.

A few weeks ago, I had heard from some of my English friends who also have CF, that a documentary was airing about a 21 year old CFer, Kirstie Mills.  Kirstie was in the end-stages of our disease and was placed on the transplant list.  During her time on the transplant list she was also planning her wedding, sure that no matter what the outcome, she wanted to spend the rest of her life with the man she loves.

This is going to sound terrible, but when I first heard about it, I was somewhat relieved it wasn't playing in the U.S. because I didn't think I could handle watching it.  I have known one person very closely, who has gone through transplant, but I never saw him prior to that. I'm going to be 100% honest right now and say that it terrifies me to think about the end-stage of this disease. I don't like thinking about not being able to walk without oxygen, or not being able to do the normal daily things that I currently take for granted.  I am petrified about the changes that come with end-stage disease.  So, I was glad I didn't have the option to watch it.

Then, I found out it was being aired in the United States under the title, Breathless Bride: Dying to Live.  Suddenly, I had to face the decision of whether or not to watch it.  I, sadly enough, chose not to watch it when it aired last night on TLC.  I just didn't think I could emotionally or mentally handle it last night. I didn't even DVR it.

This morning, I woke up and realized that I needed to watch it.  My sister-in-law had seen it last night, and I was honestly kicking myself for not watching it as it aired on the television.  After preparing myself for the tears that I knew would come, I found the link for the youtube version of the show, and I watched it in its entirety.  Yes, I cried. Yes, a lot.  But, there was more to it than that. I was truly, honestly, inspired by Kirstie's story. She maintained a sense of hope and optimism through the entire transplant process.  She showed me that while the end-stage of this disease are just as terrifying as I imagined it to be, it's not hopeless.  There are days that are better than others, and there is still love and hope and happiness during that time.  It's just always overshadowed by the hardship of breathing.

Kirstie's story had a very happy ending. She has received her new lungs and is doing wonderfully! I want to remind my readers one more time that CF effects every single person differently.  Kirstie needed a transplant at 21, but there is also an 84-year-old man living with CF.  CF is not a disease that can be easily defined.  That said, the following video provides a wonderful look at what the end-stage of CF looks like, and what it is like to be on the transplant list.  I hope you take the time to watch it, think about becoming an organ donor, and also think about making any sort of donation (time or financial) to the Cystic Fibrosis Foundation.


  1. I think you summed it up perfectly for me in your last paragraph. End-stage CF may look similar for most of us, but some will hit it early, most of us will hit it later on in life and a few of us will never reach it. Thank you for your thoughts on this topic.

  2. Thanks for sharing your thoughts! Interestingly enough, I don't think I'm quite as freaked out by pre-transplant end stage CF, though I'd certainly like to hold that off for a long time. I've had a lot of friends get transplants, some of whom were extremely sick (ie in bed on the vent, unable to speak/eat/move/etc.), and after their transplants they truly received the miracle of a second life. So at least over the last few years, my views of transplant have been very positive and hopeful. I'm definitely going to watch the documentary!

  3. Ronnie, thank you for reading and commenting. I definitely wanted to convey how the disease progresses differently for everyone. I don't want someone to watch it and falsely believe that all CFers wind up on the transplant list in their twenties.

    Cindy, I'm glad you're going to watch it, and I'm glad you don't have as many fears bout transplant as I do. I hope as I get older, my worries will ease about it.

  4. I just got done watching this. It was truly inspiring.

  5. I'm so glad you watched it Anna. I thought the same thing. :)

  6. As a 20 year old girl with CF I've always been accepting of what my disease will do to me. Unfortunately for the bugs in my lungs, I'm a pretty stubborn person, and I refuse to let my lungs shut down any earlier than they need to. I do two sets of physiotherapy every day to clear my lungs, exercise for an hour a day, take all of my tablets and supplements and antibiotics on time every morning and evening and eat constantly throughout the day to maintain my weight. Everytime I begin to get a sore throat or stuffy nose I drown myself in echinacea, garlic, vitamin C and zinc tablets to boost my immune system and fight off any sniffles before they reach my lungs.

    For me, this has resulted in maintaining good lung function and reducing the amount of chest infections I get, but I know that for some of my CF friends they will be constantly sick no matter how much work they put in.

    I have many friends who died between the ages of 18 and 25 from CF, but I also know many who received transplants within the last few weeks of their life and who are now living their life to the max... married, having children, travelling, breathing easily once more.

    I hope that all of the hard work I put in to keep myself healthy will pay off in a longer life with my own natural lungs before I need a transplant as I know that transplants are by no means a cure, and it's usually a case of swapping your CF problems over for a whole set of new ones: rejection, nonexistent immune system, constant bronchs and tests etc. but I'm so happy to see the life it has brought back to the eyes of my friends who would otherwise not be here.

    All I want is for everyone, old and young, of every single race, colour, religion, age and size to sign up for organ donation. People just don't get around to signing up with a "I'll do it later" attitude and before you know it, you're dead and your organs go to waste in many cases. You don't need them when you're dead and gone, but think of all the people who can continue their life with them and put them to good use!

  7. My best friend is coming to the end stages of cf n it scares all of us, you can see the fear in his eyes whilst he tells us hes ok. We dont know what to say or do n if im honest I cry quite often for him. The positivity in kirstie is amazing and really encourages you to have strength. I couldnt imagine what cf is like we try to joke about it and make light of it but as its getting closer and closer its getting harder to do that. I love my friend with all my heart and as much as kirstie came so close to the edge I pray he is as lucky. I have big respect to anyone with or close to someone with cf because it my experience it only seems to happen to the best kind of people. Thank you for showing this x kazz x


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