IAmA 17 year old with the condition Cystic Fibrosis, AMA
Thank you! I wish you the best of health
My ex-girlfriend passed away this past August at 26 from complications dealing with her lung transplant required for her CF. She was a beautiful person. RIP Heather. I don't have a question. I just want to tell you that I have an idea of what you're going through and I'll say some words to the sky for you.
I'm so sorry to hear that, I hope you are okay and I know it probably means very little coming from a stranger but you would have meant the world to her and have been the best thing for her when she was sick, there is nothing more comforting than having someone who loves you and cares and holds you, she was lucky to have someone like you
I too also have cf, I'm 36 and have been quite lucky up til now. I was healthy enough growing up that i was able play sports and had to stop around 33 because it finally caught up to me.my lung capacity is around 40 percent. And up until now never spoke to another person who has it. What meds are you taking?
I'm really sorry to hear that you've been unwell over the last couple of years. I have only spoken to a few CF people myself. I take flucoxocillan, tobramycin and azithromycin (sorry for the terrible spelling). If you aren't on azithromycin you should definitely ask your specialist about it, it is a complete life saver.
I've tried zithromax in the past and it is not very effective for me, all depends on what bacterias you have. If you're taking tobi then you might qualify for cayston. Do you or have participated in any studies? BTW, 2 things I found what I believe to be detrimental to my health is:
1. Not drinking enough water. If your lungs are well hydrated then it is easier to cough of the mucus (10 years to figure this one out).
2. When I was prob about 27 my blood sugars started to rise and the doctors didn't test for it. So initially, I got sick every 6 months and had to go on IV antibiotics. After being diagnosed diabetic(common in CF) my health got better and haven't been on IV since.
I have only participated in an insulin study (so number 2 is checked off). I never realised that about the water but it is genius! I never drink water but I will definitely start now
Azithromycin is actually more commonly used in CF for it's anti-inflammatory properties as well as anti-bacterial.
Yes that's correct, for that reason it is one of the best medications for cf people
Knowing that you might not live for that long, what do you plan to do with your life?
Secondly, how do you feel about this all?
It's not something I have put a lot of thought into to be honest. At present I have the same plans as most.. travel, university etc. But when I get older I dont know if I will be able to have/will want to have children. I'm a pretty positive person, but occasionally when I'm very sick and have spent weeks in hospital or missed out on something I get pretty down and start to question whether it's worth doing all the treatments and things that keep me healthy
Have you read the fault in our stars by John green? It really opened up my eyes to this terrible disease. Thanks for this AMA
No I haven't but I will definitely check it out, thank you heaps!
I cried the whole way through it. You said before you feel like you won't ever have children... I guess I have two questions: do you think that is harder to process as a woman? Also, is your body physically capable of handing the stress of pregnancy, and it is your choice not to try?
Ill try to remember to talk to you after I've read it! I'm still quite young so I can't really say how it will effect me yet, or whether its harder for a girl. But I do really love kids and spend a lot of time around them. It is possible to be pregnant if you have CF and I have heard of some successful stories and some not so successful. It does have an impact on your health, but my main concern would be whether its fair to bring someone into the world if you don't know how long you'd be able to look after them for
My sister has CF as well, she is 27. Almost 5 years ago she had a double lung transplant. It's funny to hear someone ask "what sport do you play?" Not being rude, just honest, my sister has never had the energy to walk up a lot of stairs much less play a sport
I hope your sister is doing well! Thats definitely the case for most CF people, luckily for me I have huge lungs so my lung capacity is usally quite high. But I definitely know how hard it is to do the simplest of things like walk up a flight of stairs or walk down to the bus stop when you're sick
I'm 24 and also have CF. Currently, the longest known living person with CF is in their early 50s (well, that might just be the longest living patient at my hospital, pretty sure there are older CFers out there). With the recent developments of treatment for certain gene types, hopefully CF won't be such an issue in the near future for many people. I'd also be happy to answer any questions. I will also go through and add to questions that may have not been fully answered by kirra5152.
If anyone's interested you should come join us over at /r/CysticFibrosis where I help moderate. We do weekly check ups and try to inform and help others affected by CF.
Edit: Thanks for the Reddit Gold, whoever you are!
Thank you for this, and definitely add to or correct any of my comments!
I don't think people here fully appreciate your daily regimen and challenges. Can you please explain your routine (drugs taken, vest usage, etc.) and what happens if you miss one or two of them?
Well firstly if I miss a medication or treatment it can range from severe stomach cramps and diahorrea to ending up in hospital. My daily treatment goes like this: Morning - wake up and inject myself with slow releasing insulin for my cystic fibrosis related diabetes (CFRD). Take an oral antibiotic called staphlex. Have breakfast and take enzymes, up to 5 depending on how much fat is in the meal. Day - take enzymes every time i eat, including breakfast and dinner this can mean up to 30 enzymes Afternoon - inhale nebulised hypertonic saline (usually takes about 15 minutes and makes you cough like crazy) then do physio using a device that you breath in and out of with a resistor (takes around 30 minutes) then inhale nebulised tobramycin with is another antibiotic (takes about 10 minutes) Night - take three different kinds of vitamins since my body doesn't absorb them naturally, take two different kinds of antibiotics (all oraly) than check my blood sugar levels using a finger prick
Do you know why cystic fibrosis caused your diabetes?
My school raised money for the Cystic Fibrosis Trust in the UK, seemed like they were doing a lot of good work and raised a lot of awareness for the disease.
I remember hearing that CF is equivalent to constantly breathing through a straw, does that sound about right?
Because mucus has block my pancrease the enzymes inside it have damaged it to the point where it has turned to scar tissue and pretty much stopped functioning.
That's really good to hear, the cf trust do a lot of really good work
And yes it is definitely like breathing through a straw. Or like when you take a full breath then try to take another breath without releasing the first one
I'm intrigued by all the antibiotics you take. Is that a typical treatment for cystic fibrosis? I've honestly never heard of someone taking antibiotics chronically, so I guess I'm just wondering what that's like. Since you're on so many antibiotics, do you not get sick easily? Sorry if this is a stupid question but I think it's really interesting!
The antibiotics prevent me from getting infections but not the cold or the flu, if that's what you mean?
The clinic my daughter goes to has started testing for diabetes every 6 months, she's on Tobi & Pulmozine every other month, and is currently doing her Vest therapy while watching TMNT. Do you have any words of wisdom for a her growing up?
It sounds like a really good clinic. Hmm words of wisedom, exercise, enjoy eating, sleep a lot and keep positive
What type of vitamins do you take? Are they special for CF or just the normal ADEK?
There's a couple of different ones like folate and vitamin D. But vitabdeck is the main one - it legitimately tastes like vomit. I'm not sure whether it is prescription or not though.
I grew up with my sister having cystic Fibrosis, and my mom was a single mom. So we spent a lot of time hanging out at the hospital. Our favorite games was wheel chair racing, what is yours? Also favorite hospital food?
I hope your sister is healthy and doing well Wheel chair racing is a favourite, baloon tennis kept me going last time i was in hospital too! On sunday mornings they give you a cooked breakfast.. that would definitely have to be my favorite
I was in the hospital a few years ago for a month with a collapsed lung, pneumonia and a lung abscess. It's an awful feeling having your organs hurt and I wouldn't wish it upon anyone. Oatmeal was my favorite hospital meal because I would load it up with brown sugar and butter. Thanks for sharing your story.
How do your parents handle the insurance? My trip with insurance still cost me close to 80k.
I hope you're better now! I don't know where your from but I'm from Australia, so medicare pays for everything.
Wow, i too have cf and i feel like everything i just read came from my own mind. Iv really started to notice through the years that people with cf seem to be really down to earth people. So from one Cfer to another, every time u go to the hospital, do u feel like u'll always get back to ur baseline no matter how sick u are?
I completely agree, I haven't been in contact with many CF people but everyone I know is such a nice honest person. I have a pretty high baseline, I have been one of the lucky few that doesn't get sick very often. I always thought that not matter how sick I get I will always be able to get back to my normal but I got really sick in october last year and I'm still struggling to get back to normal, so I'm not sure i do believe that so much anymore. How about yourself? If you don't mind me asking of course
Ever since i hit puberty, my lung function dropped around 30% and it seemed to drop like 2% or so with each hospitalization. But even with that large of a drop i feel and still feel that my lungs are at least going to get back to my baseline and when it doesn't i think of it like a test in school, its only a % or 2. I say i got cf pretty bad, but if i can still do physically demanding things for fun then why worry about pft's that much. The way I c it, if i cant do anything physical that's probably when its time to worry. U said u surf, so do u gauge how well u are doing by surfing?
I don't surf a lot, i think that response was in answer to what sport do i watch. I do try and do a lot of running when I'm well and have represented my region for cross country before. So running is usually how i gage it. Some times i can't run at all, others i can run for 5kms
haha my bad. Since u left the hospital have you tried running? U thinking your not getting back to normal cuz u cant run has far?
I have gone for short runs but I'm still building up to longer distances
My daughter has CF, she just turned one in December. She is doing quite well, though we've already had to spend a week in the hospital for a serious round of antibiotics. My question to you is, is there anything you wish your parents would have done differently for you? Do you ever find it hard to eat as many calories as you're supposed to? Can you say something positive about having CF? On the day to day I don't have to think about my daughter's disease. But whenever I hear these stories about people dying so young I get so sad. Here's hoping that Kalydeco makes a better world for us all. Good health to you
My parents have been incredible, they are constantly researching every possible treatment and risk. And although this meant i missed out on a lot and felt overly protected by my parents i couldn't thank them more now because i know that is what has kept me so healthy. The only thing i wish is that now that i am older and more mature and looking after myself is that they trust me to know my own limits. There are definitely positive things about CF, its taught me to value my health, made it even easier to stay away from deathly things such smoking and binge drinking, and given me a group of friends that i know genuinely love me. The calories are my favourite part of cf, i have a stash of my favourite foods in my cupboard and eat as much and as often as i like without becoming overweight, its fantastic. I wish the best to you and your daughter, if you want i can give you an email address or something if you have more questions for me or my parents
Have you ever had a chance to use The Vest? My dad used to work for the small company that developed it, though they got bought by a major medical co, so it probably costs more to have nowadays. Always sounded like a remarkable treatment for CF though.
No it isn't quite popular in Australia yet but from what I've read it sounds incredible!
When my son was born, I found out he was a carrier. It was the scariest month of my life waiting for that sweat test. How do your parents handle it? Did you get treated with kid gloves or were you able to have a pretty normal childhood?
My parents are both scientist so when they found out they practically just researched day and night until they found out everything they possibly could. I definitely got treated with kid gloves, everything was disinfected, washed, sterilised. I wasn't allowed to go preschool, we moved house because there was too much mould in our house, i wasn't allowed to see any family or friends if they were sick. My parents are still extremely controlling now, I'm not allowed in heated or chlorine pools, I'm not allowed late nights incase I get run down and I carry a bottle of detol everywhere I go. In spite of all this I'm so thankful that my parents do all this because I doubt I'd be so healthy without it.
is there anything positive you can think that you would get out of this?
Yes, you can eat anything you want and as much of it as you want and not put on weight. If I put on weight as easily as a normal person I would be obese. Granted although this is a perk for girls, it sucks for CF guys who are very thin and can't gain muscle easily.
I have Cf and I recently put on a ton of muscle you just have to work hard and eat heaps. do you have to take creon? doesn't help when you cant diegest anything but you can get there. the only thing is keep the carbs and protein up when you're sick. it melts off you if you dont
Yeh i take creon 10,000 i don't have too much of a problem with weight, i just enjoy eating haha. Best thing about cf, being as skinny as the skinny girls but eating as much as obese men
Hey, CF research scientist here. I work primarily on the infection aspect.
Which allele(s) do you have?
Forgive me but what's an allele? Is it another word for growths?
My girlfriend (also age 17) has CF. I struggle to help her when she is down, I ask and ask what I can do to help but ultimately I will never understand what she is going through. Do you have any advice on what I can actually do? I try to keep her up with her diet and exercise, but is there anything more?
Also, stay healthy, I wish the best.
This is so lovely of you, you have a very lucky girlfriend I think the best thing is to make her always feel comfortable around you even when she's sick. It can get quite messy when you're sick but my boyfriend has never made me feel disgusting for coughing up mucus or not being able to keep up with him. Also personally i have a lot of trouble with my stomach and there is nothing better than having it massaged (as weird as that sounds haha). I'm sure more than anything she will just appreciate having someone who cares about her
I am a male who carries the cystic fibrosis gene. I'm sure you probably know, but this means that if my partner also carries the gene, there's a 25% chance our child would develop cystic fibrosis. You might not know the answer but: If I was to have a child, what is the detection process (if at all) for the unborn child? Is it possible to have the fetus checked before born and potentially abort the child, should it be found that the child has Cystic Fibrosis? I'm sorry if that's a strange/hard-hitting question, but I've always wondered it since I found out I carried the gene.
Well from what my parents told me, the best option would to put a couple of eggs in one dish and sperm in the other and pick the ones that didn't have cf and use them. I know that that way is definitely possible but if you see a genetic councellor they should be able to give you more options
Hey, I'd just like to ask you two questions if you're still here. What kind of treatment do you get? Have you heard of Kalydeco? My country, Ireland, just added Kalydeco to the national health service. This will result in around 200 people in Ireland being able to get Kalydeco free of charge.
I look forward to your answer, if you're still here.
Still here, no sleep for the weak. I have a whole heap of different treatments including nebulisers and antibiotics, but i don't use kalydeco. Unfortunately kalydeco.only works on certain cf mutations and not my type
I don't know where you're from but Ireland has the highest incidences of CF in the world and just last week our health service announced plans to provide Ivacaftor (Kalydeco) for all CF patients with the mutation that leads them to gain benefit from this drug. Have you heard about Kalydeco because there's been quite the buzz about it in recent weeks for limiting the number of antibiotic required infections?
From what I've heard (which may not be correct) kalydeco only works on a certain mutation of cystic fibrosis
I know this may sound wierd but if there was a cure to your disease would you be cured, however you would still have the pain/cough but just wouldn't get the infections. Anyways thanks for doing this and I'm sorry if this question offended you.
No its okay I'm pretty sure I understand what you mean. If there was a cure it could not repair any damage that had already been caused but it would stop patients from getting any worse and would make their existing infections easier to treat
No sorry, i dont think kalydeco has hit Australia in a big way yet. Make sure he takes all his vitamins and eats lots because most boys get self esteem issues from being skinny and not being able to put on muscle etc
What are three things people with CF wish the healthcare community were doing more of and is there anything professionals incorrectly assume and therefore wrongly attempt to solve for in your opinion?
Personally I wish the healthcare community would 1. Raise more awareness of CF so the wider community would have a better understanding 2. Better management of CF clinics so there was less cross infection 3. Cater for the psychological impacts of the disease, especially on adolescents who miss out on school, parties and camps and feel isolated and give up because of these things. I absolutely love my specialist and have very little bad things to say about him, the only thing that I find hard is the lack of focus on digestive problems. It seems doctors are only concerned with the lungs and often neglect digestive issues
Are you hopeful that as the years go on, the life expectancy will grow with medical advances? Or do you ever have the "35" thing just lurking in your head on down days?
Very hopeful, there are so many new drugs being realised. They have already found a cure for one type of CF, unfortunately for me its not my type though. Its rare that the 35 expectancy looms in my head but it sometimes gets to me when I'm very sick, or when in class and studying the life expectancy of those in third world countries and mine is equal or lower to theirs and everyone in the classroom knows it too.
Glad to see a CFer doing so well! And definitely, exercise is key.
What are your thoughts on the 16 year-old with CF who auditioned for American Idol this week? Link
American idol loves drama that goes without saying, i think the boy may have mentioned 'terminal' a little too much for my liking and failed to raise any legitimate awareness for the cause but in all honesty he was probably just giving american idol a sob story they would enjoy
Thank you for doing this! I think i read somewhere its the infections of the mucus that cause the problem..is this true? Or is my cuntbrain fooling me
Yeah thats kind of true, its caused by a defect in the cell that basically makes all the mucus in the body very thick and sticky, which makes it really hard to clear mucus and makes it really easy for infections to grow
Damn, is it uncomfortable? (having the nonproductive cough) and do they do blood work regularly to check for infections?
When I'm sick and I have a bad cough it's pretty ugly. The cough sounds disgusting and you lose so much weight from swallowing your own mucus and therefor not feeling hungry. Blood work is done at least every 6 months, but if you are sick it is done more regularly. Blood work is usually just to check if you have enough of each vitamin because it's hard for CF people to digest food well. They check for lung infections through samples - basically when you cough up mucus you put it in a jar and send it to the pathologist.
I want to be a pathologist! Maybe some day i will analyze your mucus!
Thank you for answering these questions. I wish you the best of luck and health
Haha well I would be honoured to have my mucus analysed by you one day. Thank you, you too!
What do you spend most of your time doing? 1: favourite sport to watch 2: do you like games?
I spend most of my time doing what normal people my age do, see friends, school, go to the beach 1: Probably surfing, although some don't call it a sport 2: What kind of games? Forgive me I'm Australian and don't know if you mean sporting games or not haha
Since you cant be around others with CF, do you find it very isolating / lonely? Would you like to have the ability to connect with others your age with CF?
Definitely, there have been so many times I've just wanted to talk to someone who understands exactly what I'm going through. It would also be nice to recieve/give advice to other CF people on how to manage certain treatments etc
I'm not too familiar with CF. Why can't you be around others who have it?
There is a really high chance of cross infection between CF people
I'm sorry if this is inappropriate!
Cystic fibrosis is kind of (in)famous in molecular biology because it's the textbook example of a genetic disease. Similarly it's a go-to example in bioethics courses when talking about cause and effect.
On the ethics part, what do you think about abortion on basis of genetic screenings for CF?
Ethically that's a really hard question. I hear about so many people living poor lives and dying young, and on the other hand hear about people living healthy into old age. I think its up to the parents to make the decision - but i think it would hurt a lot of CFers if people were to abort. No one wants to feel like a burden, or to feel like a mutant.
Does the desiese effecting your lungs affect you, like getting out of breathe easily?
Yes definitely, it makes it a lot harder to get fit and takes a lot more time then most.. but is still possible if you're relatively healthy. But when you're sick you get out of breathe doing simple things like walking from class to class.
Are you in line for a lung transplant?
No, luckily I'm not bad enough to need a transplant at present
I have a friend who has CF, and another lung disease. Poor guy routinely is under the weather and coughing up blood. Because of his lung disease, he will never be allowed a transplant.
Im so sorry to hear that, the way transplants are dealt with is utter bullshit
Are your friends aware of your condition? What effect does it have on your social life? Lastly, do you find yourself having to take painkillers when it gets bad or is it more just debilitating and not painful?
Also, it's really cool how you tough this out, bravo.
Most of my friends are aware, but they usually forget when I'm healthy until i start popping pills with my lunch. For me its only once been so bad I've needed painkillers, but I'm not sure what its like for others
I have a Niece with it who is 20. Her lung function has held up pretty well. How have you been doing? She was diagnosed from birth and has been on enzymes essentially all her life. Still quite healthy. What's your story??
BTW: her twin is a carrier, but doesn't have it.
That's really good to hear, there's so many negative stories about people with CF its refreshing to hear some positive ones, i hope she stays well! I was also diagnosed at birth and have been taking enzymes all my life. Luckily for me I have big lungs so have always had quite a good lung function.
When you tell people you have this disease do they treat you diffrently?
Most people here have never heard of it, or know very little about it. But of the few people who do know most treat me like I'm about to drop dead.
I'm 15 and a good friend of mine has CF and has a very positive attitude about it. Do you feel like as you have gotten older your outlook on your life with CF has changed?
A little bit, not dramatically. I was very lucky and didn't get very sick until the end of last year, before you would have never guessed I had anything wrong with me. So I think that put things in perspective a lot for me.
I don't have a question, I just want to say good on you for having a positive attitude about this. My little cousin has CF, and her life has been hell so far from it. I've lost track of the number of hospital visits/surgeries she has gone through. Hang in there, life is what you make of it.
Thank you, i hope she gets better soon. My thoughts are with you and your little cousin
How exactly does it affect your organs? I don't know anything of the disease honestly, other than it's something that you are born with, not that "get" or "catch".
Basically anything that uses mucus is affected. So that's mainly lungs and pancrease (which breaks down the food) which means digestion is greatly effected too. There are also weird things like having clubbing (swelling in tops of fingers) and sweat containing ridiculously high amounts of salt
Hey I have cf too and I'm almost 28. Keep your head up, medical advances are breaking new ground for us every day. Don't neglect treatments either :)
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