A woman from Runcorn is leading a campaign to raise awareness about lung disease and improve treatment for sufferers as cases rise and treatment dips due to coronavirus.
Sarah Jones, 39, suffers from asthma and lost her dad to idiopathic pulmonary fibrosis in 2013, a progressive and irreversible disease that causes scarring of the lungs.
John Jones experienced breathlessness, a dry cough and clubbing of his fingers and was eventually sent for a CT scan and diagnosed with IPF.
The family was devastated to learn that IPF is a terminal disease from an internet search, which the specialist failed to mention.
Mr Jones’ next appointment was due six months later but was delayed by a month and he passed away at home in the meantime.
Sarah said: “Paramedics worked on my dad for 45 minutes and couldn’t save him.
“He missed his first grandson by five days and my wedding and giving me away by two weeks. It was a horrible time for the family.”
Sarah and her dad
A post-mortem later confirmed that Mr Jones died from a pulmonary embolism which caused respiratory arrest, as a result of IPF.
Since Mr Jones’ death the family has been passionate about raising awareness of IPF and improving treatment for patients.
Sarah teamed up with MP for Weaver Vale in Cheshire, Mike Amesbury, Taskforce for Lung Health, the Pulmonary Fibrosis Trust and Action for Pulmonary Fibrosis (APF) and petitioned Health Secretary Matt Hancock to widen the availability of life-prolonging anti-fibrotic drugs.
Currently patients only qualify for the drugs when their lung capacity declines to between 80% and 50%.
Sarah said: “I want the capacity range to be increased because it’s a terminal disease and I want you to be able to get as much time with your family as possible.”
MP Mike Amesbury said: “Helping to keep IPF on the health agenda both locally and nationally is something I’ll continue to do.”
The large number of people hospitalised with Covid-19 increased the urgency of this campaign due to it’s similar lung scarring effect in many cases.
Prof Gisli Jenkins said: “Pulmonary Fibrosis and Covid-19 have shared mechanisms in respect of cell injury and failures in lung repair.”
Chair of APF Steven Jones said: “As we face a potential surge of pulmonary fibrosis patients as a result of coronavirus it is more important than ever that anti-fibrotic drugs can be prescribed as soon as people are diagnosed.”
Mr Jones himself suffered from IPF for eight years and received a life-saving single lung transplant in 2016.
He said: “It’s a really cruel and horrible disease, worse than most cancers; with cancer you have a chance of remission but there’s no chance with IPF except a lung transplant, which are only given to very few people.”
The coronavirus epidemic is also limiting monitoring of IPF, such as lung function tests which allow patients to understand the status of their condition and how long they have left.
Prof Toby Maher said: “Research confirms that early treatment with anti-fibrotic medication extends life expectancy by at least two to three years.
“Given this, it is heart-wrenching that in the UK, unlike in all other European countries, doctors cannot treat people with pulmonary fibrosis at diagnosis but instead have to wait until their lungs get irreversibly worse through scarring."
Aside from campaigning Sarah is an ambassador for the Pulmonary Fibrosis Trust which is a charity that offers practical, emotional and financial support to patients.
She also runs local and online support groups for people suffering with IPF and their families.
Sarah said: “It’s our legacy to my dad to make a difference to people and to never let a family go through it alone like we did because we struggled without any support.”
The main online support group is Pulmonary Fibrosis UK Facebook group, where Sarah met Ron Flewett, a sufferer of IPF on the waiting list for a double lung transplant.
Everton fan Ron at Goodison Park
Mr Flewett, 59, said: “Living with IPF is like living on a knife edge all the time, knowing that there is no cure, and catching a simple infection or cold can be fatal.
“Physically it affects everything I do, walking up a slight incline is major task and leaves me exhausted; mentally I feel like the world is closing in on me.”
He expressed gratitude for Sarah’s friendship, highlighting the importance of these support communities.
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