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)

Is Orkambi available over in the UK?

There is a child in one of my children’s primary school class with CF. What information would it be useful for me to know?

No questions, I have a friend who's DD has CF bit wanted to send and and for you.

It's a bastard of a condition.

No we don't have Orkambi, we keep fighting but Vertex and NHS England can't work it out, my son would benefit from it greatly.

Information that's helpful for you to know is that the child with CF will have a compromised immune system, please don't send your child in if they're unwell, it could make the other child very sick.
Also if they're friends with your child, invite them to play, don't treat them like you're scared of breaking them. My son has been left out so many times because other parents don't want to have to deal our tablets with food or similar.
They might spend a long time off school, encourage your DC to play with them as if they've never been gone and a smile and a kind word to the parents makes a world of difference.

Thanks for the support, it's shit and I genuinely believe it will take my boy.

Thanks for starting this thread. I have friends with a little girl with CF who are deciding whether or not to have more children (due to the risk of them also having CF which I believe is around 25%) What made you decide to go and have more?

DS2 was already born when DS1 was diagnosed so that was a risk I didn't know I was taken. DS3 and DS have a different father and he was tested before conception so we knew they'd only ever be carriers.

I don't personally agree with having more and taking the risk of giving that burden to another child but I would never tell someone they were wrong if they chose to do that and many people do, I know one family with 5 CF children!

How can people support you as parents of a child with CF? An extremely close family member has just had a baby diagnosed with CF and my main priority at the moment is trying to support them. What helps? What doesn’t? I intend to learn all about the medication and associated treatments, so that I can help with childcare. But it would be a great help to know what else you need as a parent with a CF baby / child.
Thank you.

Did your first marriage break up due to the strain of a Cf child or was that not a factor?

How /when was he diagnosed? Is everyone diagnosed by the heel prick nowadays?

Have you got a campaign page or petition or similar we could share?

how do you manage to juggle all the hospital stays and appointments etc with everyday life? (Asking out of sympathy, my son has a much less serious condition but I still find it adds a lot of extra work and time on top of “normal” parenting)

Thanks for this thread OP. Have either you or your son watched anything by Clare wineland on YouTube? I love her channel, she has cystic fibrosis and her outlook on life as well as her condition is incredible

And I am so sorry it must be dreadful to know that there is a drug that could really make a difference. This is definitely worth raising awareness for.

So sorry to hear that your son is in decline OP. I don't know a lot about CF, but read on here recently that it isn't considered a big deal now and that life expe Clancy is very good; are there different types of CF and what are the main issues that come with it? Would a transplant be helpful for your ds?

How can people support you as parents of a child with CF? -

By being understanding, I will suddenly disappear to the hospital, I won't know how long we'll be there and what will happen. Although I always look calm, I am scared and hosptials are lonely, when you're there a lot, people stop visiting.

Someone who took the time to learn the medication and more importantly physio therapy so they could help with DS would be a god send to us.

Understanding that the condition isn't just lungs is also helpful, it's digestive/liver/kidneys/reproductive/heart.

Did your first marriage break up due to the strain of a Cf child or was that not a factor?

Partly, ex couldn't cope with the demands of CF, he would often 'forget' medication and wasn't prepared to do the long Hosptial trips or to keep DS2 at home while I did them. He's better than he used to be and his wife also gets involved, generally he's angry about it and that clouds a lot of other things.

How /when was he diagnosed? Is everyone diagnosed by the heel prick nowadays?

He wasn't diagnosed until he was 2.5yrs, he was always very sick but I was 16 and no one really took me seriously. In the end both his lungs collapsed and finally the doctors agreed to look into it and did a sweat test.

I campaigned for heel prick tests and birth and now they're in they diagnose 99% of cases at births there are a few rare mutations though that don't get picked up and some people are still being diagnosed in adulthood.

Have you got a campaign page or petition or similar we could share?

We have a big following on social media, #OrkambiNow #Orkambi will find everything, there is a main Facebook page for it.

how do you manage to juggle all the hospital stays and appointments etc with everyday life? (Asking out of sympathy, my son has a much less serious condition but I still find it adds a lot of extra work and time on top of “normal” parenting)

It's hard, I work full time but I'm technically self employed and the person I work with knows me as a friend first so she's understanding when I disappear.
This three weeks alone DS has has over 50 unplanned tests so I've been going back and forth from the hospital and picking the others up from school early to take them with me when DH isn't available.
It's draining mainly to be ahead of the ball and make sure everything is accounted for. We don't have much family support practically but we have amazing community nurses that really help.

I'm quite ignorant about CF. What is the life expectancy? Would your DS need a transplant?

I lost a family member I thier teens to CF. I just wanted to say we need to raise awareness.

It’s such a stressful journey for the families. Heartbreaking. Fingers crossed we get the medications needed in time for your little boy to have a much longer life :)

Clare Wineland - No I haven't, I will have a look now, some other ones to watch are Ben Mudge and Charles Michael Duke. I try to get DS to watch and engage but he's not that interested.

So sorry to hear that your son is in decline OP. I don't know a lot about CF, but read on here recently that it isn't considered a big deal now and that life expe Clancy is very good; are there different types of CF and what are the main issues that come with it? Would a transplant be helpful for your ds? -

It's a very big deal. A baby born today has a chance of reaching 40 but people still die in their childhood and teens everyday, either while waiting for transplant or they get to sick for a transplant.
DS is 13 and has significant lug damage, his lung function has dropped by 40% in the last 5 years and he has over 30 treatments a day to keep him alive.

The sticky mucus that's produced fills every organ in the body, it creates inflammation and infection and then scarring and eventually death. DS's lungs are badly effected. He's completely pancreatic insufficient so relies on a gastrostomy (tube feed) to gain weight to put him through puberty, he doesn't digest food at all.
His liver is starting to get damaged from the toxic antibiotics and if he reaches adulthood he will certainly be infertile.

He is in hospital at a minimum of every 3 months for 2 weeks at a time (it's usually 3 weeks though)

Hopefully he will be get a transplant at the point he needs one, there are several bugs that grow in the lungs that exclude you from having one and there is a drastic shortage of organs in the UK.
Once you have transplant though it's a another ticking clock before the lungs are rejected.

There are many mutations of CF, some have been effectively cured by Kalydeko but the most common one that DS has hasn't. Orkambi can address the main underlying issues of CF, it isn't funded.

3 people every week are dying when they're eligible for Orkambi and over 200 people have died since the price negotiations have started.

Thanks Op I will go and find those pages.

I’m so sorry it took so long for you to be taken seriously when he was little.

I've worked in cystic fibrosis clinic and found that life expectancy and the degree to which it affects people varies massively between patients. We have one lady in her 60s but another who's very, very poorly but in his 20s. Siblings etc too.

It's a horrible illness, truly very distressing.

It must've been hard to cope with at such a young age?

I'm quite ignorant about CF. What is the life expectancy? Would your DS need a transplant?

Life expectancy is hard to gauge, for someone born today, they have a 50% chance of living until late 40's but a high percentage pass away in their 20's or earlier. Usually whilst waiting for lung transplant or after transplant due to rejection.

DS will probably need one unless these medication become available, they're said to effectively stop the clock on the decline of the disease.

Just joining in to raise awareness also. It's a horrible disease. I'm so sorry your ds is in decline with it, Titsalina. It sounds like you've been on a very difficult and harrowing journey with having him young then struggling to get him diagnosed. I'm so sorry they wouldn't listen

Do you do his physio or do you have community physios out? How many times a day does he have it?

I'm interested in CF partly as well because I have a sister disease (though mine only affects the lungs, so far less all consuming, but bad enough, I can't imagine what you go through) - so I take drugs developed for CF etc and physio etc.

The IVs can be very strong, can't they. They must leave him exhausted - and you! I know what you mean about people losing interest with repeated hospital visits. It's vital that people keep visiting and supporting.

Does he ever do IVs at home as well as hospital?

What else can we do to raise awareness?

He does get exhausted, he's on a greatly reduced timetable at school and will only take GCSES in core subjects. He can't maintain a full day at school.

It was hard being young, I had one particularly unpleasant GP who called in social services because DS's skin was so sore and stained from 20+ nappies of undigested fats coming through him everyday.

I do IV's at home sometimes but it's hard work alongside working full tome and 3 other children, I am also chair of a local CF charity.
We don't have funding for anything here so no community physio or anything. We're currently trying to raise money for a new physio machine as ours are all wearing out.

The problem is a lot of people just don't know what CF is or how it effects families, people that have heard of it know it as 'the lung one with the patting' (referring to the percussion physio) and a lot of people hear the initials CF and think you're talking about cerebral palsy.

In terms of raining awareness about the new drugs, we've being trying to get people to write to their MP and ask them to get heath secretary to step in and help, Weber already got to debate and to PM question time and Theresa May has previously asked Jeremy Hunt to speed up the process and get it sorted but recently negotiations completely broke down.

Thank you for answering questions. I just wanted to say it all sounds so so very stressful and that you sound like an amazing mum.