[TOPIC] Cystic Fibrosis - the personal experience

I dont have CF but was considered to adopt a baby with CF. I do also have a friend with CF.

Certain things I can tell you - he used to get so angry at people who smoked. People with healthy lungs who self inflict damage. He was angry at his parents for having more children after he was born and diagnosed with CF - because of teh high risk they would have it too (as its genetic).

There are varying levels to which people are affected in their daily life. My friend had frequent hospitalisations through school. He took pancreatic enzymes and a modified diet. Daily chest therapy, IV antibiotic treatment in hospital off and on..
He didnt like special treatment from ppl or anyones pity. Mostly hates like hospital stays because theyre overcrowded and you get sick of them pretty damn fast.

The life expectancy is a lot better these days, and he attended university, and planned for his life as though he would live forever ! His health comes and goes but he's in his mid 20s and still kicking strong most of the time :0)

I also have a friend with CF. I've known him for five years. Obviously, I won't be able to answer from a personal perspective, but I can tell you what it's like as a friend...

What does it feel like?
I obviously don't know what it feels like. But I can tell you that it sounds like a person has a bad chest cold or bronchitis. The cough is persistent, and can be irritating, if you forget for a moment, why someone has this ongoing, congested, hacking cough.

How does it affect daily life?
Mostly, from what I've seen, having to make time for therapy, and meal time. Also, he lives knowing that he has a much shorter lifespan than others - death is very real to him.

What meds?
- Takes digestive enzyme pills (several at a time) with every meal.
- Salts food heavily.
- Does respiratory therapy with a therapy vest three times a day.
- Frequent doctors appointments to check lung function, weight, etc. (

Future?
- At 15, his family threw him this huge party. Sort of what you would throw someone turning 50 - as life-expectancy then was around 30. (Statistically, more than 40% of people living with CF in the US are 18 or older.) The life-expectancy recently rose to 36.5 years in '05, up from 32 years in '00.
- As I said earlier, death is very real to him. He's very aware that there is no cure for CF. However, he's now 21, and attending college, living with his cousin. He has a girlfriend that he's quite serious about.

Like/dislike people knowing? Why?
- I don't know if he likes or dislikes people knowing. He's been pretty open about it. Though I have heard him make comments about it being awkward if people don't know. That they'll hear him coughing, and offer him water or cough drops.

Like/dislike people helping? Why?
- Another one I'm not sure of. I'm sure he appreciates help when he needs it. And I know that he is always the one (and sometimes the only) to volunteer to help somebody else.

Hospitalizations?
- Absolutely hates them, and is terrified of needles. But he battles lung infections several times a year.

Feeding tube details?
He's quite small for his age, and does have a feeding tube to help him pick up extra calories. Oftentimes before the noon meal, I'd see him pouring one of those supplemental calorie drinks into his tube. He had to do this before lunch. Once, part of the tube broke, and he went and attempted to drink the "calories" straight from the can. It didn't go very well.

PricelessOne

--

2004: Belief (Won)
2005: Mercy (Won)
2006: Faith (Won)
2007: Community Watch (Won)
2008: Fall Rising

my neighbor growing up has CF and while different people react differently there were some things that were always consistant.
as a child it didn't seem weird to me that he had to be hooked up to his breathing therapy machine for an hour or so a day while we all sat and played cards, and i didn't think it odd that my mother had a bottle of his enzymes in the cupboard so that he wouldnt have to run home for his meds if he and his sister stayed for dinner.
as i've gotten older i've been able to see more the stress that it has played in the family. it caused divides in the family, and when he was a teen he lashed out alot at everyone around him since he couldn't at his disease. the average life expectancy is still fairly young, though getting better all the time due to new medicines and therapies, but i can't imagine what that would be like to be him....watching other kids die around you and wondering if you'll be next, or if you'll beat this just one more time. he has to go to the hospital several times a year to get his lungs cleared out and any upper respitory infection can be a big deal. generally, there are exceptions, but generally, CF kids never get too big, tend to be thin and weaker due to their health. my neighbor was unusually healthy, i didnt realize what the norm was until a bunch of other CF kids came over for a pool party. we have prayed, cried, and in our hearts died with him through this. but he keeps on trucking, though i can't say i know him much anymore, once he hit highschool everything changed and while i still know his family, we dont know him sadly.

if you don't get enough info, my friend's 13 year old son has CF. i could put you into contact with her.

Melissa
-----(the poet who would try to write a novel...again)
2007: The Malachite Necklace (51,020 words)
2008: Mariposa

My cousin has CF. We're more like sisters, since we grew up in the same house and everything like that.

Outlook: first of all, her outlook (and the outlook of all the other people I know with CF) tends to be very positive and uplifting. That's one of the things I've found about people with CF; they seem to be very cheerful and optimistic people. Perhaps this is not true of all, but it is true of all or most all of the people I've known. Maybe it's because they have had to come to terms with dying and they've thought a lot about death, and so consequently, they've thought about their life. I guess they want their life to matter, to be worthwhile, meaningful, happy. Unlike most people who take this for granted, I think CF people don't. They (the ones I know) love life and make every second worthwhile. Being depressed and moping all the time would basically be like... wasting the little time you have left.
This doesn't mean they're always happy, of course. My cousin has gone through some very dark times... all the 'why me?' stuff. But for the most part, she lives her life to the fullest and the tiniest of things make her very happy. She's very resilient. I think you have to be to deal with something like this.

Daily life: I'll try to think of how growing up was like and so on. Sometimes things like this seem natural to you because you live with it, but you don't realise that it's not like this for everyone.
Every day, I think once or twice a day at least, she would have therapy. By therapy, I mean pounding on her chest repeatedly, and then her back repeatedly, for a certain amount of time. At first my aunt used her cupped hands, and then they bought this machine that does it for you. A percusser I think. This was to dislodge the mucous in her lungs... because CF people don't have a way to get rid of all the mucous naturally. I used to ask to have this done too. When my cousin was getting it done, or sometimes doing it to herself, we'd watch tv and I'd do it too. I just did it with her, even though I didn't have to.
Also, while watching tv she'd do her mask. It's like one of those oxygen masks you see in the hospital or whatever, and it puts some kid of mist into her lungs. I'm sorry I don't know the exact details of this. I think it was a saline solution. It was a regular thing that whenever we watched tv and she did this we'd just have to turn the tv super loud so we could hear it.
At meals, and I guess in between meals too, she'd take a handful of enzymes. They looked like tiny little white balls in see through capsules. When she was really young she had to take like a million, but as the years went on she could take less and less. It either had to do with her getting healthier, the capsules and drugs being simply better, or both of those things. When she was little her mum would crush up her drugs in apple sauce so she would eat them.
She'd also take vitamins, and sometimes this powder that she'd put in drinks and food. It helped give something to her diet, like protein or fat or something. CF people also need to get more fat in their diets. I think it has to do with her not digesting things properly, or getting enough of the fat and other things.

Details: there are two kinds of CF, in a way. Digestive and Lungs... like all CF people have problem with both areas I think... but some have more problems with one of them. like my cousin has more digestive problems than lung problems, and actually I think is m,ore all around healthy than most with CF. She's 26, and has not had any major major problems. She did have to get her gallbladder out, though, and also used to have these attacks where she'd be doubled over in pain. She has also had pneumonia, which is very bad to get if you have CF.
Her friends with CF aren't quite as healthy as she is. One girl who is I think early twenties has already had a lung transplant. Another girl is always in the hospital with various complications. Another sad thing is when someone with CF gets Burkholderia cepacia. I think anyone can pick it up, either if it's the airborn kind or the kind you get from direct contact. However, if a CF person gets it it can be fatal. Also, the really sad thing is if a CF person gets it they can't really have contact with other CF'rs, because of the risk of infection. One girl I know just found out that she had been in contact with someone who has it now... her friend. This means that all of her CF friends, especially the ones from the same hospital she was at, can't go near each other for TWO YEARS. It's very sad, because there really is no one else who will truly understand what you are going through than another person with CF.

Um... another thing has to do with how other people might treat her (or anyone with CF). Most people look at my cousin and she looks healthy. People don't realise what she has to live with, just because she doesn't "look sick". I mean, even daily breathing for her has been compared to plugging your nose and breathing through a straw. So, just imagine what it would be like to have a cold and have CF.
Also, she hesitates even telling prospective employers that she has it. She has had plenty of good interviews in the past, where they have been thrilled with her skills, personality, etc... but then she says she has CF... and they go, oh... and she doesn't get the job. Of course they can't discriminate based on this; they always have a different reason.. like, a lame one. But she knows this is the reasons she doesn't get some jobs, even though it would rarely interfere with her performance. Also, people think she's a smoker because she has that cough. I mean, I can tell the difference between a CF cough and a smokers cough, but a lot of people can't. And yes, she gets very angry when people smoke, because they are purposely, knowingly, wrecking their lungs. She knows people aren't perfect... but... people in her family smoke sometimes, and I can understand that this would anger her a lot.

Um... don't know what else to say, though I could probably say tons more. If you have any questions at all, seriously just PM me on here. If I don't know something I can also just ask my cousin or someone else. I don't mind, and I don't think she would either. Any awareness about this terrible disease is good I think.

Edit: Also, once a year we'd travel to Sudbury to the hospital there for her annual checkup. I think everyone I know who has had CF has atleast this annual visit, or more than that.
Also, as she was growing up the average life expectancy was always just a little higher than her actual age. As she grew older, they kept changing it... which is good. When she was born, I think the expectancy might have been as young as 3 or so... and then it kept going higher. I don't know if it's 32 now... or higher. I have known a few people who were in their forties and had CF. Most of them are not alive anymore, though. :( But... atleast one still is. That's good.

Oh, and she would have a big bottle of enxymes at some of her friends houses as well as mine (when we moved to seperate houses) and our grandparents. She couldn't have a meal without them or miss them at all, so that was kinda important.
Also, she did have a lot of complications when she was a baby. I think the first year of her life (or more) was in the hospital... so many surgeries and so on. When she came out, she was so tiny yet a had been alive for a few years. People didn't realise how old she really was.

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Thanks so much everyone for all your answers. A couple of you have mentioned a therapy machine - could you describe it for me? Even better if you know where I could look for a picture/video of one.

Pats - classic CF therapy: http://www.youtube.com/watch?v=hDo3RvGyPgs

Vest treatment: http://www.youtube.com/watch?v=uBBdsMgc5ls

Going to YouTube and typing in "cf therapy" will yield all kinds of results like this.

Also...a resource for the personal aspect you're looking for: http://community.livejournal.com/cystic_fibrosis/

PricelessOne

--

2004: Belief (Won)
2005: Mercy (Won)
2006: Faith (Won)
2007: Community Watch (Won)
2008: Fall Rising

My husband has CF, so I have lots and lots of information about it. I will highlight some stuff here, but feel free to PM me with any specific questions. I am going to try to focus on stuff that others haven't mentioned.

When people think about CF they think about lung issues. But it affects so much more than that. As someone else mentioned, it frequently affects the digestive system. Many (if not most) CF patients are pancreatic insufficient and they take enzymes with every meal. To give you an idea, my husband takes 8 enzymes with each meal and they are each about the size of a multivitamin (but are capslets and have tiny little balls inside). Additionally, many people with CF become diabetic, because, again, the pancrease does not function properly. People refer to this as CFRD (CF related diabetes). The diabetes is often managed with insulin. With the digestive issues, there are also problems with absorption of nutrients. This is one of the main reasons that people with CF generally have difficulty gaining weight. Also, vitamin levels can be an issue. So, there can be bone issues.

CF can also affect the liver--and does so in about 20% of CF patients. With this, the liver ducts get clogged and it caused it to back up into the portal vein (portal hypertension). This can cause varacies in the esphogus, and bleeding can occur there. Also, the liver issues can cause an enlarged spleen, which can affect a lot of things, particular platlet counts. My husband has very low platlet counts, which is difficult to deal with because of all of the surgeries, etc. that can happen with CF.

A lot of people with CF also tend to have sinus issue. Polypus are common and surgery for that is common as well. CF also affects the reproductive system. 98% of men with CF do not have a vas deferens and can only conceive natural children through in vitro fertilization. The percentage of women with reproductive problems is not a high, but I don't know the numbers off the top of my head. Basically it becomes an issue with cervical mucas. And with women, their health is always an issue.

Physically one of the things that doctors always look is clubbing of the fingers and toes.

People with CF take A LOT of pills! In addition to the enzymes I mentioned and the insulin, my husband takes a bunch of vitamins, one for portal hypertension, one for blood pressure, one for inflammation, allergy pills, one for his liver. He also does inhaled antibiotics daily (supposed to do twice per day). He also uses hypertonic saline (inhaled) for airway clearance. Other CF patients use a medication called pulmozyme which thins the mucus in the lungs to help it make its way out. For inhaled antibiotics--TOBI is the one that many patients use. He also does a vest treatment.

He is hospitalized a few times a year with infections. He has a port-a-cath so that he can get IV antibiotics while at home now too, so that has reduced hospital stays.

Another problem that people with CF have is bleeding from their lungs. If you are interested in more about that, PM me.

My husband is 31 and works full time. But it is hard. He feels run down a lot and doing the treatments, etc, takes a lot of time.

As mentioned above, there are reproductive issues as well, so that has put some stress on us about having children. We are unsure about it not only because of this obstacle, but also because of the situation that we would bring a kid into.

My husband's family does not deal with the CF very well, so that has been an issue as well.

My husband does NOT like to tell people about his CF. He is really adamant about it, though he is starting to soften up about it a bit. He doesn't want to be treated differently. And also, he doesn't like to be reminded about it.

He coughs a lot and is self-conscious about the coughing. People will make comments about "oh you must have a horrible cold" or stuff like that all of the time. Movie theaters are a place that I really notice the cough.

Okay, I think that is enough for now, but seriously PM me if you want any specific information, etc. Good luck on your book!!

I don't have CF, but it does run in my family. I had a cousin who died from it before I was even born, she was 3 years old.

It's always left an impact on the family, even those of us who never knew Josie. It's really something that leaves an impact on everyone. Because it's genetic, everyone in the family who plans on having kids needs to get tested to see if we're carriers, but so far we've been pretty lucky. Her brother was a carrier, but his wife wasn't, so they didn't have any trouble. My sister wasn't a carrier, so she's fine. I don't plan on having kids, so I never got tested.

But I know that when my sister was starting to want kids, my aunt, Josie's mother, was very insistant on my sister getting tested right away, and that if both her and her husband were carriers, then she should get her tubes tied and adopt, because she didn't want her to go through the same thing she did.

2005: Shattered Mirror (21k/50k)
2006: Dream Diary (38k/50k)
2007: A Wish for Dawn (23k/50k)
2008: December Parasite (50k/50k, won!)

I am involved in the CF community and like I said before my hubby has CF. We think the same way as you do--if I am a carrier, there is no way in he** that we would have a kid. But there are people with CF that are willing to take that chance! Also, there are people that have one child with CF and decide to have more children, despite the risks. I think that it is crazy...but I have met some people that are really adamant that no matter how bad CF is, life is a "gift", etc. There is some debate in the CF community about the genetic testing and trying to get rid of CF that way and then talk of eugentics, etc., comes up.