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Stark Facts about Pulmonary Fibrosis

  • Without treatment, 50% of Pulmonary Fibrosis [PF] patients will die within 3 years of diagnosis. Treatment extends life expectancy to 7-8 years.
  • Men are nearly twice as likely as women to suffer from PF.
  • There are 8000 new cases of PF every year in the UK.
  • 33,000 people are living with PF in the UK.
  • More than 30,000 people will be diagnosed with PF in the 27 EU countries each year.
  • PF is more common than all leukaemias combined.
  • 3-20% of people with PF have another family member with PF.
  • Most patients can be diagnosed from 1 to 12 years after their first symptoms.
  • Pulmonary fibrosis costs the NHS £88 million per year.
  • 5 million people worldwide have PF.
  • 1 in 3 PF patients are expected to die within the next 12 months.
  • There are 5,000 PF deaths per year.
  • 9,000 admissions and 86,000 hospital bed days accounted for by IPF patients per year.

HAVE A LOOK AT OUR PULMONARY FIBROSIS WISH LIST

PLEASE HELP US CHANGE THESE STATISTICS

DONATE NOW!!! 

 

Breathing Matters Joins the 2.6 Challenge to Save UK Charities

Breathing Matters calls on supporters to join The 2.6 Challenge on Sunday 26 April 2020

Breathing Matters, like so many charities across the UK, has been hugely impacted by the cancellation of thousands of fundraising events due to COVID-19.

In response, the organisers of the biggest mass participation sports events across the country have come together to create a new campaign to raise vital funds to help our fundraising and save the UK’s charities.

The campaign, The 2.6 Challenge, will launch on Sunday 26 April 2020 – what should have been the date of the 40th London Marathon, the world’s biggest one day annual fundraising event, which raised £66.4 million for charities in 2019.  The challenge will last for a week. The 2.6 Challenge is open to anyone of any age – the only requirement is that the activity must follow the Government guidelines on exercise and social distancing and remember to stay local.

From Sunday 26 April 2020, we are asking you to dream up an activity based around the numbers 2.6 or 26 and fundraise by donating on the Breathing Matters’ 2.6 Challenge donation page or by creating your own 2.6 Challenge fundraising page from the Breathing Matters Justgiving home page.

The 2.6 Challenge can be anything that works for you,” says Nick Rusling, Co-Chair of the Mass Participation Sports Organisers group (MSO) and CEO of Human Race. “You can run or walk 2.6 miles, 2.6km or for 26 minutes. You could do the same in your home or garden, go up and down the stairs 26 times, juggle for 2.6 minutes, do a 26 minute exercise class or get 26 people on a video call and do a 26 minute workout – anything you like. We want people to get active, have fun and raise money to help Save the UK’s Charities by giving money or raising funds for the charity close to your heart.”

“For many of the UK’s charities, the day of the London Marathon is the biggest fundraising day of the year,” said Hugh Brasher, Co-Chair of MSO and Event Director of London Marathon Events. “One of the founding pillars of the London Marathon was ‘to show how the family of mankind can be united’. We hope that The 2.6 Challenge, which starts on the day our 40th Race should have taken place, will embody that spirit and inspire people, families and communities to fundraise for their chosen charity to help Save the UK’s Charities. Right now, our vulnerable members of society need the help of charities more than ever before..”

Donna Basire, Fundraising Manager from Breathing Matters said “This event could really be a lifesaver to so many as money raised for Breathing Matters funds research into respiratory conditions such as pulmonary fibrosis, COVID-19, bronchiectasis and pneumonia.  You can do this at your own pace and in your own house or garden – so the challenge is open to everyone. All we ask is that you donate £26 to the Breathing Matters 2.6 Challenge page, or set up your own page and have your own target to raise – could you raise £260?”.

The mass-participation events organisers behind The 2.6 Challenge are: Human Race, parkrun, The Great Run Company, Run 4 Wales, Grounded Events, London Landmarks, Virgin Sport, Limelight Sports, Threshold Sports, Running High and London Marathon Events. The design and website has been created by Studio Republic and the social media campaign by LiveWire Sport. Both agencies are working pro bono.  The campaign is supported by the Charities Aid Foundation, the Institute of Fundraising, the Small Charities Coalition, the Office for Civil Society, Sport England, Let’s Do This, Virgin Money Giving and JustGiving.

For more information on The 2.6 Challenge, visit twopointsixchallenge.co.uk

Virtual Fundraising Challenges

For you lovely people out there who are itching to fundraise, but have not been able to during the COVID-19 pandemic – this is especially for you!

Our event partners, Run for Charity, have just launched a virtual challenge series for young and old, for the fit and not that fit.  It’s a great way of making the most of your time in isolation, having a purpose and keeping/getting fit.  It  is a unique chance for those non-marathon runners to get involved and run/walk a marathon or climb a mountain at your own pace.  And you can win a medal at the end (we all love a good medal!).

Here is a list of our current virtual challenges:

Climb Everest – A 12 week challenge perfect for isolation. Challengers will have to complete a climb equivalent to Mount Everest (8,848m) either out and about on your daily exercise, climbing the stairs indoors, or a combination of the two!

Inca Trail – A virtual challenge to tick off the bucket list. A 25 mile run, walk or jog to be completed in as many efforts as you can manage.

Team Gotham – Not all Superheros wear capes….some have to fight crime in isolation too! This is a 5K challenge, so perfect for families who want to complete it together, or anyone looking to stay fit during lockdown.

The Big Charity Run – The ultimate challenge; you can choose your own.  Pick a 30, 60, 90 or 120 mile distance to complete over a single month. You can run, walk or cycle, so perfect for setting an ambitious target and staying motivated!

Plus there are lots of location specific races that you could join, including:

  • Richmond Park – Half Marathon
  • Tatton Park – 10K
  • Victoria Park – 5K, 10K & Half Marathon
  • Brixton – 5K & 10K
  • Wimbledon Common – Half Marathon

All you need to do is choose your challenge, register and start.

  1. Choose and register here. Receive confirmation email to log in to our Secure Charity Portal.  Payment covers the cost of the place and medal only.
  2. Breathing Matters will get notification that you have signed up for your challenge and will contact you  Or you can contact us directly at breathingmatters@ucl.ac.uk
  3. Set up a Breathing Matters personal fundraising page.  It is up to you how much you want to raise for Breathing Matters; there is no minimum sponsorship limit.
  4. All you need to do is gather evidence along the way of your challenge (eg. photo of your fitness tracker, Strava) and, once completed, simply email it to virtual@runforcharity.com

Your medal will be sent out by our partner in the post.

Run for Charity have partnered with one of the top virtual events companies in the UK, ‘My Race’, who have been producing and fulfilling Virtual Events for many years, so you are in safe hands.

All funds raised will go towards much needed research into pulmonary fibrosis,  COVID-19, bronchiectasis and lung infection.

So you can keep safe, stay alert, social distance, and do something worthwhile at the same time!

What are you waiting for? …

 

ILD Roundup: What you have helped us achieve in 2019

Breathing Matters was established 8 years ago with the aim of finding better treatments for interstitial lung diseases (ILD) and lung infections. Since that time, we have raised money and awareness into these often neglected conditions. Looking back over the 8 years, we have come much further than any of us would have anticipated in the beginning. We have established new theories on the development of ILD or lung fibrosis and the role of the immune system in particular the clotting cascade and neutrophils. We also have better ways of monitoring and diagnosing these conditions and our novel nuclear medicine imaging programme and relatively non-invasive lung biopsy service are the first in the UK. We could not have achieved any of this without the support of our funders and our patients, so thank you all. This review highlights our achievements to date and our future directions in ILD.

Relatively Non-Invasive Lung Cryobiopsy (2014-ongoing):

Objective: To find a less invasive and better diagnostic tool for every patient with ILD

Main benefactors:     Teresa Timberlake and family – equipment purchase + Lawrence Matz Memorial Fund – Clinical Fellow                

Breathing Matters investment: £52,000 salary; £36,000 (total £88,000)

Leveraged funding: £347,000

Outcomes:

1.      Novel cryobiopsy service, first in the UK including training other centres; presentations at European Respiratory Society (2015), British Thoracic Society (2014-6); publications: review 2016; papers in preparation:

2.      Completed Lung-INHALE study Study (2019) to assess inhaled drug deposition using CLB.  This will allow drug companies to develop inhaled therapies for IPF and be sure that they are reaching the part of the lung where they are needed. The use of inhaled therapy will avoid some of the side-effects of anti-fibrotic drugs that are taken as tablets.

This project was developed in discussion with a family whose mother had had a surgical lung biopsy towards the end of her life. Her experience was such that her family felt that a less invasive alternative must be available. Dr Theresia Mikolasch, the Lawrence Matz Clinical Fellow, took this on for Breathing Matters to find out about and train in new techniques. Dr Mikolasch then returned to UCLH and established the first and only UK cryoscopic lung biopsy (CLB) service. CLB is a new way of obtaining larger lung biopsies using a flexible bronchoscope passed into the lungs through the mouth. The patient is sedated and surgery is avoided. This is not only better for the patient than a surgical lung biopsy, but also provides a solution to the lack of biopsy samples available for scientific research.  GSK were so excited by the technique that they awarded Dr Mikolasch and Dr Porter a grant of over £300,000 to carry on the service for an additional 3 years.

Novel FDG-PET Imaging to Predict Prognosis and Response to Treatment in ILD (2014-ongoing):

Objective: To find a new test (biomarker) that will enable us to predict prognosis and response to treatment in each individual patient.

Breathing Matters investment: £34,766

Leveraged funding: £173,850

Funding from BLF for clinical trial of FDG-PET in post transplant bronchiolitis £40,000

Outcomes: Novel FDG-PET imaging programme in ILD – first in the UK; presentations at American Nuclear Medicine Society (2015), British Thoracic Society (2015-6); American Thoracic Society (2017)

Publications:

Pulmonary 18F-FDG uptake helps refine current risk stratification in idiopathic pulmonary fibrosis (IPF). Win T, Screaton NJ, Porter JC, Ganeshan B, Maher TM, Fraioli F, Endozo R, Shortman RI, Hurrell L, Holman BF, Thielemans K, Rashidnasab A, Hutton BF, Lukey PT, Flynn A, Ell PJ, Groves AM.  Eur J Nucl Med Mol Imaging. 2018 May;45(5):806-815. doi: 10.1007/s00259-017-3917-8. Epub 2018 Jan 16.

Synergistic application of pulmonary 18F-FDG PET/HRCT and computer-based CT analysis with conventional severity measures to refine current risk stratification in idiopathic pulmonary fibrosis (IPF).

Fraioli F, Lyasheva M, Porter JC, Bomanji J, Shortman RI, Endozo R, Wan S, Bertoletti L, Machado M, Ganeshan B, Win T, GroveEur J Nucl Med Mol Imaging. 2019 Sep;46(10):2023-2031s AM.

Next steps:

1.      FDG-PET will be used as a response biomarker to see if we can detect which patients benefit from anti-fibrotic therapy and which patients do not benefit. We are applying to the NIHR for a £400,000 grant to carry out this study:

2.      We and others have shown that patients with IPF are more prone to blood clots. We have some very exciting work looking at anticoagulation in IPF.  We have completed 2/3rds of the study and will then publish our findings later in 2020 (see below).

Interstitial lung disease (ILD) consists of a heterogeneous group of diseases with varying amounts of interstitial inflammation and fibrosis. Survival in the most severe form of lung fibrosis, idiopathic pulmonary fibrosis or IPF, is particularly poor; however, there is heterogeneity in outcome. Some patients gradually deteriorate; some undergo stepwise progression, whilst others decline rapidly. Moreover, much of the prognostic data heralds from an era when the criteria for diagnosing IPF were less well and differently defined than at present.  There is a definite need to find prognostic biomarkers to predict outcome in IPF patients

Positron emission tomography (PET) offers the ability to non-invasively investigate cellular metabolism in vivo. PET studies in animals have yielded valuable insights into the biology of IPF and ILD and there is potentially encouraging evidence that PET may aid the development of therapeutic interventions to treat these debilitating conditions. It has been recently demonstrated that 18F-Fluorodeoxyglucose (18F-FDG) PET signal is consistently raised and can be objectively measured in patients with IPF. Moreover, these PET signals are shown to be stable and reproducible.

We have shown over several years and imaging hundreds of patients with ILD that the baseline measures of pulmonary 18F-FDG PET signal to predict survival in patients with IPF compared to other more established prognostic data.  We have also shown that combing PET data with our clinical scoring system based on gender, age and physiology (GAP) data (“PET modified GAP score”) refined the ability to predict mortality.

Future studies are to investigate the role of FDG-PET scanning in other ILDs, such as Rheumatoid arthritis (see below) and systemic sclerosis.

Rheumatoid Arthritis (RA) Associated ILD (2018-ongoing):

Objective: To discover why 1:5 patients with RA will develop lung fibrosis and what novel treatment can prevent disease progression.

Breathing Matters investment: £34,766

Leveraged funding:  £102,766

Outcomes: Novel biomarker test for neutrophils extracellular traps (NETS) in ILD in discussion with UCL business for further development; presentations at American College of Rheumatology (2014-6); British Thoracic Society (2016); British Rheumatology Society (2014-6);

Publications:

The lung in a cohort of rheumatoid arthritis patients-an overview of different types of involvement and treatment. Duarte AC, Porter JC, Leandro MJ. Rheumatology (Oxford). 2019 Nov 1;58(11):2031-2038. doi: 10.1093/rheumatology/kez177.

Autoimmune rheumatic disease IgG has differential effects upon neutrophil integrin activation that is modulated by the endothelium. Khawaja AA, Pericleous C, Ripoll VM, Porter JC, Giles IP. Sci Rep. 2019 Feb 4;9(1):1283. doi: 10.1038/s41598-018-37852-5.

Next steps: To work with a group in Cold Spring Harbour, USA to see if inhibiting NET formation prevents fibrosis.  To see if the presence of NETs in the blood can predict whether patients will develop lung fibrosis.

RA is a chronic debilitating disease estimated to afflict 13% of the world population. Around 10% of patients with RA will develop an ILD that is very similar to the lung fibrosis that we see with IPF. Dr Akif Khawaja was funded by Rosetrees and UCL to carry out a PhD into the aetiology of RA-ILD. His work proposed that RA is a disease that starts in the lung. That chronic lung damage caused by smoking, infection and other insults causes the immune response to recognize the lungs and joints as “foreign” and attack them causing chronic damage. His work implicated neutrophils in this process and, in particular, the p38 MAPkinase pathway.  We are hoping to develop a new test using blood or sputum to detect early activation of neutrophils in the lungs of patients at risk of ILD.  This same test may act as a biomarker for prognosis and to detect early response to novel therapies.  

A Trial of Anticoagulation in IPF (2016-2020):

Objective: To assess the potential of anticoagulation as a treatment for IPF

Main benefactors: The Hulme Family – The Mark Hulme Clinical Fellow

Breathing Matters investment: £40,000

Leveraged funding:  £100,000 from UCL/H NIHR BRC

Next steps: A trial of anticoagulation with heparin in IPF using FDG-PET as a response biomarker

At present, we do not know the exact cause of idiopathic pulmonary fibrosis (IPF), although research has identified lots of processes that are likely to be involved. Currently, we believe that microscopic injury occurs in patients with IPF and then the body responds to repair this, but does so in a way that leads to more damage and scarring. One of the processes involved in repair pathway is coagulation, which minimises blood loss when tissues are damaged.  Patients with IPF are at increased risk of blood clots and this can reduce their already low life expectancy. We also think that these blood clots drive the worsening of their lung disease. Researchers have shown that clotting is over-activated in the lungs of IPF patients and we want to investigate how reducing this might improve the disease.  Based on work carried out at UCL, we believe that anticoagulation with heparin is safe and may even prevent disease progression in IPF. Patients will be asked if they would be willing to take the oral anticoagulant dabigitran for 3 weeks, to reduce clotting. We will perform blood tests and FDG-PET scans before and after taking the drug to judge response.  If we find that the heparin is safe and the patients report some improvement that we can confirm with questionnaires lung function and FDG-PET scans, then we will progress to leverage funding for a much bigger trial.  We have completed 2/3rds of this study and have analysed the results. We have found a small effect and the suggestion is that we look in a few more patients that we will recruit early in the New Year.

A Trial of a Novel Treatment (Compound X) in IPF (2019-2022):

Objective: To assess the potential of Compound X as a treatment for IPF

Main benefactors: NIHR BRC £100,000

Breathing Matters investment: £40,000

Leveraged funding:  Application to British Thoracic Society, Wellcome Trust and NIHE.

Next steps: A trial of Compound X in patients with IPF

Assessing effectiveness of treatments for IPF is difficult as often they do not make patients feel better, despite decelerating disease. Currently, we are guided by regular breathing tests and special imaging of the lungs, which are insensitive to changes and may be unpleasant for patients. We need better tests like a simple blood test to predict the prognosis for individual patients, and their responses to treatment. Causes of IPF are unknown, but we have found that specific white blood cells, called neutrophils, are increased in the lungs of patients with IPF. We also found that the more neutrophils in the lungs, the faster the decline from IPF. This suggests that neutrophils are actively worsening IPF. Neutrophils produce a substance called X that we detect in the bloodstream of patients with IPF. No-one has investigated whether X causes or worsens IPF. We plan to quantify X in the blood and lungs of patients with IPF. By comparing X levels in patients with IPF against healthy individuals, this will establish whether X is increased in patients, whether high levels of X indicate more severe IPF and whether treatment for IPF reduces X levels in patients that respond. These results will ultimately help design future clinical trials testing Compound X that is able to block X as a treatment for IPF.

Understanding Mucin 5 B and Its Role in IPF (2019-2022):

Objective: To assess the role of Muc5B in IPF

Main benefactors: NIHR £300,000

Breathing Matters investment: £40,000

Next steps: Further investigations in patients with IPF of the effects of blocking neutrophil activation

Publications: A review on mucins in lung disease has been submitted and we hope this will be published in 2020.

It is unclear what causes IPF, but it is thought to be a response to damage to the lining of the airways (epithelium) following an unidentified injury. This results in the

formation of excessive scar tissue which disrupts the delicate architecture of the lung and ultimately death follows from respiratory failure.  We have shown from research previously sponsored by The Rosetrees Trust that a certain type of white blood cell which is specialised in fighting infections called neutrophils may play a role in PF. We have found that neutrophils are increased in the blood and lungs of patients with PF and the more neutrophils you have, the worse the individual’s outcome.  In addition, it is recognised that you are more likely to develop IPF if you have a commonly occurring genetic mutation that causes increased mucus production by the lung epithelium, and in particular a protein called Mucin or MUC5B that gives sputum its stringy quality. We propose that the overproduction of MUC5B may stress the epithelium, making it more prone to damage and scarring. In addition, the increased MUC5B will attract and activate neutrophils from the blood and these white blood cells can cause further damage. We hope that, by identifying treatments that limit the number of neutrophils moving into the lung, we can protect patients from developing PF or from PF progressing. We will use neutrophils and epithelial samples form patients and healthy volunteers to compare differences and see how the MUC5B affects neutrophil activation in the lung. Lastly, we plan to block neutrophil activation and recruitment with a specific treatment that is already being developed for other indications and has an excellent safety profile. If our results are encouraging, we can take this medication into an early clinical trial for patients with IPF.

We have also shown that we can detect very early changes in the CT scans of patients that make too much Muc5B and this might be a very early sign, even before the scan looks abnormal, that these patients are at risk of lung disease.

If you are a UCLH patient and want to get involved in any of the above studies, please discuss this with your consultant.

Black Friday Sale – Stunning New Fundraising Events

Hot off the Press!!

Global Adventure Challenges, who we work with, have released their 2021 events and they have SIX new challenges for you – plus they have BLACK FRIDAY offers next week!

 

Below are the new challenges which are amazing and diverse – why not book up before they’re snapped up:

Global Adventure Challenges are giving supporters a whole week to save 25% on their registration fee for selected iconic adventures, including their newly launched 2021 events!

Make sure you keep an eye on their website and Facebook pages for more offers throughout Black Friday week!

Have fun, take the challenge and raise vital funds to support our research – thank you.

 

 

 

 

 

Management and Treatment of IPF – Update

Idiopathic Pulmonary Fibrosis, or IPF, is a growing problem worldwide with increasing numbers of people being affected. There is no cure and treatment options are limited to expensive anti-fibrotic drugs that can slow down the progression of the disease, but not reverse it or stop it completely. These medications have multiple side effects, which can further impact on patients’ quality of life, and only patients with moderate lung function impairment have approved funding to receive them.

The management of patients with IPF is multifaceted and consists of patient education and support, regular outpatient surveillance, symptom relief, pulmonary rehabilitation, annual vaccinations to prevent respiratory infection, supplemental oxygen, managing of comorbidities and ultimately palliative care or, in a minority of patients, referral for lung transplantation.

Following the publication of the ASCEND (A Phase III Trial of Pirfenidone in Patients with Pulmonary Fibrosis) and IMPULSIS (Investigating the Safety and Efficacy of Nintedanib in IPF) trials, two new anti-fibrotic treatments became available for patients who meet stringent National Institute for Health and Care Excellence (NICE) criteria. Pirfenidone and Nintedanib neither cure nor reverse the fibrosis, and have little impact on symptoms, but have been shown to reduce rates of lung function decline and, in the case of Pirfenidone, improve progression-free survival.

Both Nintedanib and Pirfenidone, which are available for use in patients with moderate IPF as defined by an FVC of 50-80% predicted, are associated with side-effects that can affect a patient’s ability to tolerate treatment. Commonly reported side-effects of both are gastrointestinal including diarrhoea, nausea, abdominal pain, and vomiting as well as weight loss and liver enzyme derangement. Additionally, Pirfenidone is associated with skin photosensitivity. These side-effects can be managed with dose reduction, anti-motility agents, taking medication with meals and avoiding sun exposure, but undoubtedly further impact upon health related quality of life.

Update by Dr Emma Denneny

Get Out of Breath for #Breathtember

 

September is #Breathtember – Global Pulmonary Fibrosis Awareness Month

 Get out of Breath for #Breathtember

Tweet Tweet!

https://www.breathingmatters.co.uk/wp-content/uploads/2013/06/twitter.png

To help raise awareness, we ask that supporters tweet different challenges during September including the term ‘#Breathtember and ask their followers to retweet and share this information as widely as possible.

Think outside the box for your challenges – getting out of breath for you could mean:

  • Cycling around your local park
  • Doing a colourful or musical 5K/10K run or walk
  • Singing until you are out of breath
  • Walking over the wondrous London bridges
  • Blowing bubbles … or windmills!
  • Skydiving
  • Or just simply walking up the stairs!

The important thing is that you tweet your challenge including the hashtag ‘#Breathtember’ to raise awareness of pulmonary fibrosis.  Add a photo if you like.  This September, we want as many people as possible to see the term ‘#Breathtember’.

Follow us on Twitter for further details: @Breathingmatter 

twitter

 

 

Pulmonary Fibrosis Wish List

  • £5 – For equipment to take blood for testing antibodies for a patient with IPF.
  • £50 – Allows us to grow individual fibroblasts (these are the cells that produce the scarring) in the laboratory from the lungs of patients with IPF to do further studies.
  • £500 – Allows us to isolate the platelets from patients with IPF so that we can examine them in the laboratory and compare them to platelets from people with normal lungs.
  • £1,000 – For specialised antibodies to help us develop a novel blood test for early detection of PF.
  • £1,500 – For accessories for our lung function equipment for one year.
  • £3,000 – For a study to assess whether treatments for rheumatoid arthritis help the lung disease associated with RA.
  • £5,000 – To provide all the equipment and running costs for ‘Western Blots’ which allows us to look at abnormal proteins in the lungs of patients with pulmonary fibrosis.
  • £10,000 – For a research nurse for a year working 2 days a week to collect valuable patient data and samples for research.
  • £50,000 – Pump priming a blue sky research proposal: allows a senior clinician to undertake a substantial period of research (a year or more) as a named research fellow to develop an hypothesis that is then submitted for full funding (£300K+).

Visit our new Justgiving page

Breathing Matters has a brand new Justgiving page.  We have transferring to a campaign page under the UCLH Charity Justgiving Page.  The new link is: https://www.justgiving.com/campaign/breathingmatters  This will save us on Justgiving fees for Breathing Matters, so more of your money goes directly to where it’s needed!

Please visit our new online donation page to donate, or to just have a look-see 🙂

Current open Justgiving pages on our old Justgiving Page will remain active, so you don’t need to do anything.

Together we can do more!

 

Winter 2019 Newsletter