Liam Hill
A new survey, published by the charity Action for Pulmonary Fibrosis has revealed what it is like for people living with this debilitating condition, as well as highlighting what needs to change to ensure they feel supported throughout their journey.
Pulmonary Fibrosis is a respiratory condition which can often take years to diagnose. However, with its rapid progression and often late diagnosis, the average life expectancy for people with Idiopathic Pulmonary Fibrosis (IPF) is just five years.
Pulmonary fibrosis (a group of progressive interstitial lung diseases) is a fatal disease where the lungs become progressively stiffer and smaller. The only curative treatment at present is a lung transplant as current medicines can only slow the progression.
However, people with the condition say that despite Pulmonary Fibrosis being as serious and deadly as cancer, healthcare awareness and support for people with PF is much more limited.
One person who took part in the survey said:
“It’s an awful thing to say but I wish it was cancer. There would be more support if I had cancer.” And another said: “PF is the ‘poor relation’ of terminal conditions.”
Issues start before sufferers are even diagnosed, as 76% said their GP did not say they may have PF before making a referral, and 10% waited between 7 and 10 months to see a respiratory consultant.
Pulmonary Fibrosis sufferers have also said that, unlike cancers, many healthcare professionals are not clued up on PF, and fail to recognise the symptoms, which can lead to a late diagnosis.
One person said they were diagnosed in their 30s but when they went to the GP complaining of breathlessness, they were sent away without tests and told to go to the gym. “It took years for diagnosis because health professionals kept saying it was something else even suggesting it might be psychosomatic,” another said.
What needs to change?
Diagnosis
According to the report, an early, fast, and accurate diagnosis is essential to improving the outcome for people with PF. The progressive nature of the disease means the sooner someone is diagnosed, the quicker they can be started on disease-slowing drugs.
Despite this, many participants said they had symptoms such as breathlessness and persistent cough for a long time but they were often diagnosed with other respiratory conditions, such as asthma or COPD, before being referred.
Issues have also been raised about the delivery of the diagnosis, with many saying that they were informed of their condition in an insensitive manner, and were sometimes not told their condition was terminal.
Meanwhile, some people said they were not informed of their diagnosis and found out subsequently, such as when looking at their records in the NHS App or when asked to participate in a clinical trial. Others said they were informed by letter, over the phone or by voicemail, which left them feeling shocked and unsupported.
The statistics show that nearly half were not signposted to information or support services at the time of their diagnosis, and nearly a third said their diagnosis could have been delivered more sensitively.
Management
Issues have also been raised around the management of the disease, as the report shows there is an ‘enormous variation’ in care following diagnosis. Some people report having helpful nurses and access to a specialist to help their condition.
However, it seems there is a discrepancy in care across NHS Trusts and regions as others said that ‘appointments are simply not available’, and it’s ‘impossible’ to speak to a specialist.
“A two-month wait to see a consultant after diagnosis without any real support and understanding of PF was shocking and very difficult to manage my partner’s deterioration. He died before ever getting to his first appointment,” one person said.
Meanwhile, many said they would like more regular contact with their consultant (more than every 12 months) and more regular lung function tests to monitor their progress. People with PF also said they would like their GPs to have more training and access to specialist information so that they can assist with monitoring their PF.
Many have said that some healthcare professionals do not understand the condition, and make problematic suggestions such as weaning off of oxygen, even though this would rarely be the case for people with PF.
Access
According to the report, people with PF say they feel isolated and disconnected due to the lack of access to support groups as nearly half said referring to a support group when they were diagnosed would have made a difference.
Issues were also raised about access to appointments, treatment, mental health appointments and end-of-life care.
Access to appointments
Accessibility issues were raised around mobility and problematic travel to specialist appointments. Some people said it was stressful to get to hospital appointments while using oxygen and mobility aids, and others were forking out hundreds of pounds to pay for hotels the day before their appointment to minimise stress.
One person said they have to ‘pre-plan’ every excursion and calculate their oxygen supplies. While another said they ran out of oxygen on their way to an appointment because they had to travel so far.
“I have oxygen permanently and unsure of how much I need to travel with. I ran out last time I went because it’s so far to travel. I took two cylinders and could not get anyone to help me at the hospital. Eventually, someone gave me a cylinder but it’s frightening as I have to have oxygen constantly now,” they said.
Pulmonary Rehabilitation
Access to Pulmonary rehabilitation, which can improve the quality of life for people with PF was also raised, as some said the waiting list is so long, that when it came to their turn to finally have it, their condition was too bad to take part.
Those who have taken part in the rehabilitation programme spoke positively of the service, but at present, it is not offered as standard. Many reported difficulty accessing this treatment, with very long waits or the amount offered was limited.
Meanwhile, in some areas, it is only accessible once per year, whereas for others there is a supply and demand issue as some people with PF can access six weeks of pulmonary rehabilitation at the hospice but some others face long waits at the hospital.
Some also said they were doing pulmonary rehabilitation alongside people with COPD without specific support for their condition.
Oxygen therapy
PF involves inflammation and scarring of the lung tissue, leading to reduced lung function and impaired oxygen exchange. Oxygen therapy is essential to help people with PF maintain oxygen levels and relieve breathlessness. However, according to the report, obtaining oxygen supplies is a ‘postcode lottery’ causing major issues for sufferers.
The report explains that the availability of oxygen has a substantial impact on day-to-day life and can leave people frightened to leave the house as they fear running out. Others have said they struggle with the weight of oxygen cylinders, which can lead to isolation.
Meanwhile, others have said trying to work out the oxygen needed for the length of time spent out is stressful and delays and challenges exist when trying to order supplies.
“Being on oxygen is bad enough, but it’s so stressful worrying if you have enough to last. It restricts how often you can go out,” one person said, while another added that they ‘live in fear of running out’.
Mental health
Concerningly, despite being diagnosed with a terminal illness, almost 90% of respondents said they had not received support from a mental health practitioner.
Those involved describe PF and the uncertainty that comes with it as being very difficult to cope with, reporting depression, anxiety, lack of motivation, isolation and lack of peer support. There was an overwhelming desire for better condition-specific mental health support.
One person said: “I’ve had NO support whatsoever… I got help off my own back, I had a breakdown last year,” and another added: “People can’t see this illness. They say you look well but they don’t know how you feel inside”.
Overall, it is clear that more needs to be done to support people with PF to ensure that sufferers have a better quality of life. Figures show that there are more deaths from IPF every year than some cancers, so more research, understanding and support need to be available.
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