Now ye're talking - to someone with Cystic Fibrosis

Boards.ie: Niamh

Welcome to our first first AMA of 2019 everyone!

Today's guest is a woman who has Cystic Fibrosis. It's a condition which requires constant monitoring and medication. Ireland has the highest incidence in the world of Cystic Fibrosis per head of population and also has some of the most severe strains of it. Our guest is here to answer your questions about her experience of life with CF.

What is Cystic Fibrosis?
Cystic fibrosis (CF) is an inherited chronic disease that primarily affects the lungs and digestive system of about 1,300 children and adults in the Ireland (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:

• clogs the lungs and leads to life-threatening lung infections; and
• obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.

In the 1950s, few children with cystic fibrosis lived to attend primary school. Today, advances in research and medical treatments, including in Ireland, have further enhanced and extended life for children and adults with CF. Many people with the disease in Ireland can now expect to live into their 30s, 40s and beyond. People with CF in Ireland are increasingly going on to attend third level colleges, accessing employment, and living more independent lives, with the support of family and friends.

Let's get the AMA forum off to a great start with some questions for Succubus_ :cool:

[Info above is from Cystic Fibrosis Ireland]

Ariadne

Hello everyone, I'm delighted to do the first AMA of 2019. Feel free to ask away about anything CF related

Hello! Delighted to see doing this AMA

qo2cj1dsne8y4k

Hey. Thanks for doing this AMA. I lost two very young cousins to this illness so I’m delighted to see you kicking its ass.

What was it like for you growing up with CF?

qo2cj1dsne8y4k

Also, I read somewhere a while ago that CF seems to be an Irish disease, and became apparent around the time of the Irish famine. Is there any truth to that, do you know?

Ariadne

Harry Handsome Bulldog wrote: »

Hey. Thanks for doing this AMA. I lost two very young cousins to this illness so I’m delighted to see you kicking its ass.

What was it like for you growing up with CF?

Thank you very much for your question Shoesday



I'm very sorry to hear that you lost two very young cousins to CF. I feel very lucky to still be here and still doing relatively well as I know many others haven't been as fortunate.



I'm unusual in the sense that I was diagnosed with CF when I was fourteen, so I thought I was just like everyone else. Most people with CF are diagnosed as babies or small children. I did get frequent chest infections and colds as a child and I was misdiagnosed with asthma when I was thirteen because I started to have a chronic cough around the age of eleven. So, I suppose I had a pretty normal childhood in the sense that I didn't know I had CF and so I wasn't doing the heavy treatment regime that was needed. I got away with it in that sense

Ariadne

Harry Handsome Bulldog wrote: »

Also, I read somewhere a while ago that CF seems to be an Irish disease, and became apparent around the time of the Irish famine. Is there any truth to that, do you know?

Oh thank you for this question, I haven't heard this before. We do have the highest incidence of CF in the world per capita (1 in 19 Irish people carry one CF gene) but it isn't an Irish disease.



I suspect we have such a high incidence possibly because we've been a bit more isolated on an island and so the gene pool is smaller. Also because CF is more common in white people though it is also found in other races, but it tends to be more rare.



We don't know exactly how long CF has been around but one of the genes I have (which is the more severe one, DeltaF508) is thousands of years old. People wonder why it has survived since it's such a deadly gene so there have been theories about the benefits CF can offer, such as being more resistant to typhoid fever and cholera. Though we don't know why it's more prevalent in Ireland then as that wouldn't have been a prevalent disease here compared to other countries.



CF wasn't officially recognised as a disease until the 1930s but in European folklore there was a saying “woe is the child who tastes salty from a kiss on the brow, for he is cursed, and soon must die”. I think that was in the 16th century or so. People with CF lose a lot of salt through their skin and often taste salty to kiss so this is what people must have realised.

I have no question. Just want to thank Boards for this discussion and wishing Succubus and all those living with CF all the best.

harry Bailey esq

So Id be wasting me time trying to sell you cheap tobacco so

Ariadne

harry Bailey esq wrote: »

So Id be wasting me time trying to sell you cheap tobacco so

Well we're all human and some people with CF do smoke. I don't know how they do it because even if someone smokes around me outside I start to cough but maybe their lungs are in better shape than mine.

harry Bailey esq

Succubus_ wrote: »

Hello everyone, I'm delighted to do the first AMA of 2019. Feel free to ask away about anything CF related

Fair dues cleo. It's a lot more common than people think so it'll help a lot of people

miamee

Do you have to follow a strict diet or avoid certain foods, like maybe dairy?

Will you need a lung transplant at some point in the future or is that not the case for everyone with CF? I don't know much about the disease but would have read a bit in the media about Orla Tinsley being on the waiting list last year and eventually getting a transplant.

MetzgerMeister

Thank you Succubus for doing this AMA.

My sister was diagnosed with CF at the age of seven. She is incredibly active although her lung function fluctuates from time to time. I also have a friend who had a double lung transplant a few years ago and is doing very very well.

How does CF affect you physically?

Ariadne

miamee wrote: »

Do you have to follow a strict diet or avoid certain foods, like maybe dairy?

Will you need a lung transplant at some point in the future or is that not the case for everyone with CF? I don't know much about the disease but would have read a bit in the media about Orla Tinsley being on the waiting list last year and eventually getting a transplant.

Thank you for your questions Miamee

I should really avoid certain foods because I have acid reflux which is very common in CF. I take several different medications for it but it can still aggravate my lungs (the acid can leak into the lungs and inflame them) and make me cough which makes me vomit at least a couple of times a week. It's usually brought on if I eat something or climb the stairs, if a coughing fit is brought on really. With reflux you're supposed to avoid a lot of foods, like spicy food, onions, chocolate, garlic, tomatoes, caffeine and alcohol. I try to take it easy on the alcohol and spicy foods but that's about it. I find it too restrictive to cut out all of the above foods. I haven't noticed any food that particularly makes my lungs worse but my lungs are definitely more productive (produce more mucus) the day after I drink alcohol.


Most people with CF need a lung transplant at some point really, so I will need one in the future, though nobody can tell me when. My lung function is 51% when well and you're generally referred for transplant when it reaches 30% so it really just depends on how quickly or slowly my lungs deteriorate. For example, when I was 19 my lung function was brilliant, it was 93% at best, now 11 years later it's 51%. So the main thing is to do as much treatment as possible to try to slow down the deterioration and that's about all you can do really. It's brilliant that Orla got her transplant and that it generated so much awareness of CF and organ donation. I've heard it's like a new lease of life for people getting new lungs, though it comes with its own complications.

qo2cj1dsne8y4k

Is there exercises or physio that you have to do to keep your lung function up or is it more down to how you take care of yourself (medication not smoking/drinking)

Did you know anything about the illness before you were diagnosed yourself?

I enjoy reading your posts across boards and notice you always tend to be in good humour, and pretty positive. How do you keep yourself in such good form, considering you have a life changing condition?

(Sorry for all the questions)

Ariadne

MetzgerMeister wrote: »

Thank you Succubus for doing this AMA.

My sister was diagnosed with CF at the age of seven. She is incredibly active although her lung function fluctuates from time to time. I also have a friend who had a double lung transplant a few years ago and is doing very very well.

How does CF affect you physically?

Thank you very much for your question MetzgerMeister



I'm sorry to hear that your sister has CF but I'm delighted that she's very active. Exercise is so important with CF. Unfortunately I'm really bad at that side of things but it really does help to keep you healthy and I'm working on it


Physically, I suppose I find that tough to answer sometimes because I don't even realise when I'm compensating for CF. Like climbing the stairs leads to a coughing fit which can bring on vomiting, so usually I wait until I really have to go upstairs for something or if I'm ill I might ask someone to throw something down to me. Carrying things around or up the stairs can be difficult too, I can do it but it will lead to a coughing fit so usually someone else carries it for me, but I'll do it myself if it's not too heavy or there's no one else around.



Sometimes even the steam from a shower sets me off on a coughing fit and I have to try not to puke in the shower I suppose I get tired more easily too, like I usually have a sit down on my bed after I climb the stairs, or I have a sit down after I hang up the washing or do something physical.



Usually my wife or parents do the vacuuming or more physical housework. Like for example I find changing bedsheets exhausting so I change the pillow cases and my wife does the rest. Or my wife carries the washing upstairs and I hang it up. I do things like put the groceries on a table so I can put them away without bending down as it aggravates my lungs/reflux. I've gotten very good at picking things up with my feet :pac:



I get out of breath when I'm walking so I can't walk for too long or too far. I find it difficult to walk and talk too, usually I can't say too much unless I'm walking very slowly, and I tend to walk more slowly than other people. Basically I can do most things other people can do but more slowly and with more breaks needed.



The other thing that can sometimes cause me a bit of trouble is joint pain, it tends to happen when I have a chest infection, they thinks it because the inflammation in your lungs causes inflammation in your joints too, so my joints get sore and my body aches. I also have chronic sinusitis so tend to get frequent sinus pain and I have little to no sense of smell due to a lot of operations on my nose and chronic infections.



Sorry if that was a bit too long :P

Ariadne

Harry Handsome Bulldog wrote: »

Is there exercises or physio that you have to do to keep your lung function up or is it more down to how you take care of yourself (medication not smoking/drinking)

Did you know anything about the illness before you were diagnosed yourself?

I enjoy reading your posts across boards and notice you always tend to be in good humour, and pretty positive. How do you keep yourself in such good form, considering you have a life changing condition?

(Sorry for all the questions)

Oh feel free to ask away! I'm glad that there's interest in the topic

Well exercise is very important with CF, the physios basically torment us about it all the time. Even when I'm in hospital with a chest infection and on IVs they are torturing me on the treadmill, sadists :P The other thing that is important is physiotherapy, there are different types you can do. I do one that's called autogenic drainage. It needs to be done twice a day for about a half an hour and it's basically a breathing cycle technique that (sorry this will be gross) gathers the mucus from the bottom of your lungs and helps you to cough it up more easily. It's gentler on your lungs than coughing fits because coughing like that leads to inflammation. Some people with CF produce very little mucus and others produce loads, it depends on your health really. I used to produce very little up to the age of 19, now I produce buckets of the stuff and it's like glue :pac: Seriously, if I knock over my sputum pot the phlegm doesn't even move in there, so that shows how sticky it is and difficult to get up. Basically the more mucus you can get up the less that's down there to get infected and cause more lung damage. Sorry if all of that is TMI

Actually a few months before I was diagnosed with CF, I was complaining about being ill again and my mother told me I should consider myself fortunate that I didn't have CF :pac: I didn't know what it was so I asked her and she told me it was this awful disease that leaves you bedbound and physios have to come to your house every day and you die young. Now that was all very out of date information at the time but my mother had a cousin who died of CF at the age of 17 in the 1970s so I assume that was all that she knew of it.

I suppose I've never really thought to myself ''why me'', and I think that's because I know I'm quite lucky for someone with CF. Some people still die in their teens or early twenties and I got away without even knowing I had it until I was fourteen. A lot of people with CF spent most of their childhoods in hospital and I didn't have any of that. I didn't start need IV antibiotics until I was nineteen which is very good going and I have so far avoided some of the more difficult complications with CF. A lot of people with CF are also diabetic, have osteoporosis and liver disease and I haven't developed any of these complications (yet) though I have low bone density, they don't know if or when I will develop those complications. I take a lot of medication/inhalers/nebulisers every day but compared to some other people with CF I don't take as much medication as they do at all. So I suppose that makes me feel grateful and fortunate. I feel like I'm not as healthy as someone without CF but I'm healthier than a lot of people with CF. Sorry if my answers are too long!! It's hard to know how much to say.

Corkgirl18

Hi, thanks so much for doing this.

I've heard that it can be very dangerous for people with CF to be around each other. Is there much truth in that?

Ariadne

Corkgirl18 wrote: »

Hi, thanks so much for doing this.

I've heard that it can be very dangerous for people with CF to be around each other. Is there much truth in that?

Thank you very much for your question

It's definitely risky for us to be around each other and we're brought into a room on our own as soon as we arrive at the clinic and we have to be in a room on our own if we're hospitalised. I might see someone with CF in passing along a corridor but that's about it. The reason for this is because the majority of us have bacteria in our lungs due to the sticky mucus we have, we take daily oral and inhaled/nebulised antibiotics but these just try to control the bacteria levels really, they don't get rid of them.

When I say I have a chest infection what it really is called is an ''exacerbation'' or worsening of symptoms, so I might get joint pain, temperatures, my infection levels in my blood go up and my lung function drops. Then I will need a course of IV antibiotics that will last for two or three weeks and will dampen down the infection again until it flares up a few months later.

So, I have one type of bacteria in my lungs, MRSA, some people have more than one type. Don't worry, I'm not contagious unless you've been severely burned and/or have an open wound, or if you have CF/severe lung problems. Not everyone with CF has MRSA, they may have another bacteria called pseudomonas or they could have something very dangerous called cepacia. So if I were to mix with someone who has CF I could give them MRSA on top of whatever bacteria they have already and vice versa. We could both end up having to battle more bacteria basically.

This wasn't always known so children/adults with CF used to mix but by the time I was diagnosed the regulations had been introduced so I've never had a friend in real life with CF. Some people still risk it because they've been friends for years, and some are siblings who grew up together so they don't have a choice really. Some just don't care and risk it.

One thing I actually find difficult is that I often have uncontrollable coughing fits in public and I find it very embarrassing as people often turn around to look at me or they move away, assuming I have a cold or the flu or something contagious. I find it quite embarrassing as sometimes I'm coughing for a few minutes and I can't control it.

Oh edited to say that my sister is getting married in April and someone else with CF is going so we were trying to decide what to do. We've decided that we'll just sit away from each other and try to avoid each other for the day, which is a bit weird :P

MOLF Hunter

Im sure most of us have read the story of Richard Mason, the head honcho of moneysupermarket.com who found out his 3 kids aren't his only because he was diagnosed with CF.....
Now a couple of things... children? Is that something that you see in your futures?
And ....when you see people like Mason (who is 55)... Does this give you a renewed joie de vivre knowing that you could perhaps have a much happier and longer life than was deemed possible when you were originally diagnosed?

1hnr79jr65

Nothing to ask myself as i know all too well about CF as one of my nephews aged 11 has it. Stupid and horrible affliction to have and glad to see your making it through ok with it. A friend of my sister had little girl aged 13 who died in Oct just gone, they had a pair of lungs for her but couldn't transplant as she had bad chest infection and unfortunately she lost her life.

There are good support networks here for anyone affected, CF Mammys on facebook is a good help for those that need and can contact CFI.ie for support.

All the best in your future.

The Sneeking Squid

Hi succubus,

thank you for the honest Q&A session, i think this is really interesting for most of us who don't know a lot about cystic fibrosis.

I imagine it being quite hard if you can't meet up with friends who are going through the same or something similar. Do you have a dedicated GP or healthcare team, or do you have to see whoever is on rotation?

Also, you seem to know a lot about this condition, but i wonder if it still surprises you sometimes with new developments or things you hadnt heard of before?

Thank you!


Thank you
The Squid

Ariadne

MOLF Hunter wrote: »

Im sure most of us have read the story of Richard Mason, the head honcho of moneysupermarket.com who found out his 3 kids aren't his only because he was diagnosed with CF.....
Now a couple of things... children? Is that something that you see in your futures?
And ....when you see people like Mason (who is 55)... Does this give you a renewed joie de vivre knowing that you could perhaps have a much happier and longer life than was deemed possible when you were originally diagnosed?

Thank you very much for your question Sirius, I mean MOLF

I've heard the story about Richard Mason alright. With regards to children, it's a tough decision really. About 97% of men with CF are infertile but can have children with medical intervention and some women with CF need IVF, you don't really know if you'll need IVF until you start trying. So that's one obstacle.

The next concern would be making sure that the person I have a child with doesn't carry a CF gene, as I have two CF genes it would give my child a high chance of having CF and they'd automatically be a carrier. Though you can do a process called PGD which tests your embryos for CF and then implants the CF free ones via IVF.

Then there's the potential health problems that I'd face, I have 51% lung function and some doctors don't recommend pregnancy if your lung function is 60% or less, and others say 50% or less. Either way I'm in a position where carrying a child could put pressure on my lungs. There are certain medications and antibiotics you can't have when you're pregnant and breastfeeding and some women lose lung function they can't regain. I've heard of some women with CF having a baby and a couple of years later needing a lung transplant or unfortunately passing away.

Now there are quite a few women with CF who've had children in recent years and they're getting on grand and they've bounced back, but you really don't know.

I have my reservations about having children when there's a high chance I wouldn't see them live until adulthood or I'd be very ill and unable to do things with them as they grow up. Perhaps if I desperately wanted a child I'd feel differently. I'm also a woman married to a woman so that presents obstacles too.

Well when I was diagnosed they were telling me that some people were making it to their forties and I was a bit puzzled wondering if that was supposed to be good, until I found out what the life expectancy was previously When I was born my life expectancy had my parents known I had CF was 14, and I'm 30 now and not going anywhere any time soon

No one can tell me how long I could live but I am hopeful I could make it into my 50s or possibly older. New drugs and medications are coming out all the time and I think that the future for children with CF looks positive, hopefully CF will become a manageable disease, like asthma or diabetes.

With regards to diagnoses like Richard Mason, I don't really take much comfort from it. Generally speaking the older you are when you're diagnosed with CF the milder your CF tends to be, I've heard of people in their 70s and 80s being diagnosed with CF in America, I just think that they must have very mild forms of it to have lived so long undiagnosed, especially without the proper treatment.

MOLF Hunter

Thanks for the answer succubus...hugs and soooo much love for you as always

Dj Stiggie

I don't really have a question, but thanks for doing the AMA. One thing a lot of people don't realise about CF is that due to cross-infection, we don't really get to connect with other people with CF. I have a cousin that I haven't seen in about 11 years because of this. So it's always interesting to get another CF'ers perspective on life and treatments.

Ariadne

Sour Lemonz wrote: »

Nothing to ask myself as i know all too well about CF as one of my nephews aged 11 has it. Stupid and horrible affliction to have and glad to see your making it through ok with it. A friend of my sister had little girl aged 13 who died in Oct just gone, they had a pair of lungs for her but couldn't transplant as she had bad chest infection and unfortunately she lost her life.

There are good support networks here for anyone affected, CF Mammys on facebook is a good help for those that need and can contact CFI.ie for support.

All the best in your future.

Thank you for your post Sour Lemonz. I am sorry to hear that your nephew has CF but I hope he's as well as he can be and will be for a long time. I'm so sorry to hear about the friend of your sister who lost her little girl at such a young age, thirteen is far too young to pass away. It's terrible that they even had the lungs for her but she was too ill at the time to have the transplant. It's hearing about teenagers passing away from CF that makes me feel very grateful to still be alive as many others haven't been as lucky.

Ariadne

The Sneeking Squid wrote: »

Hi succubus,

thank you for the honest Q&A session, i think this is really interesting for most of us who don't know a lot about cystic fibrosis.

I imagine it being quite hard if you can't meet up with friends who are going through the same or something similar. Do you have a dedicated GP or healthcare team, or do you have to see whoever is on rotation?

Also, you seem to know a lot about this condition, but i wonder if it still surprises you sometimes with new developments or things you hadnt heard of before?

Thank you!


Thank you
The Squid

Hi there Squid, I'm glad to hear that it's interesting for people who don't know much about CF and thank you for your questions

I suppose I'm used to not having people with CF in my life but it would be nice to be able to go to a support group sometimes as they are the only people who really understand what it's like. There are online groups but I haven't joined any as they are mostly on facebook and I don't really use facebook.

I have a dedicated CF team, I'm living in Cork so I go to CUH and there is a team of three CF nurse specialists, two dieticians, two physiotherapists, several registrars and my CF consultant. The nurses and dieticians are always the same unless someone leaves the job, the physiotherapists tend to rotate every once in a while but some specialise in CF and are there for years. The registrars (junior doctors) rotate every year or so I think. I've had the same CF consultant since I was 19 (aside from when I moved to Dublin and had a different CF team, and I lived in the UK and had a different CF team there too). So really it's the CF nurses I have the most contact with and therefore the best relationship with. I go to the CF clinic every six weeks or if I have an infection, and then I may or may not be admitted for IVs. My GP just prescribes my regular medication, she doesn't want to go near me about anything else really for fear of messing up :pac:

I knew almost nothing at all when I was diagnosed at fourteen and then I buried my head in the sand until I was 19, then I joined an Irish and an English CF forum and I got chatting to people with CF and I found out and discovered so much. I honestly didn't have a clue about CF before that. I still like to keep up as things change all the time and I've discovered lots of things that have surprised me along the way.

Ariadne

Dj Stiggie wrote: »

I don't really have a question, but thanks for doing the AMA. One thing a lot of people don't realise about CF is that due to cross-infection, we don't really get to connect with other people with CF. I have a cousin that I haven't seen in about 11 years because of this. So it's always interesting to get another CF'ers perspective on life and treatments.

Hi there Stiggie, nice to hear from another CFer We can meet up with hazmat suits on :pac: I'm glad that you're happy with the AMA because I know that everyone with CF has different opinions and perspectives on things, so this is just me and my experience. I'm sorry that you're not able to see your cousin, that must be difficult at times. I don't have any family members with CF so I don't have anyone to avoid there. Feel free to join in if you have a different opinion/perspective on a question. I hope you're doing well at the moment

The Sneeking Squid

I'm sorry, I have another question.
It sounds like many people probably don't know/ understand what it's like to have cystic fibrosis (or any similar conditions?), so do you ever feel like you have to explain yourself? For instance if someone stares because you are coughing or so

Thank you
The Squid

Ariadne

The Sneeking Squid wrote: »

I'm sorry, I have another question.
It sounds like many people probably don't know/ understand what it's like to have cystic fibrosis (or any similar conditions?), so do you ever feel like you have to explain yourself? For instance if someone stares because you are coughing or so

Thank you
The Squid

Ask as many questions as you like. I think a lot of people don't know very much about CF so it always helps to raise awareness

Well I can't count how many times someone has offered me a glass of water or told me that they have the same cough/cold that's going around If I don't know them then I just agree politely and accept the water, it's well meaning Sometimes strangers on the street tell me to stay off the fags, actually a few weeks ago a guy in a supermarket told me that my cough should get me a few days off school :pac:

I find it's most awkward with acquaintances or say people I've just met at a party or event. I don't usually want to explain that I have CF so I'll say I have a cough or cold. If I'm sitting down I don't cough as much so I could spend an evening with someone and they may not realise there is something wrong with me. It's more awkward if I'm walking around because I'm out of breath and I feel rude not replying to someone when they're talking to me so I find that a bit awkward. I have scars on my chest from previous operations and I am occasionally asked what caused the scars or if they are love bites, so that involves a bit of explaining too and it can get a bit tedious sometimes. When I'm on a night out I just want to forget I have CF.

Another thing that can be a problem is using my disabled badge. I only use the badge if I'm not feeling well enough to walk very much or if there are no other spaces free. Unfortunately though sometimes people shout at me and tell me I shouldn't be in that space because they see me walking away from the car looking perfectly normal. They don't even check to see I have a badge up, I guess they think I took a relative's one. I lost my temper one time with a woman who was giving out to me when I was going back to the car with two heavy bags of medication from the pharmacy :P

Sometimes it's tough having CF because it's very much a hidden illness, I look perfectly healthy. When I'm standing on public transport and struggling, no one is going to notice and offer me their seat. Then again it also means I can just pretend there is nothing wrong with me and I don't have to disclose that I have CF unless I want to.

cbyrd

It's great to get an insight to this disease, all the questions you'd love to ask but it'd be rude to. My son has pulmonary hypertension and aspirates so he's peg fed, and his jack and Jill nurse works in the CF unit in Crumlin so I have a tiny bit of experience with lung issues. Listening to her stories can be heartbreaking.
One thing that I did come across when he was in icu last year was they're now giving children with CF high dose vitamin D, something I have used for a few years as allergy control (from an allergy specialist in Beaumont) have you come across this or is it new (can't remember if the Prof said treating or trialling)
What's been the hardest treatment you've endured.?

Ariadne

cbyrd wrote: »

It's great to get an insight to this disease, all the questions you'd love to ask but it'd be rude to. My son has pulmonary hypertension and aspirates so he's peg fed, and his jack and Jill nurse works in the CF unit in Crumlin so I have a tiny bit of experience with lung issues. Listening to her stories can be heartbreaking.
One thing that I did come across when he was in icu last year was they're now giving children with CF high dose vitamin D, something I have used for a few years as allergy control (from an allergy specialist in Beaumont) have you come across this or is it new (can't remember if the Prof said treating or trialling)
What's been the hardest treatment you've endured.?

Hey there Cbyrd, I know you definitely have a lot of experience with lung problems yourself as you're caring for your son.

Thank you for your input and your questions

Actually most people with CF, about 85%, are born with a pancreas that doesn't produce the enzymes needed to absorb fat. So they need to take enzymes with their food and even then a lot of people with CF are underweight and need to eat a lot of calories a day. Some peg feed overnight as well. I'm in the 15% that still has a functioning pancreas, though I'm tested every couple of years to see if it is still working, so I have no problems putting on weight and I'm a bit of a heifer, which is pretty damn unusual in the CF world :pac:

I didn't know about taking vitamin D for allergy purposes, that's interesting, I must look it up. Since I was diagnosed sixteen years ago I've been on this tablet called aquadek which has vitamins A,D,E and K in it (all fat soluble vitamins due to pancreas problems), even though my pancreas still works it may not be fully functional so they give me this tablet as my vitamin levels tend to be low sometimes.

I also take calcichew D3forte which has calcium and vitamin D in it. These are maintenance doses, the last time my levels were checked they were low so I'm going on high dose vitamin D for a few weeks. I think they've been doing this for a good while now, at least the last sixteen years anyway. My dietician told me that everyone in Ireland should take vitamin D during the winter anyway as there isn't a sufficient amount of sunlight to have adequate levels and she said they are going to roll it out for children in Ireland at some stage in the future.

Your last question is an interesting one, and I'm having to think about it. I've had two portacaths placed in my chest and one removed over the years, not pleasant but not the worst. I've had a good few operations on my nose and I think the one that was most painful was when I had a deviated septum corrected and I had tissue removed. I had a bronchoscopy once which wasn't that pleasant, they insert a tube into your lungs while you're sedated but awake and they take samples and they also flush out your lungs which feels a bit odd and like you're drowning I suppose nothing stands out as horrifically painful, more like uncomfortable and sore and not pleasant but not screaming in pain sort of stuff.

I think what can be the toughest is that there's no break from CF, and I'm sure you know what this is like yourself, there's no day off from medication or treatment, you can't take a holiday from it. It's a lot of treatment to do every day and I don't always do all of it, I do struggle with that. Another thing which isn't much fun is having lines placed in your arms for IVs, either cannulas or PICC lines, it's fine at first but your veins get worn out after a while and it becomes harder and harder to get blood or get a line in for IVs. Thankfully I have a portacath now so my veins get a break

Mango Joe

Hi Succubus - Thanks for doing the AMA, I must say you come across so well, your attitude is great and I wish you and your wife all the best for the future in every respect.

I was wondering if there's any fundraising initiatives supporting those with CF or research projects you're aware of and could bring our attention to? I'd be happy to set up a standing order etc for some monthly amount I could manage if it was hitting a good target.

Also there was a drug a few years back that promised to really help a certain percentage of people with CF - Are there any signs of significant help on the way in terms of new drugs or treatment options? Are our politicians stepping up to the task of supporting people living with CF?

Thanks again

Ariadne

Mango Joe wrote: »

Hi Succubus - Thanks for doing the AMA, I must say you come across so well, your attitude is great and I wish you and your wife all the best for the future in every respect.

I was wondering if there's any fundraising initiatives supporting those with CF or research projects you're aware of and could bring our attention to? I'd be happy to set up a standing order etc for some monthly amount I could manage if it was hitting a good target.

Also there was a drug a few years back that promised to really help a certain percentage of people with CF - Are there any signs of significant help on the way in terms of new drugs or treatment options? Are our politicians stepping up to the task of supporting people living with CF?

Thanks again

Hi there Mango Joe, I'm glad to be able to do the AMA and that people are interested. Thank you very much for saying that I come across well, I'm conscious of not wanting to sound too negative or self-pitying because I have a lot of good things in my life too. I suppose I feel like everyone has their own issues in their lives, and I'm very lucky to have an extremely supportive wife, and although my parents weren't always supportive, they are very supportive now. I even still have two grandads alive and kicking, one 90 and the other 89, and nobody else has any major health problems in my family so I am grateful for that too Thank you so much for your kind wishes too, everyone has been so lovely and supportive on this thread.

Here is a link to the donation information on the CF Ireland website: https://www.cfireland.ie/support-our-work

There is also information about fundraising events that are coming up or information about how to hold your own event. I'm going to volunteer for the 65 roses day which is the national flag day for CF, it's on the 12th of April. It's called 65 roses because that's how young children with CF learn how to say Cystic Fibrosis Basically you go to shopping centres and other areas and you sell purple roses to raise money for CF services and supports. I don't know of any trials you can directly donate to but I know that the money the CF association raises goes towards helping people with CF and their families and also goes towards improving facilities which has a direct impact on life expectancy.

For example the build4life campaign raised enough money to open a new respiratory ward in CUH in 2015 that has a number of beds ring fenced for CF patients, so we get priority if we need to be admitted. This was so important because people with CF used to have to share rooms with other people who had respiratory conditions or CF and there was a risk of cross infection every time you were admitted for treatment. In fact the life expectancy in Ireland used to be worse than in Northern Ireland (and the worst in the western world) and it was completely down to a lack of isolated facilities in Ireland. So the money really does make a difference. Any donation at all would be greatly appreciated by the association and thank you so much for your offer, it's very kind

The drug you're talking about is called Kalydeco, and it's made a significant difference in the lives of people with CF who have the gene for it. Some people have gained up to 10% more lung function and others have put on badly needed weight, which can help the body fight off infection. Some don't need to take as many enzymes with their food and some don't have as many exacerbations. It's the first drug to actually target the underlying cause of CF and not just the symptoms.

Unfortunately only 4% of people with CF can benefit from this treatment however another drug came out called Orkambi which treats the most severe gene that's so common in Ireland. It hasn't been as successful as Kalydeco but people have also gained lung function, not needed as many IV antibiotics, and gained some weight. I have one severe gene but one other rare gene that's responsible for me still having a working pancreas and being diagnosed later. It means though that I can't have Orkambi because you need to have two of the severe genes in order for it to work. I'm so delighted for the other people with CF who have benefitted so much from these drugs and I'm sure they'll have something for people with my genes at some stage, I always have to be awkward

For a long time the situation with CF facilities in Ireland was appalling and people were dying as a result of it. Politicians did very little and until there was a campaign by people with CF themselves nothing really happened. However this has thankfully all changed, now there is a dedicated CF ward in St Vincent's Hospital in Dublin that has 20 beds and there's the Respiratory ward in Cork with ring fenced beds for CF patients.

I think improvements have also been made in the other CF clinics in the country but these are the two major adult units, along with Beaumont hospital in Dublin, and the two I have personal experience of.

To be fair to the Irish government, there are still CF patients in the UK hoping to have Orkambi approved because the drugs are so expensive that the NHS is refusing to do so except on compassionate grounds (so if someone is at end stage CF already). It's terrible to see people deteriorating when you know that there is a drug available that could help them. The Irish government has approved both drugs so people with CF are in a fortunate position in Ireland now.

Martina1991

Hi Succubus. I think you’re great for doing the AMA. There’s so much the general population don’t know about CF. I don’t know anyone personally that has it, but my undergrad project for college was about CF, particularly the use of the sweat test on adults. The sweat test is usually only performed on kids but I found that adults with elevated sweat test results can actually indicate they have a mild form of CF or be a carrier of Delta F508. Did you have to have a sweat test when you were diagnosed?

With regards fertility, I apologise if this is a personal question. Did you or your parents ever have genetic counselling about passing on the CF gene? Do you think this would be a good idea in an attempt to decrease the incidence of people passing on the CF gene in Ireland?

Ariadne

Martina1991 wrote: »

Hi Succubus. I think you’re great for doing the AMA. There’s so much the general population don’t know about CF. I don’t know anyone personally that has it, but my undergrad project for college was about CF, particularly the use of the sweat test on adults. The sweat test is usually only performed on kids but I found that adults with elevated sweat test results can actually indicate they have a mild form of CF or be a carrier of Delta F508. Did you have to have a sweat test when you were diagnosed?

With regards fertility, I apologise if this is a personal question. Did you or your parents ever have genetic counselling about passing on the CF gene? Do you think this would be a good idea in an attempt to decrease the incidence of people passing on the CF gene in Ireland?

Hi there Martina, thank you very much, it's been a pleasure to do the AMA



Interesting questions there!

I had a CT scan when I was thirteen because the asthma treatments alone weren't working and it showed bronchiectasis (lung damage) at the base of my lungs. 50% of people who have bronchiectasis also have CF so my Respiratory consultant ordered a sweat test for me. My Respiratory consultant also happened to be a CF consultant and he was convinced I had asthma before the CT scan :pac:

So there was a mistake made about a year prior to my diagnosis, I was seeing an ENT (ear, nose and throat consultant) for a few years because it was suspected that my cough was caused by a drip down to my chest as I had a lot of sinus problems. So my ENT arranged for me to have a sweat test, but it never happened and we don't know why. We were told that the test was negative and I was having so many different tests at the time that we didn't think anything of it.

However, when I went to have the sweat test that my Respiratory consultant ordered, myself and my mother realised I'd never had this test before as they put electrodes on your arm and they wrap your arm in clingfilm so it isn't really a test you'd forget! So my sweat test was positive for CF but only just, a negative result is less than 40, an inconclusive result is 40-60 and a positive result is over 60. My result was 62, but a lot of people with CF would be getting results in the hundreds. In fact I was lucky that the sweat test came back positive so I could get the diagnosis and the proper treatment because a lot of people with my rare gene fail the sweat test and continue to go undiagnosed and without proper treatment. I had a blood test afterwards which identified my two genes.

My parents never had genetic counselling because I was born in 1988 and the first CF genes were only discovered in 1989. My parents had no idea that they were carriers. After I was diagnosed my older sister was tested and is a carrier of Delta F508. I definitely think that genetic counselling is necessary to explain to people their chances of conceiving a child with CF.

Unfortunately I don't think it's usually offered unless you have a relation with CF that you know of. Basically how it works is, one in 19 people in Ireland carry one CF gene, so they don't have CF themselves (you need to have 2 CF genes to have CF), but they carry it. If two carriers get together and have a child, each child they have has a 1 in 4 chance of having CF. So I had a 25% chance of having it and so did my sister.

My mother and my father both passed on one CF gene each to me, so I got two CF genes and that's how you end up with CF. One of my parents passed on a CF gene to my sister but the other didn't so she is a carrier but doesn't have the illness. I think there should be more awareness of the high carrier rate in Ireland and the consequences of that.

However, people still go on to have children regardless. My parents told their siblings about their chances of being carriers but some still went on to have children without getting themselves tested first. Some parents who have a child with CF go on to have more children even though they know of the 1 in 4 risk per pregnancy. So I think genetic counselling does help to reduce the number of CF pregnancies but it doesn't always have an impact.

Martina1991

Succubus_ wrote: »

So my sweat test was positive for CF but only just, a negative result is less than 40, an inconclusive result is 40-60 and a positive result is over 60. My result was 62, but a lot of people with CF would be getting results in the hundreds. In fact I was lucky that the sweat test came back positive so I could get the diagnosis and the proper treatment because a lot of people with my rare gene fail the sweat test and continue to go undiagnosed and without proper treatment. I had a blood test afterwards which identified my two genes.
.
.
However, people still go on to have children regardless. My parents told their siblings about their chances of being carriers but some still went on to have children without getting themselves tested first. Some parents who have a child with CF go on to have more children even though they know of the 1 in 4 risk per pregnancy. So I think genetic counselling does help to reduce the number of CF pregnancies but it doesn't always have an impact.

Yes the reference ranges you quoted there (>40, 40-60, <60) were established in young children so they aren't really suitable for teenagers or adults. It also seems that those with more severe CF will have a higher sweat test result so those with milder forms slip through the cracks.

I guess if more people were aware that CF is genetic and how the disease manifests, people might reconsider their family planning options and how it could affect their lives. The same could be said about any genetic condition of course, but with CF being so prevalent in Ireland, the more people that know about CF the better.

Which is why this is a great AMA!

Ariadne

Martina1991 wrote: »

Yes the reference ranges you quoted there (>40, 40-60, <60) were established in young children so they aren't really suitable for teenagers or adults. It also seems that those with more severe CF will have a higher sweat test result so those with milder forms slip through the cracks.

I guess if more people were aware that CF is genetic and how the disease manifests, people might reconsider their family planning options and how it could affect their lives. The same could be said about any genetic condition of course, but with CF being so prevalent in Ireland, the more people that know about CF the better.

Which is why this is a great AMA!

Oh that's interesting, I didn't know that the reference ranges don't apply to teenagers or adults. Do you know what ranges apply to teenagers, out of curiosity? That's exactly it, a lot of people with severe CF will have results in the 100s, whereas someone with a milder form may not have a positive result and gets missed. On older people they are unsure about they often do a blood test to check for CF genes rather than the sweat test for this reason.

I definitely think people should be made aware of the high carrier status in Ireland because it has repercussions. However, having children with CF can be a bit of a controversial topic in the CF community from what I've seen on forums. Some people with CF don't like the idea that with modern techniques to screen for CF embryos, they may not have been born if such techniques were around before they were conceived.

Some acknowledge it's not an easy life but they'd prefer to be alive with CF rather than not exist at all. Some parents of children with CF go on to have more children, risking the chance of them having CF, because they think that they'll love the child just the same and they know how to look after them.

It's a sensitive topic because personally I wouldn't take the gamble, if I became pregnant by someone who wasn't a carrier then the child wouldn't have CF, but if the person was a carrier my child would have a high chance of having CF, I think it's possibly 50% but I might be wrong there.

For me, I prefer a life with CF to none at all but I don't think I could handle the guilt of knowingly bringing a child with CF into the world when there are now ways to prevent that from happening, but that's my own decision and it's not for me to decide what others should do.

I mean I nearly have a heart attack when my cat sneezes more than a couple of times, and he's rushed off to the vet, I think I'd be in a perpetual state of fear if I had a child with an illness and I really admire people who manage to cope with it.

qo2cj1dsne8y4k

I am curious, if both parents were a carrier of the deltaf508 gene, would that be one gene they’d pass on to the child or could the two genes be the same type? I hope that makes sense, what I’m trying to say

Ariadne

Harry Handsome Bulldog wrote: »

I am curious, if both parents were a carrier of the deltaf508 gene, would that be one gene they’d pass on to the child or could the two genes be the same type? I hope that makes sense, what I’m trying to say

Basically each person has these particular two genes, and they are either both CF genes (so you have CF), or one is a CF gene and one isn't so you are a carrier, or both of your genes are CF free.

So in the case of two parents with a Delta F508 gene, if both of them passed on their Delta F508 gene, their child would have two Delta F508 genes, or as it's known, they would be double delta F508. These genes are the most common genes found in Ireland, and I think about 70% of people with CF in the world have at least one of these genes.

CF is supposed to be most severe if you have two of these genes however it can vary, I've heard of people with double delta who are healthier than me, but most would either be as ill as me or more ill.

Even two siblings with the exact same CF genes can have very different experiences. Sometimes one sibling dies quite young and the other doesn't.

There are theories that there are other genes in play that can possibly modify how severe or mild your CF may be. My rare gene is typically considered ''mild'' but I use that word with reluctance because my condition right now is moderate to severe and it's a progressive disease. Really no two people with CF are exactly alike, it's so varied.

Now I hope that makes sense because it can be kind of difficult to explain, if you want further clarification just ask

bolgbui41

Hi Succubus,

Thanks for answering all these questions in so much detail. It's a great insight into CF and how you cope with it.

My first question is kind of picking up on something you said in earlier responses - about how it's tough knowing that you can never have a break from disease/have a holiday from it. I know quite a lot of people with chronic physical illnesses (myself and my gastrointestinal diseases included) also develop mental health problems like anxiety and depression, so I was wondering how/if you deal with the psychological implications of CF. Do you have any sort of counsellor or anything on your medical team? Or have you any opinion on how the HSE should or could be dealing with the mental health side affects of serious illnesses?

Secondly, can you say anything about how your parents deal with the CF? I know I sometimes forget how hard it is for my parents to have a sick child, and have a tendency to lash out at them (and immediately regret it) when I feel particularly bad. Do they have any support themselves? How engaged are they with your medical care?

Ariadne

bolgbui41 wrote: »

Hi Succubus,

Thanks for answering all these questions in so much detail. It's a great insight into CF and how you cope with it.

My first question is kind of picking up on something you said in earlier responses - about how it's tough knowing that you can never have a break from disease/have a holiday from it. I know quite a lot of people with chronic physical illnesses (myself and my gastrointestinal diseases included) also develop mental health problems like anxiety and depression, so I was wondering how/if you deal with the psychological implications of CF. Do you have any sort of counsellor or anything on your medical team? Or have you any opinion on how the HSE should or could be dealing with the mental health side affects of serious illnesses?

Secondly, can you say anything about how your parents deal with the CF? I know I sometimes forget how hard it is for my parents to have a sick child, and have a tendency to lash out at them (and immediately regret it) when I feel particularly bad. Do they have any support themselves? How engaged are they with your medical care?

Hi there Bolgbui,

I'm glad that you don't think it's too much detail! Thanks for your questions too I'm very sorry to hear that you also have a physical illness to deal with, I hope you are as well as you can be, it is tough when it's chronic.

Well, truth be told, I've struggled with mental illness for a very long time and it is partially to do with CF but not entirely. I suppose my physical illness really started to have an impact when I was eleven but my mental health problems began when I was about ten. So, by the time I was diagnosed with CF I was actually already suicidal and in a bad place, I didn't even care about CF really from a life expectancy point of view because I didn't want to live. The diagnosis didn't help of course and I think it tipped me over the edge, I had a breakdown when I was fifteen due to anxiety and depression and it took me a couple of years to recover.

I did recover and my physical health calmed down until I was about nineteen, then CF really started to kick in and cause me more trouble but I was better able to handle it mentally. In the last couple of years my mental health dipped a lot and I was diagnosed with Borderline Personality Disorder a few months ago, which is causing anxiety and depression. The last time I was this depressed I wasn't as physically ill so I could focus more on my mental health recovery but this time I can't just ignore my CF and it is having an impact. My mental health started to dip when I wasn't physically well enough to work anymore but I think that if I could improve my mental health I could possibly work part time, so they are I suppose both contributing to my situation.

Having said that though, like you said, depression and anxiety are common in people with chronic illnesses and 1 in 3 people with CF suffer from anxiety. When I lived in Dublin there was a dedicated CF psychologist who you could talk to when I was in hospital or when I came to the clinic, it's brilliant that such a service is available. They are trying to get the same sort of service in Cork but it hasn't happened yet. However, my CF team have been brilliant and they admitted me for IV antibiotics but also so I could see a psychiatrist right away last September because I was in a bad way. Now I have started a year long weekly course of therapy to help with the BPD, depression and anxiety and had my antidepressants increased.

It was set up so quickly and it's all on the public system, really it has been brilliant. I was living in the UK until September but came home because it felt like I was getting worse and worse mentally and nobody was listening. In the UK I was seeing a CF psychologist but she could only offer me eight sessions of CBT and that was it. Even though my CF team have been so good, there's still a lot of misconceptions about mental illness. Like last week I had an appointment at the CF clinic and I told one of my CF nurses that I think I've turned a corner and things seem to be improving for me mentally, more good days than bad. However, she then asked me why I wasn't doing my physiotherapy, even though I am taking most of my medication and I am trying my best. I had a suicide attempt a month ago which I now regret but that she's aware of, it's the second one in the last few months. I am really struggling to do all of my treatment and instead of her understanding that, she asked me was it laziness or could I just not be bothered to do my physiotherapy. I was trying to explain to her that it's difficult to find the motivation to do your treatment when you don't really care whether you live or die, but she didn't seem to really get it and I just came away feeling worse about myself for being lazy.

As for my parents, well to be honest they didn't handle the diagnosis in the best way, in fact it was treated as if it hadn't happened and we all went on with life as normal. I remember my mother waking me in the middle of the night to angrily give me some cough bottle before I was diagnosed, as I kept them awake for about two years with my coughing at night and I just remember her being annoyed with me whenever she came in. They didn't do any research or try to find out anything about CF and I struggled to do my treatments because of my mental health problems and because I wasn't used to this sudden treatment regime but instead of being supportive they were angry with me for not doing my treatment, as if I was doing it to spite them when really I just couldn't cope.

To be fair to them, I guess they couldn't cope with suddenly finding out I had CF and they desperately wanted to believe that I was mild and I'd be ok so they just continued on. My mother has said some very hurtful things to me over the years without realising it, such as she isn't sure if she would have aborted me if she knew I had CF, and she often talks about CF being a burden on her and my dad, and on my wife. I think that's why I've never thought ''why me'' when it comes to CF, because I'd hear my parents arguing with my sister about who would have to take me to the CF clinic when I was a teenager because nobody wanted to do it. If I got ill it was my fault for not doing enough treatment or not wrapping up well enough. I've always felt like a burden or a hindrance. I'm getting help with feeling like that now though and I'm hoping to change that.

To be fair to my parents, they are much better now, my mother has had therapy herself and we had family therapy when I was a teenager, recently I had to tell her that she has to stop referring to me as a burden or a strain and she's realised what that sounds like and she's really making an effort not to say things like that. I'm living at home with them at the moment and they honestly couldn't do enough to help me now, they are always fussing and helping me as much as they can. I think they feel guilty for their past behaviour and they are really doing their very best now. I am really very grateful to have them.My parents didn't get any support when I was diagnosed and they should have, we all should have, but nothing was offered and they didn't think to ask. I think it's tough for them to see me ill but they try not to show that side to me and my mother always tries to be positive. It can still be difficult for me because if I cough at night now I'm still wondering if I'm waking them and are they annoyed with me.

Before I moved back home last September, I was living in the UK like I said and before that even when I lived in Cork with my partner, my parents hadn't been engaged with my CF care since I moved out of home at the age of 21. My mother used to help me make up my IV antibiotics when I lived at home but it was always a chore for her that she hated doing and when I moved out my partner started to help me and to help me get my prescriptions and get to the hospital and things like that. My partner is so very supportive and never ever makes me feel like an inconvenience

So, when I moved home in September my parents didn't really have a clue what medication I was on or what treatment I was doing. Now though my mother is totally up to speed and she helps me organise my medication for the week and she picks up my prescriptions from the GP and my medication from the pharmacy, or my dad does. They also bring me to my appointments at the CF clinic (I'm struggling with agoraphobia at the moment so it's hard to take public transport and I can't drive). They are now very much involved in my care, I still worry that they resent it and they resent me but I'm trying to trust that they don't feel like that anymore

Martina1991

Succubus_ wrote:

Oh that's interesting, I didn't know that the reference ranges don't apply to teenagers or adults. Do you know what ranges apply to teenagers, out of curiosity? That's exactly it, a lot of people with severe CF will have results in the 100s, whereas someone with a milder form may not have a positive result and gets missed. On older people they are unsure about they often do a blood test to check for CF genes rather than the sweat test for this reason.

Succubus_ wrote:

Some acknowledge it's not an easy life but they'd prefer to be alive with CF rather than not exist at all. Some parents of children with CF go on to have more children, risking the chance of them having CF, because they think that they'll love the child just the same and they know how to look after them.

Succubus_ wrote:

It's a sensitive topic because personally I wouldn't take the gamble, if I became pregnant by someone who wasn't a carrier then the child wouldn't have CF, but if the person was a carrier my child would have a high chance of having CF, I think it's possibly 50% but I might be wrong there.

Its the same reference ranges for everyone. There is no age specific range for the sweat test. My project involved performing the sweat test on adult volunteers and establishing a new reference range. It was just a once off study for my thesis though so it didnt hasnt been applied in the clinical setting.

The ethics invloved with genetic screening is still a sensitive subject for many people and it can border line on eugenics. I don't know if I'd risk a child potentially having such a hard life.
It'd certainly raise some difficult questions for some families.

Hi Succubus I have three questions for you.

Your name is that of a demon which originates from a Jewish book called Zohar. It is some kind of mystical writings. The book mentioned Adam's first wife Lilith who it turns out was a Succubus. Anyways. Are you interested in Demonology?


If I gave you one tub of Ben and Jerrys icecream can you guess which one it would be?


Do you ever get scared ?

Ariadne

Deleted User wrote: »

Hi Succubus I have three questions for you.

Your name is that of a demon which originates from a Jewish book called Zohar. It is some kind of mystical writings. The book mentioned Adam's first wife Lilith who it turns out was a Succubus. Anyways. Are you interested in Demonology?


If I gave you one tub of Ben and Jerrys icecream can you guess which one it would be?


Do you ever get scared ?

Well hello there Perse, thank you for your most amusing questions

I never knew that Adam's first wife was a succubus, it turns out all of us are capable of picking the wrong person. Then again Eve ate the apple so he didn't make the best choice there either :P I can't say I have a particular interest in demonology really, I just thought the idea of a demon disguised as a woman and taking advantage of sleeping men would be a funny name for boards I'm more interested in Greek and Roman mythology and also history as those were the subjects I studied for my undergrad

Now this second question may be the toughest question I've faced so far, and it depends, if you give me the tub, will you be eating most of it or will I? If you're eating most of it then you'll offer me phish food but if I'm eating most of it then it's got to be chocolate fudge brownie as I have a weakness for anything chocolate related! Oh that's another thing that causes my lungs to become more productive, if I eat a load of sugary rubbish my lungs definitely become more annoying. Not that it stops me from doing so from time to time :pac:

Do I ever get scared? Yes, I do sometimes. Not often, but I've read the blogs of people with CF who are at end stage, meaning they need a transplant or they will die within a year or two, and they are hoping for their call as there is a shortage of donors and many people die waiting on the list. The way they describe their every day life, how they are on oxygen 24/7 and mostly bed or couch bound. The lung collapses some of them have, the blood some of them cough up, huge bleeds can kill you. I've had one lung bleed and I was scared, I was lying in bed a few years ago with my wife and I could hear this bubbling sound coming from my chest and we both looked at each other like ''what the hell is that?'' and then I started to cough, I thought it was just phlegm but when I spat it out it was mouthfuls of blood. I have to admit, I think that was the first time I'd ever felt scared with CF.

Luckily I haven't had a bleed like that since and at first I kept worrying I'd have another but that has faded in time, I might have another but sure that mightn't happen for years, sometimes there are streaks of blood in my phlegm and you have to take it easy with physio then so as not to aggravate the lungs further. I know that the end stage won't be pretty and won't be easy, the struggle for every breath even when you're sitting with an oxygen mask on, the chest pain, sometimes people break ribs from coughing. When a lung collapses they need to put a chest drain in and I've heard that is quite a painful procedure.

To be honest though I don't really think about it too often, because if I did then I'd probably go mad. These are all ifs and buts, I've no idea when I will reach that stage so if I spend all of my time thinking about it then I'll have wasted my healthier years worrying and before I know it I'll be at end stage.

I think it is a matter of opinion whether or not you believe Adam chose badly. Both women were the go getters of their time! :pac:

The icecream is a very important one. The person needs to be trusted to buy the favourite tub and also that they wouldn't eat it all themselves. So of course it would be chocolate fudge!

Its ok to be scared sometimes. Your attitude though is spot on. Its hard to focus on the good if we are caught up in the fear of the future. That applies to us all I think.
Here is to many more years of icecream eating and erm Succubying

Tell me how

Succubus_ wrote: »

Hi there Bolgbui,

This answer is one of the best single post answers I have read in any AMA.
It is literally a 1500 word book. Parts of it brought tears to my eye.

I'd like to echo those that have thanked you for doing this and praised your courage.

Is your partner still in the UK? Do ye get to see each other much?

Ariadne

Tell me how wrote: »

This answer is one of the best single post answers I have read in any AMA.
It is literally a 1500 word book. Parts of it brought tears to my eye.

I'd like to echo those that have thanked you for doing this and praised your courage.

Is your partner still in the UK? Do ye get to see each other much?

Thank you very much Tell Me How, I'm really delighted to hear that. I wasn't sure if I was oversharing or indulging in a bit of self-pity. It's really moving to know that you felt that way reading it.

Yes, my partner is still in the UK. We moved over together last January as she was offered an excellent job and I thought maybe all I needed was a fresh start to get me out of the hole I'd crawled into. Unfortunately I was more isolated than ever and went downhill. It was struggle trying to get an NHS number sorted out and join a new CF team, and the hospital I was attending took a bus and two trains to get there if my wife couldn't bring me (it was only a half an hour drive away but awkward to get to on public transport). Of course I felt guilt at being too useless to learn how to drive and being a burden on her. I had to navigate a new health system which had its own way of doing things and let's just say wasn't as relaxed as the Irish system which gives you a bit more leeway :pac: For example, my portacath has to be flushed every six weeks so I tried to make an appointment with the CF clinic in the UK but they couldn't make an appointment with me on the system unless I had an NHS number and I was waiting for it to come through. I ended up having to go back to Ireland to get it flushed at my Cork clinic.

If you go to the GP there it's free which is brilliant for people but you are only given a ten minute slot and only one ''issue'' is dealt with at a time and some of the medication I get here wasn't available on the NHS so I had to get a substitute or go without. My lung function dropped to 46% and when I came back to Ireland and it was partially because they'd been giving me half the dose of one of the IV antibiotics that I take for MRSA. It was based on weight and I was one kilo off needing the double level so they didn't give me what I usually get in Ireland. It was that kind of pedantic stuff. I got the full dose in September when I came back home and my lung function shot back up into the 50s. All in all it was just a lot of upheaval and readjusting and I don't think I was in the right frame of mind for it. I mean there is so much to see and do there and I felt guilty wasting my time sitting around inside. I downloaded meet up but I was too embarrassed to go because I felt like I was totally useless because I wasn't working and people would ask me what I do, and I'd try to diet but end up having a binge as I was home alone all day, and I'd tell myself maybe I'd volunteer or get a part time job once I lost weight and so it became a vicious cycle of trying to do things and not knowing where to start. I took a knitting lesson, I tried to bake, but I was just going through the motions and my physical health was worsening too which made it harder to do things. I didn't want to go out and have people stare at me as I cough, my breathing would get worse and my heart rate would go up, it became a struggle to leave the house at all on my own. Some of it was just bad luck too, like my port flipped over and couldn't be used and it was looking like it would need to be replaced so I was back and forth to the hospital about it (thankfully it weirdly seems to have flipped back ) and my wife was very stressed in her new job. I felt like a burden on her as I had no one else to help me and she had so much going on with work it wasn't fair on her. Most days I didn't go out unless I had a hospital appointment and spent all of my time alone.

A fresh start worked before when I moved to Dublin, those years weren't the best for my health as I was in hospital a lot but they were definitely the best for me mentally. I was in a new place making new friends and in a new relationship, not in Cork where everyone knew me as ''crazy'' Succubus who got drunk and did self-destructive things and just generally seemed a bit unhinged. I moved in with my partner and I went to UCD when I was 22 which is something I never thought I'd manage to do, mentally or physically, I missed a lot of secondary school due to mental and physical problems so I didn't have high expectations for my leaving cert but I actually didn't do too badly.

So, for the time being my partner is still in the UK and our plan is that I will go back to the UK when my therapy finishes until she feels like she has enough experience in her job to be in a good position to get the same kind of job in Ireland. She'd like to have two years of experience before she comes back so I may not need to move back to the UK at all depending on how long the therapy takes. It was always our plan to come back to Ireland eventually. We'd like to move back up to Dublin and maybe buy a little house outside of it, possibly in one of the more affordable commuter areas. That's what I try to hold onto, that one day I'll live in Dublin again where I have some very good friends and that hopefully I'll either work part time or volunteer or do courses to keep me busy, to do something with myself and my life. To contribute, to not be useless or worthless. I don't want my partner to have all of the financial strain on her, it's something I feel guilty about, as well as her having to do so much for me when I'm ill and even helping with prescriptions and things. Well now I'm my parents' burden again :pac:

I miss my partner very much (sorry, I switch from wife/partner because we had a civil partnership so I use either) and she misses me too. In some ways I think it's more difficult for her because she's on her own in the UK and she doesn't know that many people, she has our cat of course who I miss very much too We'll see how things go, if she feels very lonely or if we can't stand being apart she might come back in six months or so. We do try to see each other once a month and we call/text every day.

TL;DR: Yes she's still in England and we see each other once a month

Tell me how

Succubus_ wrote: »

TL;DR: Yes she's still in England and we see each other once a month

Well, I am delighted for you that you have someone who makes you feel loved and I am sure you are not a burden or a hindrance to her.

I think it's great to have a positive attitude and to have an ideal that you are working towards. I hope ye get to that little house and that you become active in some role and get to enjoy life.

I would never tell anyone that they should suck up their problems because others have it worse but I hope some reading this take from your positivity.

Bobtheman

I think with the more mixed population last 20 years the rate of Cf will decline? Correct?

Does climate affect CF symptoms? As in, is the Irish climate worse than say, New York or California?