I'm a Cystic Fibrosis Parent AMA (thinly veiled thread to boost awareness)

[TitsalinaBumSquash]

I'm a mother to a 13 yr old who has CF (doesn't stand for cheeky fucker in my world!)

He spends most his life in hosptial and is declining faster than we expected, I'm trying to raise awareness of CF and campaign for the government to step in and make a deal to fund a drug to save his life.

I have 3 other children (not carriers) one who has ASD and 2 'healthy' littler ones.

(I'm also a community care worker who specialises in dementia, ask about that too if you want)

How is daily life for your ds? Is he able to do things he enjoys? Being a teenager with lots of limitations on what you can do must be hard.

I know a family with a dd with cf but they genuinely don't seem to think it's a big deal. Are there different degrees of severity? Or maybe they just choose to downplay it when talking to people.

Thank you for starting this thread. My DD also has CF, it is a truly crappy disease and we also are praying for Orkambi.

It is such a misunderstood disease and so little knowledge from the general public as evidenced in this thread. Big hugs

Sorry just to clarify.....So both parents have to be carriers? But that doesn’t always mean the child will have CF? Did you know that you were carriers? Is there a test available prior to birth that can determine if the baby has CF/increased likelihood of CF?

[waves]

remembers name change

( it's Doris) well done for this thread and awareness raising, it's so hard on top of the treatment load and I have to be honest I struggle to find the time.

for you and your son x

No because each parent gives a child one chromosomes. And a carrier only has one CF affected chromosome. So if both parents are carriers, there's a 25% of a child having CF, 50% of being a carrier and 25% of being completely clear.

The NHS offer genetic testing and embryo selection for people who are known carriers

Do you ever struggle to get your DS to do physio? We have a lot of resistance and it's so hard to know the best approach.

I will be writing to my MP about Orkambi today

Knitjob - surely they must be downplaying it. Perhaps they just want their dd to be treated as a ‘normal’ child. My dd only has one kidney and it’s crossed my mind never to tell her or anyone of her friends /new people we meet as I don’t want her to always be ‘that’ child. She’s had numerous health issues in the past (heart and stuff) and it’s always a toss up between telling people about it all (to get support) and wanting to not tell anyone so you can venture out into ‘normal’ life and pretend everything is normal when she’s recovered from these things.

knitjob for me atleast, I downplay it to most people other than close friends and family as to be honest its so upsetting to say outloud how serious the condition is/can be. For example someone will say "life expectancy is so much better now/meds better aren't they?" inside I think well yes statistically but I'm frequently seeing young teens/early 20s patients losing their battles and I regularly push the thought of losing her to the back of my mind so I will say "yes things have improved" and largely day to day I just want her to be treated the same and not having that sad look and sympathy from people iykwim!

It's hard for me to downplay it because at 13 DS is frail and tiny, his chest and abdomen are all scars/tubes etc.
He can do a lot of stuff 'normally' to an extent, he can go into town and eat with friends for instance but that will be his energy gone for the day and he'll have to feel comfortable to take his tablets before food (Creon) to digest the meal or face having an embarrassing explosive accident later on.

He is a bugger with physio! He currently has an iSleep but it's old and clunky and doesn't provide high enough pressure to inflate his lungs so we're trying to raise money for the unit to buy clearway machines.

Some children can have CF and no one would ever know, they're well and do everything a normal child would do.

I get frustrated with the outpouring of media (and the CF trust often) that paints a picture of these kids that live their lives normally and make cf seem like a casual chesty cough, for my DS and many, many others it consumes lives and you can see life being taken slowly from these people.

I didn't know we both carried the gene, it's nowhere in the family history so DS was the first, if I had known I wouldn't have naturally conceived 2 children with ex, I would have opted for embryo selection.
It's a simple blood test to test for carrier status and DH was tested before the other 2 children were conceived.

For example here is DS's 'typical' day.

Wake up at 3am to turn the overnight feed pump off and disconnect it from his gastrostomy.

7am wake up for 2 hrs of back to back medication and 45 mons of physio and breakfast.

Train is 9am (an hour later than peers) to school, he goes to the library and attends limited lessons, he has lunch and break early to avoid too much scuffle and the risk of damage to his gastrostomy site)

He's boarderline CF related diabetes so has to do his blood sugars at school.

He get picked up at 2.30 by me.

Home for another round of nebulisers and a short burst of physio.

Dinner.

Then more 45 minuet physio, nebs and pills and then a set up his overnight feed and he's exhausted by 8pm and take himself to bed.

He doesn't have time or energy to be a normal 13year old.
I have to pressure of all the medications even when he's in hospital because staff can't handle that big a load for one patient.

When he's in hospital it's that routine plus 4x IV antibiotics, the physio is done by the physio team and depending on where we are he goes to hospital school (our local unit doesn't have school, specialist unit does) he will also see a consultant, dietician and CF nurse and he has bloods most days.

I get frustrated with the outpouring of media (and the CF trust often) that paints a picture of these kids that live their lives normally and make cf seem like a casual chesty cough

That's how my friend makes it seem. Her child's lungs are mildly affected but not heart or anything else. ? I don't really understand. It must be frustrating for you when people don't understand the reality of life for you and your son. It sounds very hard on the whole family.

Do you think there is any chance of him being prescribed the medicine he needs in the near future?

Are you going away anywhere in the summer holidays or do you need too much medical equipment to travel far? Does he need to stay near his regular consultant or do you usually find good quality care wherever you go?

Thank you very much for answering me and for your candid responses on here. We are very early on in the journey and taking it a day at a time. At 7 months he is doing fairly well so far, but we are all aware of potential mountains to climb in the future.
I hope you can answer some more questions, is too good an opportunity for me to ask things that I wouldn’t want to upset my family member with, she had enough on her plate.
Have you or your son ever taken any counselling offered / know anyone who has? If so, was it helpful?
Aside from writing to our MP, what else can we do to help? Which charities do you feel are best to support in terms of having a chance to make a tangible difference?
What tips do you have to try and keep family life as happy as possible? I know my family member has a worry that all times of happiness will have a shadow of the uncertain future.
I’m determined to help as much as I can. We’ve only had him for 7 months, but life without him is unimaginable.
Sending you and your family lots of love. Thank you for the thread.

Sorry for typos I’m on my phone.

He will will only get prescribed it if the NHS and Vertex can strike a deal to fund it, they do offer it on compassionate grounds but it's a last chance saloon type of thing and by that point it's usually to late to have the benefit.

We have been away once but that was a Starlight Wish holiday to Florida and it took a year of planning and a lot of stress to get everything signed off and his medication on the plane safely, it was fantastic and we'd love to go away again but in the last 6 months he's spent 4 of them in Hosptial.

If we go far I always plan ahead and find the nearest CF centre and I carry a letter from his consultant to pass on if it's needed. We pack heavy though at all times.

Thank you very much for answering me and for your candid responses on here. We are very early on in the journey and taking it a day at a time. At 7 months he is doing fairly well so far, but we are all aware of potential mountains to climb in the future.
I hope you can answer some more questions, is too good an opportunity for me to ask things that I wouldn’t want to upset my family member with, she had enough on her plate.

Have you or your son ever taken any counselling offered / know anyone who has? If so, was it helpful?

We have a team psychologist who is wonderful, I also had some counselling through a local children's charity that our community nurses come from and it helped me massively, DS hasn't wanted any, he treats the psychologist like a mate rather than someone to talk to.

Aside from writing to our MP, what else can we do to help? Which charities do you feel are best to support in terms of having a chance to make a tangible difference?

If you're travelling distance to London, there are protests and marches happening regularly to keep CF in the public eye and to remind ministers that were not going away until these drugs are funded, you could attend those if you're available. The Facebook and twitter pages have all the details.
The cftrust is the main charity that does a lot of research but whichever hospital your relative attends will have their own charity to make life easier for the patients. Apart for that raise awareness, learn about cf and tell people about it. It's woefully short on awareness.

What tips do you have to try and keep family life as happy as possible? I know my family member has a worry that all times of happiness will have a shadow of the uncertain future.

We try and make sure we do normal things, dinners and parties and things, we have brought the whole family to the hospital with a tree and gifts at Christmas and we try to enjoy day to day life as much as we can. At 7 months the future is a lot brighter for your little one, I have always been determine that if my DS's life is shorter then it can still be full and happy, each day at a time and try and maximise opportunities, we don't often say no to activities and events and we try to bring the fun to him when he's unable to go to it. The uncertain future is terrifying for me too but I put on a brave face and use my worry/anger and frustration to channel into raising awareness.

I’m determined to help as much as I can. We’ve only had him for 7 months, but life without him is unimaginable.
Sending you and your family lots of love. Thank you for the thread.

Thanks, I hope your little person is keeping well for many years to come.

Thank you, your advice is very much appreciated x

Thanks for answering my questions too, Titsalina. Do you have any links to helpful letters to move/information about the drugs needed? I want to write to mine.

I also get quite disheartened by the way the media portray it as a mild disturbance. It's the same with my condition (cystic bronchiectasis) - they play up people who seem to be able to live a normal life, run marathons etc as it they're the paragons and how we should all do it, then when we fail we feel like we're rubbish and people just think we're not trying.

But for many people with the condition it's a long, daily slog as you describe, and so incredibly exhausting. My physio is only 45 mins day and night so not at all as much but even that is so very shattering along with all the meds etc.

I really can see why the way the media talk about it is upsetting and unhelpful

Sorry, that should say letters to MP, not move!

With him being in hospital so often, does he have everything he might need to keep him occupied? iPad, kindle, Nintendo DS etc?

Yes he is well equipped for hospital stays, thank you.

The best thing to do if you're on Facebook or Twitter is to search for #Orkambi there is loads and loads of pages and groups dedicated to fighting for it.
We're one of the only countries that doesn't have it available.