Reflections on the Disability Discrimination Act

To mark this week's celebrations of the Disability Discrimination Act, our Policy Manager Nick Medhurst looks at what this has meant for those with cystic fibrosis.

It is 20 years ago this week that the Disability Discrimination Act 1995 was brought into law in the UK and it is being celebrated today as a watershed moment for equality.

It marks a moment in time when we, as a society, recognised our collective responsibility to actively support people who deal with daily challenges from a health condition or disability to achieve their ambitions.

The change in the law that it brought about, now covered by the Equality Act 2010, meant that for the first time people could request, and legally expect, for reasonable adjustments and arrangements to be made for them at work and in wider society to overcome barriers and maximise their potential.

So what has this meant for people with cystic fibrosis?

We recently surveyed 1426 people from our community about cystic fibrosis, their treatments, and what matters to them – perhaps you took part – and we are very humbled by the response we got and excited to share our findings with those who took part in the coming weeks and use those results, in every way we can, to make a positive difference to people’s lives.

One message that we received loud and clear was that managing cystic fibrosis is time-consuming (sometimes all-consuming), disruptive and sometimes impossible to balance with leading the lives that we hope for.

When fighting to keep as well as possible, people with cystic fibrosis will face hundreds and thousands of small challenges every week.

The Equality Act describes a disability as a physical or mental impairment that has a ‘substantial’ and ‘long-term’ negative effect on your ability to do normal daily activities

However, when most people hear the words ‘disability’ or ‘disabled’, I can’t imagine the image of someone with cystic fibrosis immediately pops into their head.

That seems to work both ways and many people with cystic fibrosis don’t always see the fit either. I’ve even heard that some people, likely eligible, choose not to apply for Disability Living Allowance (DLA) because they do not feel ‘disabled’.

The power of the Equality Act, and its predecessor, the DDA, is that it does not label us. It protects us, supports us and empowers us, when we can be at our most vulnerable.

For people with cystic fibrosis, it should be celebrated for the recognition it gives that people who face such challenges in their daily lives can achieve so much more if they live in a society which recognises and acts on unnecessary barriers to fulfilling ambitions.

However, we recognise that people with cystic fibrosis can and do still face discrimination and we would be interested in hearing about any challenges you have faced.

Looking back on Young People's Week

Last week we held our first Young People’s Week on social media, and we want to thank you all for your fantastic contributions. There was some great discussion about the challenges of juggling CF with everything else young people have to deal with, and we received some lovely comments from parents who were encouraged to hear young people talking openly about their condition and how they handle the demands of cystic fibrosis. We particularly want to say a huge thanks to Holly Van Geffen and Charles Michael Duke for their takeover day on Wednesday, which provided an honest and often hilarious insight into life with CF from two very different perspectives (if you haven’t seen Charles’ video on how to tell people you have CF, we strongly recommend you check it out below:

We launched our call for young advisors to help guide our programme of work with young people – over on Hack it Up we’re asking you to tell us what you think the role of an advisor should be and seeking ideas on how we can involve more young people with CF in our work, so if you’re aged 16–25 get on over and have your say.

We also posted about our Bright Ideas Awards where young people with CF who have a business idea can apply for small grants to help them turn their ideas in to reality.

Zainab Nasim’s blog on graduating from uni with a First in Pharmacy was inspiring stuff – as she says, it was all down to “hard work, determination and motivation”. We’ll shortly be asking more young people to tell us about educational experiences and seeking some young ambassadors to inspire other people with CF to achieve their goals.

As you may have seen, we’re currently recruiting for a Youth Empowerment Officer, to join the team at our office and help us create an exciting and innovative programme. We’re looking for a creative and upbeat individual with a genuine desire to support young people with CF to live their lives unlimited.

The week marked the start of our new programme of work with young people, but it’s only the beginning. We want to keep up the conversations to find out what’s really important to you – and how we can empower young people to live their lives unlimited by cystic fibrosis. Next steps will be to set up our youth advisory group to ensure all our work with young people is fully informed by their views, and also to develop our young ambassadors programme, as well as starting to develop some exciting new projects and initiatives led by young people in the new year. We'll be making young people's week a regular feature too so keep an eye out for ways you can get involved as we build on the great feedback we received in Young People's week. So please continue to share your views with us.

Young People's Takeover: Charles Micheal Duke

Hi there!

My Names Charles Michael Duke, I'm a singer/actor from Bournemouth. I’m currently 20 years old, and I have cystic fibrosis. Oh, and I've been waiting for a double lung transplant since April 2015...

I was diagnosed with cystic fibrosis at birth and had a fairly healthy childhood, with around one admission for intravenous (IV) antibiotics a year. However, in 2014 it all started to go a bit downhill. I started to be admitted more regularly, and my lung function was rapidly declining.

Since 2014 I have been admitted several times,, spending around seven out of 12 months in hospital. In June 2014 it was decided that my health had gotten to a point where we needed to consider lung transplantation, so I was refereed for assessments. Unfortunately, my health didn't improve and I went through the assessments and was listed in April 2015, and have been waiting for a double lung transplant since. I’m currently spending two weeks in hospital in the hope that this will keep me 'well enough' for when my call comes, and I will be able to survive the operation and get through the rehab process.

Being a teenager with CF for me wasn’t too difficult; my CF never got in the way, and I was able to fit treatments around socials and going to friends’ houses. They were all aware of my condition and were accommodating of it. However, with my recent decline, had my health been in this state when I was younger it would have made things much harder. I can’t always go as out as I’m not well enough, I don’t do sports so would have missed out on lots of socials that involved things like football down the park. I wouldn’t have been able to stay out or over at a friends as I now require oxygen at night. Although my health isn’t in the best of states now, I would rather it happen in this stage of my life than my childhood/teenage years – I was able to have a childhood, which some people with CF aren’t able to say.

Young People's Takeover: Holly van Geffen

Baby Me

Me now, aged 23

Hello everyone!

So I have been asked to do a social media take-over today with the Cystic Fibrosis Trust to talk about being a young person with CF and the issues we face, and to talk about the help and support we need.

I am Holly and I am 23 years old. I was diagnosed when I was six weeks old and have the most common delta f508 mutation. In February 2015, aged 22, I received a double lung transplant; I had been waiting for two years, since the age of 20.


I think I will begin my story from the age of 12 as I think is the age I became fully aware of my CF and how it affected me. I was lucky as a child and only required intravenous antibiotics (IVs)  twice, aged five and 11! So up until the age of 12, CF was very much in the background of my life. As I moved to high school, I had to take on more responsibility for my own CF treatment. Although I still had a helper who would do my lunch time physiotherapy in the form of postural drainage with percussion, I was now in charge of my own enzymes at lunch time (at primary school my helper would also do that side of things for me). In the lead up to having this responsibility, me and mum discussed what I would have in my packed lunch and the amount of enzymes I would take with each item, so I felt well prepared and never had an issues of taking too many or not enough!

Outside of school, particularly in the evenings, I began to think doing my treatment was a chore and my compliance dropped. It was around this age that the hospital wanted to change my usual physio technique of my parents giving me postal drainage with percussion to using a breathing device. This gave me the control and responsibility of doing my physio which they saw as a good step toward independence. I tried the Pep mask and the Accapella, but I unfortunately didn't feel the same benefit and that I did with postural drainage, so would only do the physio sessions half heartedly! By the age of 14 my compliance was so bad with physio that my mum and dad fundraised and we were able to buy 'The Vest airway clearance system'. It was the best thing we ever did and an attitude changing moment for my young teenage self. The vest allowed me to still have independence in doing my physio, but not so much responsibility in carrying out the correct technique. Instead, I could sit and watch TV or read a book whilst the vest did the work for me. It sounds lazy, and it was, but that is the teenage brain for you! There are a lot of mixed opinions on 'The Vest', but for me it was a saviour in my compliance to treatments and was by far better than doing no physio at all.

At the age of 14 I took up cheerleading in my free time; I trained for seven hours a week over the course of three different nights.  It was great that I found a 'sport' that I loved and was passionate about at an age where often exercise decreases because it isn't cool! I would really recommend cheerleading to CF girls as a hobby that is exercise based but has a great social side to it and sense of achievement. I was a cheerleader for five years and took part in competitions with my team nationwide! It definitely kept me better than I would have been if I'd done no physical activity.

Me in the centre with my leg up! I was a flyer!

My health remained stable until I was 15 and I was diagnosed with atypical Mycobacterium abscessus. Until this diagnosis I had mainly had issues with ABPA (allergic response to Aspergillus fungus), which was treated with high-dose steroids and anti-fungal medicine. My symptoms changed from a wheezy tight chest with the ABPA to a loose crackly chest full of mucus with the mycobacterium.

This change in symptoms and the

With the trophy we won

infections in my lungs prompted a change in my treatments. IV's became a regular occurrence, because of this I had a portacath fitted because my veins couldn't cope with the long lines!

Between the ages of 15 and 19 the regularity of IVs increased from every six months, to every three months, to every month. At the age of 19 I was told I no longer grew the mycobacterium but was kept on a maintenance dose of the oral antibiotics it was sensitive to just in case.

With the mycobacterium gone my lungs became infected for the first time in my life with Pseudomonas aeruginosa. Now the competition with the mycobacterium was gone, the Pseudomonas took full advantage of colonising my lungs! I was unfortunate in culturing an extremely antibiotic-resistant strain of Pseudomonas, so treating it was very difficult. My lung function had dropped to 22% in July 2013 and I was referred for a double lung transplant. I was  reliant on permanent IV's for over two years to be in a stable condition until I got my transplant. Just a weekend off could cause the infection to flare up to the point I was bed ridden. My doctors came up with three antibiotic cocktails to rotate every two weeks; I was lucky my mum was my full-time carer so could do the IVs for me in the comfort of my own home instead of being in hospital permanently. In the two years leading up to my transplant I required oxygen and taught myself to insert a naso-gastric feeding tube for overnight feeding.

Since my transplant my routine has changed massively and I no longer need a lot of the CF treatments anymore. I don't have physio, nebulisers, inhalers, IV's or oral antibiotics anymore. I do however have to take anti-rejection medication and exercise is a key part of keeping my new lungs well and raising my lung function to its full potential.

Now you have a brief history about myself, I would like to welcome you to ask me questions about anything you like, from puberty to socialising, and letting people know about my CF to diet ideas – basically anything CF or transplant related you can think of, and I will try my best to answer from my own experiences.

I will be posting more issues I feel passionate about during the day!

Thanks for reading

Holly
x 

Why a Young People’s Week?

19-25 October is our Young People's Week! But why are we focusing on young people with cystic fibrosis? Jacqueline Ali, Head of Information & Support at the Trust, explains all!

We’re dedicating this week on our social media to all things to do with young people. This marks the start of a new programme of work the Cystic Fibrosis Trust is doing to engage with young people affected by cystic fibrosis and work together to empower and enable young people to live their lives unlimited by their condition.

We haven’t done a lot of work directly targeted at young people, and limitations posed by cross-infection can make it tricky to seek views using traditional methods such as focus groups. But that’s no excuse – there are lots of ways we’re hoping we’ll be able to engage with young people over the coming weeks, months and years – including making full use of digital technology to help us better communicate and make sure our work is fully informed by what you want.

We’ll be recruiting young people to advise us on our work as part of a new advisory group, so that we can make sure that what we’re doing is relevant and meeting people’s needs. Look out for details of this during the week. We’re also seeking young people with CF from all walks of life to be young ambassadors – so if you have a positive story to share about CF, whether it’s overcoming challenges, achieving your ambitions or excelling in education, or you would just like to share your story to encourage and motivate others, then we’d love to hear from you. Again, keep your eyes peeled for more on this during the week.

We’re also excited about this Wednesday when we’ll be handing over control of our social media to two young CF stars you may well be familiar with, so stay tuned…

Ultimately this week is all about finding out about the things that really matter to you and hopefully encouraging some discussion and lively debate. So do check in on our Facebook and Twitter pages this week and find out what’s happening, share with your friends and get involved in the discussions!

Controlling Inflammation: A view from NACFC

On the last day of the North American CF Conference this weekend, Dr Janet Allen, Director of Strategic Innovation at the Trust, attended a session focused on 'Anti-inflammatories in CF: Pathways to Therapeutics’. This session set the scene for a wider debate around the outcomes of the Cystic Fibrosis Foundation’s working group on the topic. In her blog on the session, you can find out more about the human body’s immune response to inflammation and what this means for people with cystic fibrosis.

The question is: how do we control and fine tune our body’s inflammation response to infection? 

Following an infection in the lungs, the body’s defence inflammation system responds to fight the invading bugs. The immune defence system is complex, but the primary cells recruited to start the fight are a type of white blood cell called neutrophils. These cells are not normally found in the lung, but following the detection of infection vast numbers move into the lungs from the blood and start to fight the harmful bacteria. Neutrophils are professionals and usually have all the tools they need to eat/kill the bacteria. 

If they succeed, these cells do their job and then die in a very controlled way. Or, if they sense that they are losing the battle against the infection, they send out signals saying ‘We need help’. More neutrophils and other cells of the immune system are recruited, leading to further inflammation. In people with CF, it is thought that this signalling for help carries on longer than it should (the “off switch” does not function properly) and, as a result, there is more inflammation than is necessary. This additional information is thought to damage the lungs.  

So, we need neutrophils to fight the infection but their very activation can lead to lung damage. There are ways of turning neutrophils ’on’ to do this job, but equally important there are signals that turn the neutrophils ‘off’. Most anti-inflammatory drugs stop the ‘on’ signals, as until recently we have understood less well the ‘off’ signals.

The dilemma is how to control the neutrophils to attack the infection and then stop without sending out ’help’ signals. This is a fine balancing act. In addition, the clinical trials to assess effectiveness will be quite long unless more sensitive measures of lung function are developed, which would enable the use of shorter trials with fewer participants than is possible using current techniques.  

So, the symposium addressed some of these key questions: 

• What are the best targets/drugs to test in CF inflammation?
• How can we be sure we will not stop the neutrophils from fighting the infection?
• What can we learn from previous studies?
• How can we design clinical trials to shorten their duration and still get a clear result?             

The session was well attended by CF clinicians and researchers from around the world and helped stimulate discussion and focus attention on this important challenge ahead of publication of the CFF working group’s guidance. This work will inform future research and therapeutic development globally and help to ensure it is done in a way that is safe and brings maximum benefit to people with CF. 

Dr Janet Allen is a member of the CFF working group which brings together leading experts in CF inflammation from around the world

Praising Arizona - Looking back at NACFC 2015

The past four days saw Ed Owen, Trust Chief Executive, lead our delegation to the North American CF Conference in Phoenix, Arizona. Here he gives us the highlights from the largest global gathering in the fight against cystic fibrosis.

As the 4,000 clinicians, health professionals, scientists, company reps, families and charities like ourselves pack up and head homeward across the globe, it’s worth reflecting what the 29th annual North American CF Conference has told us about the state of play in our international effort to beat cystic fibrosis.

Rebecca Cosgriff, Registry Lead, presenting at NACFC

The collective determination and purpose remains very strong and was evident throughout the four days of meetings, workshops, speeches and discussions. If anything, the sheer excitement and wonder generated by the scientific breakthroughs of just a few years ago has given way to a cool confidence at what can be achieved across a range of fronts.

This is matched, of course, with a realistic view that there is a long way still to travel before our job is done. But distant hope has genuinely shifted to steely belief and this conference showed how we, as an international cystic fibrosis community, are transforming the character and face of this condition.

A good illustration of this is to witness the attendance of the multitude of pharmaceutical and biotech companies here in Phoenix. Barely a decade ago, much of industry would have avoided investing in a ‘rare disease’ like cystic fibrosis. Today, you can’t move for industry talking about their development of new therapies, devices and diagnostic tools targeted at those with the condition.

All this is good news but not without its challenges. For example, Vertex announced this week further news on its pipeline of precision medicines with planned trials of new compounds to develop ‘triple therapies’ aimed at correcting the genetic defect of those with one or two copies of the F508delta mutation.

But, of course, we in the UK are soon to confront the more immediate challenge of ensuring Orkambi – a mere double therapy of ivacaftor and lumacaftor – is made available to those who can benefit.

A key figure in the development of the pipeline of gene-modifying therapies now coming through has been the Cystic Fibrosis Foundation (CFF)’s long-time President, Bob Beall. After 35 years in his post, he has made way for his deputy, Preston Campbell, and this conference was the opportunity for the cystic fibrosis community across the US and worldwide to mark his extraordinary legacy.

It was great to see him, and I was delighted to present himwith a bottle of fine Scottish whisky to demonstrate the thanks of the UK CF communityfor his work. With the Foundation’s therapeutic development programme, Bob has made a massive contribution to the changes we are seeing today.

But, as I said, our work is very far from done, and while we were here we sat down with Preston and his senior team to discuss a range of collaborations with the Foundation, including the prospect of longer-term innovation to develop a fundamental cure for cystic fibrosis using stem cell and gene editing techniques.

And alongside this transformational activity there is the needs of people with cystic fibrosis community today, and it was refreshing to hear Preston commit the CFF to a programme of activities to better engage those with the condition, to hear the diversity of views and experience of the people we are here for.

As I said in my speech to the UK CF conference in Manchester last month, cystic fibrosis for those living with it is not primarily a clinical or scientific issue, it’s a life issue – and we need to understand that in everything we do to help remove the barriers that prevent people with CF living the life they want.

Fighting for a life unlimited by cystic fibrosis, today and tomorrow, is what we are about, and I am pleased to report from the desert landscape of Arizona that this fight is alive, strong and growing.