Sarah Agnew and Annette Duck, ILD specialist nurses based in the NorthWest of England – a region dealing with the greatest prevalence of Idiopathic Pulmonary Fibrosis (IPF) in the country – outline the challenges and the way ahead in tackling the impact of this deadly disease
More than 4,200 people are now living with idiopathic pulmonary fibrosis in the North West alone, the highest population of patients with this fatal lung disease in England, a network meeting of specialist respiratory nurses heard recently.1
Understanding this figure is critical because it’s a reflection of the referrals we then see in our services, says Sarah Agnew, ILD specialist nurse at Aintree NHS Trust. “However, our referral rate for IPF is only around 150-200 patients a year – so where are these individuals, and why aren’t they being referred to specialist centres?”
Whatever the situation, Sarah explains, there is a clear impact on service provision – both currently and in the future. “These patients should be seen by a specialist centre and having specialist input – particularly in hotspot areas – even if it’s just to get a confirmed diagnosis from a multi-disciplinary team as set out by the NICE Quality Standards2.”
With this in mind there is also a distinct possibility the condition is under-diagnosed, Annette Duck, independent ILD specialist nurse adds. “I suspect a number of patients are under the care of general chest physicians and are not referred to specialist centres where a true diagnosis would be made,” she stresses.
Overcoming diagnosis challenges
“So although things are improving, under-diagnosis, mis-diagnosis and a lack of awareness of both services and available treatment remains,” Sarah points out. Even among those individuals referred to specialist centres, around 20% will be given an alternative diagnosis – usually another ILD, or even COPD, she says.
Ultimately, Annette adds, these are complex conditions to diagnose even with specialist expertise and it is critical these patients are picked up earlier in the pathway. “We still have cases where patients are referred to us with evidence of significant disease progression but because of the length of time it’s taken to get to specialist care may be towards the lower end of the eligibility criteria for treatment – when we know people are less likely to tolerate the drugs – or indeed too advanced to receive medication.”
So one major challenge in diagnosis and care, both Annette and Sarah stress, is ensuring patients are referred from GP to general chest clinics into the specialist ILD multi-disciplinary team. “The red flag for GPs should be an individual presenting with cough, crackles and breathlessness on exertion,” says Annette. “We have to recognise that GPs are under a huge amount of pressure so we have to make it simple, we have to make it snappy, it’s got to be one of their targets.”
Tackling resource shortfalls
Then, it’s having the resource in place to manage the sheer number of patients coming through to specialist centres. Currently, Sarah explains, the ability – or inability – to access specialist respiratory nurses is “a big problem in the centres around England. There just aren’t enough to go around.”
So ensuring patients are sent to the specialist centre, having the resources to be able to see them in a timely manner and accessing the support required to be able to treat and manage these individuals is a significant problem, they note. “We should not have to compromise the care we give because we haven’t got the resources in place.”
One way to overcome this hurdle, say Annette and Sarah, is to ensure information is cascaded down from the specialist centres to those on the front line in primary, secondary and tertiary care. In my view, says Sarah, we need more ILD training in the UK to educate GPs, practice nurses, community nurses and palliative care services – not only about the need for early diagnosis and symptom checkers – but the level of local care and support required for people living with IPF. “It’s a team effort, we’re all in this together.”
Of course, the provision of education and information extends not only to HCPs throughout the care pathway, but to the patients and families themselves.
“Patients are understandably frightened and come into clinic wanting to see a healthcare professional who understands their diagnosis; they’re hungry and desperate for information about their disease and their future prognosis.”
The information cascade
Although the information given to patients is vastly improved compared to five years ago, a large number of individuals and their families are still attending clinic armed with very little understanding or knowledge of their condition or the services they are entitled to, Sarah adds. After diagnosis, it’s about empowering patients to go to their GP and tell them that they’ve got a serious condition and they need help – and how to access information and support in the community.
“Patient power is critical – if the individuals and their relatives stand up and start mobilising the patient voice it will bring IPF out from under the radar and ensure it is made a greater priority at government level.”
“But equally we can’t forget how far we’ve come,” they say. “This isn’t the time to take our foot off the accelerator and pat ourselves on the back, the key thing is to keep the momentum up and continue pushing forward.”
- British Lung Foundation (2016) The Battle for Breath – The Impact of Lung Disease in the UK https://www.blf.org.uk/what-we-do/our-research/the-battle-for-breath-2016 (Last accessed October 2016).
- NICE (2015) Idiopathic Pulmonary Fibrosis in Adults – Quality Standard QS79 https://www.nice.org.uk/guidance/qs79 (Last accessed October 2016)
UK/RESP-161434 Date of preparation: October 2016