IamA Cystic Fibrosis sufferer currently in hospital AMA!

I have cystic Fibrosis too! I'm actually sitting in the hospital as I type, awaiting 3oclock for my dischargal! :D it has been 2 weeks as of yesterday and I've completed everything. I'm 21 and my name is Gina. (sorry that this isn't a question, but I had to say something!)

I'm sensing that there should be a meet up for you two

The unfortunate part is people with CF actually shouldn't be around other CFers. I'm sure OP can shed more light on the matter, but I believe it's frowned upon as they can make each other more sick.

Is this medically proven? Why is this?

It is what the hospitals call an Isolation policy. I don't think there is any proof that it works but they just cover their bases. I personally think its BS as I remember a time when I was younger when a group of us would play together on the ward and it was no different in terms of cross infection.

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r/ladybonersinhospital

Did you make an account to say that...

Lol. Where are you in the world?

South Carolina

It was only when I was about 8 I realised people all around the world have the same problems I do. I hope you get out soon!

How long have you been in the hospital? I hope everything goes well for you best wishes my friend.

I've only been in this time for 3 days so far. My usual admission length is 2 weeks at a time about 3 months apart each time. I am now 20 and this has been happening since I was 18 months old so you can imagine that is quite some time, but you do get used to it.

Thank you for the best wishes too.

What are the kind of treatements you could eventually benefit from ? I know you must "purge" your lungs daily through painful massages and coughing (How bad is it, by the way ?), are there any durable cure for the disease being worked on ?

I ran a painful 5 kilometers few years ago with high shool, in order to collect funds for the
research of a cure, hope it helped you somehow.

Sorry if I sounded a bit rude, english isn't my mother language, I hope the best for you.

The short answer for the bulk of your question is that there is never going to be a "Cure" that can help me. The long answer is that there are several treatments being worked on to help alleviate the worst symptoms of CF. The main one being Gene Therapy, I was actually part of the clinical trials of this until I developed Liver disease (Owing to the number of drugs I am on) and that could well help people like me get on with "normal" lives for much longer periods.

In Response to what normal treatments I go through on a daily basis, I have to take several Nebulisers which helps to treat any infections in my lungs. When in hospital, I have a 2 week course of different IV drugs which are much stronger than normal and help to really "attack" any infection.

Thank you for helping any CF charity. It is always nice to hear about, and I know first hand The research it helps.

Which drugs do you IV? Every three months I do a course of tobramycin and ceftazidim intravenously. Is the IV treatment the reason you're in hospital? When I lived in the UK I had to go in for the treatment, but here in Norway they offered me a portacath-thingamebob, which makes life alot easier, seeing as I can do my treatment at home.

I have a triple combo of Tobramycin, Ceftazidim and Caspofungin.

We just moved to a new school district and my daughter's new best friend at school has CF. The girl with CF disappeared for 2 weeks and is now back in the hospital... what would you recommend I do? Should I take my daughter to visit her friend with CF in the hospital? I read your comments and I see you just want to be treated normally, but it's hard to not feel some sympathy for the kids situation. Did you like visitors when you're stuck in the hospital with CF?

I personally love visitors but I would always check with the person in question (Or their parents) and I would brace yourself for a shock as sometimes (I know this through personal experience) the person in question can look a shadow of what you're used to seeing. Keep that in mind but on the whole I would recommend visiting.

I have CF myself and just wanted to say good luck on the stay. In the end I went 4 months on IV antibiotics and it was an absolute bitch, got new lungs 1.5 years ago and I have almost forgotten what its like to have a CF cough!

What are you doing to stave off boredom? :)

The Natural History Museum is nearby so I will probably go there weather permitting. If I'm stuck inside I guess I'll have to browse Reddit!

Talk me through a (hospital-) day.

I get up at about 8am to have some observations (Heart rate, Blood Pressure & Blood Oxygen Level) as well as breakfast and my first IV Medication (Spoiler!: There are lots) Then I have some Physiotherapy which in CF cases consists of having your chest "patted" firmly for about 10 minutes. During the day you see lots of people including a doctor of some kind (Usually a Registrar) a Dietician and an Exercise Specialist. After lunch you usually have IV medication and then more Physiotherapy, at about 10 at night you have a final set of IV medication and then (For me anyway) you start an overnight feed though a special tube called a PEG

Thanks for the answer! Why do you get your chest patted? How does a normal day outside of hospital look like?

It helps to drain off some of the excess mucous that my lungs produce and loosens it to help cough it up. A normal day outside of hospital looks like anyone else's for the most part just with a few pills thrown in and the feed at night. For the most part you have to understand that I can't get what a "Normal" persons day would consist of that would be different (Aside from the drugs and physiotherapy) For me this is normal.

Thank you! Last question (promise!): why do you have a PEG and why do you get fed overnight?

I have a PEG Because I can't put on weight through diet alone so I have to have a very high calorie supplement put through it and the reason it is at night is that it is most convenient then.

What is your life expectancy with CF? How do you cope with knowing your lifespan will be much shorter than the average person's? Is it very painful on a daily basis to live with such a disease?

The life expectancy is rising but I think it is currently mid 30s as a life expectancy and I have lost 3 friends this year alone. It can hurt losing friends when your young, I was 8 when my first friend died of CF when he was 10.

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My view is that you either have to get on with life and look at the positives or you'll go mad or become a very horrible person. Honestly think the Python song "Always Look on the Bright Side of Life" is a very good motto.

What is something you wish more people knew about your condition?

I wish people knew more about the fact the condition exists rather then anything in particular. Its getting more and more well known but having to explain the basics to people can get annoying.

What was going to school like? Or were you homeschooled?

I was very lucky. I went to a normal school for most of my schooling but when I was in hospital I was looked after by the Chelsea Community Hospital School they made sure i didn't lag behind and when my health got the better of me I started going there full time between the ages of 16-18.

How old were you when you started showing symptoms? Did your parents know before you were born that it was likely that you would have CF? Are you able to eat essentially whatever you like without gaining weight because of the enzymes and digestive system workings? Were you frequently sick with the flu and colds as a child? My nephew has CF and I'm very curious....

I was Diagnosed "late" at 18 months old. I had been shwoing symptoms from day 1 but it wasn't clocked for that long. In terms of diet I am encouraged to eat anything I want as I need all the calories I can, even with this I have had to have a PEG inserted and have nightly feeds to keep my weight up. As for did I get colds a lot; strangely no, I know of quite a few friends who are a lot more "Sickly" than I am, I've always put it down to the fact that I take a bucket load of drugs that fight a hell of a lot. But I do get hit hard when I do get a cold ect. as my immune system finds it one too many things to deal with.

What has been the best support method so far for you?

aka - what's the best way someone could make you feel better

The best support method for me is that people treat me like normal. I don't like people making a fuss of me and I always try to keep the same attitude of being talkative a little bit cheeky to everyone. IMO the worst thing people can do is treat me like I am "Different" It can feel very degrading.

If possible, is there a favorite part or least hated part of the whole hospitalization?

My hospital is The Royal Bromton Hospital in London. One big plus of being in is that you're in the heart of the city and there is so much to do nearby.

Do you take prescription digestive enzymes? If so, are there any tests that you can get to give you an idea of how much and what type of enzymes.

Are your frequent, somewhat lengthy hospital stays about mostly one problem or one symptom? If your lungs get filled with fluid or mucus (not sure what it is exactly) how can you treat that? I realize prevention would be the best option, but is prevention a problem if you're confined to a bed?

I do take Enzymes, the mix of the ones I'm on are equivalent to: (per 150mg) 10,000 PhEur Units Lipase, 8,000 PhEur Units Amylase & 600 PhEur Units Protease.

The main Problem I get admitted for is a chest infection caused by one of a few thing that live in my lungs (Yes I have several deep rooted infections that LIVE in my lungs: One fungus, One Mycobacterium and a few others) If they flare up then they have to be treated. The other thing I get admitted for is to have semi-frequent courses of anti-fungals to make sure the fungal infection doesn't get out of hand.

I was born with EPI (exocrine pancreatic insufficiency), but I don't have CF. They gave me a series of injections to kick-start my pancreas, and I began digesting within a day or two. Is this not an option for you, since you have CF?

It's not that my pancreas doesn't work, but rather there are thick mucous secretions coming from it rather than proper enzymes. (I think that's how it is but anyone who knows feel free to correct me)

How does it make you feel when religious groups oppose research that could either cure your condition or prevent newborns from even having it?

It infuriates me. Religion is all very well and good, I don't mind my religious friends praying for me or anything like that but when I think that people are against newborn or even babies being given the best chance in life it makes me feel sad and angry. I wouldn't wish my worst enemy to have CF let alone a helpless child.

I was a Pulmonary nurse in the mid 80's and had hours of percussion and postural drainage to do with patients. Do they still do that? I got pretty good at it and still use it to knock phlegm out of my family members chests' during their colds.

They still do it for younger and infirm (Oxymoron I know!) people but I now use a PEP device which puts resistance to me breathing out and thus makes my lungs work harder shifting the mucous.

Hi, physical therapy student here learning about CF and other respiratory disorders...what PEP device do you use? Have you ever used The Vest before? Thanks for this AMA!

I use a PARI PEP and no I have never used the Vest, I have use The BIRD though.

My uncle has CF. I visited him in February, and he would have to stop to catch his breath while we were just sitting and having a conversation. He recently had a lung transplant, and... Well... Here he is...

That's great to see!

Are you afraid of death? Are you religious? Sorry to be dark and I'm not trying to make you uncomfortable, but I'm just curious as to what your views are. I've had friends die of C.F. and they were always so calm about the reality and were strong in their faith.

I am afraid of death. But more of that fear rather than what death encompasses.

As for Religion. No I am not very religious, I can't see how a Divine being would give a baby something as awful as this even as a "test" I put my faith in the fact that there are people out there who are trying their hardest to find a cure for those who come after me.

Odd question...My dad does a lot of CF research, have you ever taken TOBI? He was one of the main developers for Tobramycin.

Yes I do. I have it via IV and it works wonders for me! Tell him I said Thanks!

My dad's response: "I really didn't do much, but that's neat. Working on getting another inhaled antibiotic approved for CF right now, but FDA being tough."

First-world pharmaceutical researcher problems, amirite?

I don't think there is such a thing as "First world" in this case. It help everyone and so is a problem in general.

Would you be offended if you heard two grown men referring to each other as "Cystic My-brosis", in the same sense that people say things like "bromosexual" or "Bros Equis"? Because my friends and I use all of these frequently and I'm really hoping we haven't been infuriating people...

I'm a pretty lax person and an advocate for tolerance, if you want to say it I personally don't care, others might feel different though.

My brother had CF. He didn't take very good care of himself. He was always depressed, always in his bedroom, smoked pot all day. My parents were willfully ignorant. He went for the tuneups and took his meds, but that was it.

He had a double lung transplant at 22 and had a good couple of years. The steroids caused necrosis in his knee, and after surgery his knee never healed. That sent him to the hospital wear he then caught that horrible respiratory staph infection that is hard to kill. It was that which killed him.

I am always so worried for people with CF and/or transplant patients. Hospitals are a cesspool of diseases and are the last place people with an immune deficiency should be.

Question I know physical activity in general is probably a pain in the ass for you. Do you overcome it and push yourself to be active? Do you lead a more sedentary life? Also, do you allow yourself life experiences such as partying, drinking or drugs? I've only known two people with CF. My brother, and a friend who is anti drugs and alcohol and is very very active.

I am more of a Procrastinating layabout when it comes to exercise but in terms of substances its all a no-no. I have Liver Disease and so wouldn't even contemplate doing drugs or smoking, I have a drink maybe once every 6 months but that is it.

What are your PFTs and weight/height on a good day?

On a good day my PFTs are about 65-70% and my best ever Height Weight was 176cm 62.5kg

Can you translate those measurements into American?

5ft 9in 140lbs

I wish you the best of luck in your life. How do you get used to being in a hospital for that amount of time? That's something i don't think I could do. What do you hate most (and least) about being stuck in a hospital?

The thing that you have to understand is that I've been coming into hospital for several weeks at a time for nearly 19 years, ever since I was little, It just becomes second nature and you get used to it. The single worst thing for me is having a long line / cannula in my arm the whole time, it helps that I'm not needle phobic but it still sucks.

Are you considering getting a lung transplant? If so, how much does it increase your life expectancy?

You don't "consider" you either are unwell enough to need one or not. Thankfully I don't and hopefully never will but I can't answer the second part I'm afraid.

Are there any breakthroughs in medicine that you might be trying soon?

I personally probably will not owing to the fact I have Liver disease as well.

My sister has CF and lately she's been pretty depressed. My family and I have tried a lot of things but it seems she just has to come to the realization that she can still have a great life even if it is shorter then some. You seem to have a great outlook on life and I was just wondering how you came to it and if there was anything I could do to help my sister see it the same. Hang in there!

P.S. With all of the money the CF Foundation has been making and all the research companies have been doing we have already seen massive improvement in medication and I honestly would not be surprised if they found a way to double life expectancy in my and your lifetime.

Something that I find can help sometimes is to see a Clinical Psychologist . I see one every now and then if I'm starting to feel low as it help to have someone neutral to empty your heart out to, all your hopes and fear told to someone with no bias and who won't judge you. I can't actually think when I came to have such an outlook. When I was younger I was always playing and laughing and all the doctors and nurses said "Make sure you always keep this optimism" and I have done. It make me feel better especially when I think about all the friends I've lost. I have to be happy, for them if no one else.

What is pain management like when you are not in the hospital? And in the hospital as well? Do you find that your methods of pain management work?

I don't actually use pain management and I am incredibly lucky with that for more than one reason, not only am I not in pain but the only painkiller I can have is paracetamol which isn't the best.

I'm curious - do you know what mutation you have, and if so, did your parents know they were carriers?

My second question is a little more personal - a friend of mine growing up had CF and her mother and father felt guilty for passing it to her and ended up in a pretty bad divorce. How have your parents coped with the diagnosis and do you think they feel any guilt?

I can't remember what mutation I have, sorry. As for my Parents, I don't know and probably never will. I think that's an area that is too personal to them and I would feel wrong asking. I know they love me very dearly and always want what's best for me. (Even if it involves nagging me to do my physiotherapy)

I hope I'm not being insensitive, but how have you coped with the understanding of your own mortality? You can presumably keep chugging away, so there must be something in your life that gives you purpose. What is it?

And have you given any thought to how you wish to die?

You know I don't think there is anything "giving" me purpose apart from the fact I don't want to leave anyone behind as it were.

As for your second question does anyone know how they want to die? I am of the opinion that when your time is up your time is up. Not much you can do about it.

My twin brother's wife recently passed from CF. I spent the last couple of weeks with them in the Mayo Clinic. I got to know several patients and you can't find a group of people that are more full of life and a positive attitude.

My heart goes out to you.

My question for you is what is your favorite book and why do you love it?

My favourite book is an anthology called The Saint by Dan Abnett. It consists of four stories in the Gaunts Ghosts series set in the Warhammer 40,000 Universe. I love it because all the stories are very character driven but with beautifully done set pieces.

I have a friend who has CF who I have sports with all throughout high school.

I am just curious about how it has progressed as you have gotten older as my friend besides taking loads of medication and this breathing thingamabob everyday seems fine. Are there different severity of CF as well?

There are not only different severities but it can affect different parts. Some people are only affected by Pancreatic problems whereas others can have Lung problems but their digestive system works fine.

Nursing student here - any advice on what nurses can do to make your hospital stays more tolerable? Wish you the best! :)

The nurses I have are brilliant, I can't actually think of anything they do wrong. As I would say to anyone just treat us like normal and don't be patronising in any way.

I've been reading through this thread and this sticks out for me, is it because of how long you've been doing these 2 week stays that you feel, sometimes, patronized? Like you've probably been there longer than most your nurses and doctors? You could probably train the new ones :p

I don't ever feel patronised, I just like people who have never met someone like me not to treat me any differently. As for the second part, We quite often get Student Nurses and Physiotherapists on the ward and they nearly all seem to end up coming my way, the nurses and physios tell me it's because I'm a good person to get to know and learn a few things from, that I'm very patient when it comes to new people and will explain things to them if they so desire.

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IIRC, ~95%-98% of males with CF are infertile, due to malformation of the vas deferans in utero. Female CF-ers are not usually infertile, but there is a chance.

You are correct.

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Great to hear!

what are your thoughts about Bob Flanagan?

I have to say that I had honestly never heard of him before looking him up just now. Looking up famous people who have the disease is not something I generally do as Its kinda make me feel sad to see how young they were when they died.

CF dad here. My thoughts are with you and -- if you're anything like my son -- hope your eyeballs fall out of their sockets from all the video games you get to play when you're admitted.

My son is eight years old. He had a very rough start as an infant with months and months in the NICU, then several years of doing well, and now he's going in for IV's about once a year. It's something he lives with, but as others in the thread have said -- so much of what we do is fighting for normalcy.

My questions for you (and anyone else with CF in this thread who want to chime in) are:

-- What is something that you felt your parents did right in helping you deal with your CF? -- What is something they did wrong? -- Was there ever a big conversation about, "This is how serious the disease it, it has x life expectancy, etc."? Or was it always a given?

Thanks and feel better soon.

Life expectancy can always hit hard, I find myself especially low whenever a friend passes away. My parents always pushed me to do the right thing (They still do) I may not like it sometimes but it is always the best option for me; such as getting a PEG. Now weight gain is something i don't have to think about as much.

Edited: Got confused, thought CF was something else.

My question to you is this; Since youre probably bored in the hospital, what games do you like to play/what hobbies to you have?

I play lots of strategy games on pc as well as Miniature Modelling and war gaming. Reading also helps a lot.

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I'll be out sooner rather than later but I never work with Definite dates. All that does is get your hopes up then if something goes wrong you feel even worse.

I don't have a question. Also CF sufferer who goes to the Brompton! Just wishing you well really, was in last November in the same ward you're sitting in right now probably.

Actually I do have a question! What room are you in? I was in 1! It's huge! But next to the call alarm.. you know the one. Bit loud.

Get well soon! How're you finding the food as well?

I've been in loads of them 2,3,6,7,13,14,16,18,19 and probably more. Foulis is great. currently I'm in 14.

Bloody hell, you're in regularly then! I've been lucky to that extent in the past few years, hope you can be as well, know it can be a right bastard though. I like Foulis, good location as well (in terms of what to do around London), South Ken is just a nice area. I'd just go in Hummingbird bakery every other day in a failed attempt to put on weight.

Is there a history in your family? Edit: Assume you have a port? Mines left a good scar, love it.

No history at all, I'm the first (and hopefully last) I prefer Ben's Cookies to Hummingbird personally!

Age? and is it safe to assume you are a virgin?

I'm 20 and the last part is just a little too personal for me to answer.