How does the National Aspergillosis Centre support treatment of this rare disease across the whole UK?

Tuesday, March 19, 2024

Respiratory Futures spoke to Beth Bradshaw of the National Aspergillosis Centre. Below Beth shares about the value the service provides across the UK, and educates us further on the rare disease of chronic pulmonary aspergillosis (CPA).

Rare diseases like chronic pulmonary aspergillosis (CPA) are particularly challenging because it can be hard to build up a critical mass of clinical experience in any one centre. To address this, the NHS commissioned the National Aspergillosis Centre (NAC) in Manchester, which is a highly-specialised service (HSS) that accepts referrals from consultants across the UK.

However, there are challenges associated with managing patients living far from the clinic. Face-to-face appointments are vital, but travel can be a barrier for those with reduced physical mobility and/or low incomes.

Thankfully, the whole of the NHS is going through an unprecedented time of transformation in how it uses communications technologies, especially in record sharing using new EPR systems and distance appointments, which was accelerated by the COVID pandemic. We wrote this article to share our approach to managing a geographically dispersed patient group.

What is CPA?

CPA is a serious long-term fungal infection mainly affecting people with lung conditions such as COPD, sarcoidosis, prior TB. If left untreated (or under-treated), it can lead to worsening lung function, morbidity and even life-threatening haemoptysis.

There are challenges associated with managing patients living far from the clinic. Face-to-face appointments are vital, but travel can be a barrier for those with reduced physical mobility and/or low incomes.

Why does CPA need an HSS?

  • CPA is a rare disease, with around 3600 cases in the UK (Pegorie et al, 2016), although this may be under-diagnosed. Clinical guidelines are available, but most clinicians will have minimal practical experience in interpreting fungal diagnostics and knowing when to start (and stop) prescribing antifungals.
  • It is also a highly variable infection. Simple aspergilloma (fungal ball) can be managed with observation only, but more complex patients with multiple cavities can benefit from referral to tertiary care.
  • The Department of Health takes financial responsibility for our patients and provides money for antifungals, which can be prohibitively expensive. This has allowed us to use newer azoles such as posaconazole/isavuconazole and we are regularly involved in clinical trials for new drug classes.

What do clinicians need from us?

  • Please tell us! We are currently running a survey to gauge the level of awareness among NHS doctors and better understand the needs of CPA patients in different parts of the UK. If you have a moment, we would be very grateful if you could fill in our survey (takes 1-2 minutes) and circulate this link to your colleagues. Even if you only rarely come across aspergillosis, the information is very useful to us.
  • Patients referred to NAC undergo a set of tests that allow us to give a diagnosis and staging, as well as Quality-of-Life score (we use the St George’s Respiratory Questionnaire). We design a treatment plan and monitor their drug levels via therapeutic drug monitoring (TDM), clinical response and any side effects. We continue follow-up until the condition is stable enough to discharge back to their consultant - if their situation changes, they can be re-referred at any time.
  • Consultants can alternatively present their patients remotely at our weekly MDT to get guidance from our experienced nurses, pharmacists, physiotherapists, laboratory staff and radiologists. Our OPAT nurses can advise local hospitals how to administer IV antifungals to outpatients or at home, which reduces the need for hospital admission.
  • Clinicians can also make use of our online resources including patient information leaflets and MIMS module (for GPs) . We regularly publish research in journals/conferences and contribute to clinical guidelines. Our staff are occasionally invited to speak on webinars and podcasts run by other organisers. For example Dr Kosmidis was interviewed by Respiratory GURU podcast for their episodes about ABPA and CPA, and for the MycoTalks webinar series produced by ERC CMM
We are commissioned to run patient support groups and produce information materials that are extremely important for helping patients manage their symptoms, side-effects and lifestyle.

What do patients need from us?

  • We are commissioned to run patient support groups and produce information materials that are extremely important for helping patients manage their symptoms, side-effects and lifestyle.
  • Rare diseases are extremely isolating for patients and their carers, which is detrimental to mental health. Our patient support groups are open to all UK aspergillosis patients (i.e. no referral needed), which include a mixture of real-time chats via Microsoft Teams plus closely-moderated support groups on Facebook and Telegram. These create a sense of community among people who may not know anyone else with this condition, where they can provide each other with emotional support and understanding.
  • We provide a reliable source of information through our patient-focused website (org), which is vital for rare diseases where online misinformation is rife. We also hold monthly meetings on Teams with talks on patient-requested topics such as nutrition, smoking cessation and exercise.
  • Healthcare apps are becoming increasingly popular for managing appointments/prescriptions, communicating with the care team and recording health data. However, many people struggle to use them, particularly those who are older, or have reduced eyesight/hearing/dexterity, or experience ‘brain fog’ while unwell. We provide step-by-step instructions (and occasionally one-to-one help) to make sure these technologies are accessible to all of our patients.
  • Every year we run a thorough feedback questionnaire that allows patients to tell us what aspects of the service are working well and what areas need to be adjusted. We also encourage them to give candid opinions during the support meetings

Geographical distribution of CPA

  • We receive referrals from all over the UK. While we do not yet have an accurate picture of how CPA prevalence varies across the UK, we do know that COPD (the main risk factor) is more common in the North of England. However, other risk factors such as prior tuberculosis may be more common around London and in Scotland.
  • We are keen to engage with respiratory clinicians to find out how we can better support them and hope to interact and raise awareness in meeting such as the British Thoracic Society meeting(s) later this year.

In-person vs distance appointments

  • At the time of joining the service, each patient visits NAC in person for a ‘one-stop-shop’ that includes a suite of specialist diagnostic tests including radiology/blood serum/sputum/biopsy/lung function/bronchoscopy to assess their needs. They also receive an information pack and details for joining the support groups.
  • After this, we often use video/telephone appointments but aim to see patients in person at least once per year - currently around half of our appointments are face-to-face. Our annual feedback survey found that patients are happy with this approach (Figure 1).
  • Postal packs are used for sending sputum samples to our laboratory accompanied by a video produced by our physiotherapist Phil Langridge that teaches practical tips for producing sputum.
  • We send out postal packs for sending blood samples for antifungal drug levels and Aspergillus serology, and can arrange home delivery of antifungal medication.

Figure 1   Results from our annual patient feedback survey found that distance appointments were popular with our patients

What does our CARES team do?

  • Community - Awareness - Research - Education - Support
  • Our funding allows us to employ several non-clinical staff with backgrounds in science and communications, who run the hugely successful patient support groups and manage our websites/social media. They also help clinical staff to promote their research to the public and at conferences.
  • We founded an annual awareness day #WorldAspergillosisDay (Feb 1st) that has grown in popularity over the years. You can watch recordings from previous years on our YouTube channel.

What are some ways to get involved or learn more?

- Make a referral or join our MDT

- Free online support and information for patients & carers

- Send samples to our mycology reference laboratory

- Find out more about NAC

- Join our clinician mailing list or NHS Networks group for updates