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What does a specialist ILD service look like?


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Presentation delivered by Dr Melissa Heightman, Consultant Integrated Respiratory Physician, at the Pan London Airways Network Meeting

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What does a specialist ILD service look like?

  1. 1. What does a specialist ILD service look like? Dr Melissa Heightman, Consultant Integrated Respiratory Physician DISCLAIMER: The views and opinions expressed in this presentation are those of the authors and do not necessarily represent the views and policy of PLAN(Pan London Airways Network).
  2. 2. Why a “specialist” service?  Relative rarity of diseases (increasing incidence) 1 or 2 patients/ GP practice  Challenging diagnostically (200 types)  Improved outcomes in specialist centres  All IPF patients discussed in tertiary MDT and tertiary service accessed for new diagnoses of ILD  Uniformity of access  Raise quality of care  Access to clinical trials  Specialised/ expensive treatments  antifibrotics/ lung transplantation
  3. 3. 3  LONDON  UCLH, RBH, Imperial, GSST
  4. 4. Catchment area
  5. 5. Rheumat -ologist The ILD service PATIENT with ILD Pathologist ILD specialist Respiratory physicians Clinical research fellows Palliative Care ILD research nurses ILD respiratory nurse Community respiratory services Radiology Pulmonary Rehab ILD MDT coordinatorOxygen assessmen t Transplant outreach Lung function G.P Bronch/ cryobiops y Cytotoxic biological infusion facility Respiratory pharmacist
  6. 6. Classification of ILD 6 Meghna Jani, Nik Hirani, Eric L. Matteson & William G. Dixon Nature Reviews Rheumatology 10, 284–294 (2014) doi:10.1038/nrrheum.2013.197
  7. 7. or cryobiopsy Thorax 2008;63(Suppl V):v1– v58. doi:10.1136/thx.2008.101691 Diagnostic Algorhithm for ILD cryobiopsy
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  11. 11. or cryobiopsy Thorax 2008;63(Suppl V):v1– v58. doi:10.1136/thx.2008.101691 Diagnostic Algorhithm for ILD cryobiopsy
  12. 12. Databases Definite IPF Possible IPF Idiopathic NSIP RBILD DIP LIP Pneumoconioses Hypersensitivity pneumonitis Iatrogeni c ILD Sarcoidosis ILD in RA ILD in systemic sclerosis ILD in polymyositis/derm atomyositis ILD in Sjogrens ILD in SLE ILD in UCTD Lung dominant CTD ANCA vasculitis Combined pulmonary fibrosis and emphysema Langerhans Cell Histocytosis LAM unclassifiable
  13. 13. Research  Prognostic value of PET/CT in patients with ILD: radiology, Lung function and QoL Correlation  Living airway biobank project  EME TIPAC (cotrimoxazole)  Platelet and white blood cell trafficking  MUSC-5107 (Role of nintedanib in RA-ILD)  Mechanisms of lung injury and repair  BI study of nintedanib + pirfenidone Every patient offered the opportunity
  14. 14. Treatment/ care ILD Oxygen Pulmonary Rehab Symptom control / palliation Antifibrotics Immune suppressionpharmacotherapy Lung Transplantation End of life care
  15. 15. Antifibrotics NICE approved. FVC 50-80% predicted (£71.7/ day) £20 000-£30 000 per QALY  Pirfenidone  Small molecule  First clinical trial 1999  ASCEND study, n=555. 47.9% reduction in the proportion of patients with an absolute decline of >10% in FVC or death. 132% increase in the proportion of patients with no change in FVC  Pooled analysis with previous CAPACITY study showed reduced risk of death at 1 year by 48%  Passport study: 85/311 discontinued due to adverse drug reaction (GI/ skin rash/ photosensitivity)
  16. 16. Antifibrotics • Nintedanib (£71.7/ day) – Intracellular inhibitor of tyrosine kinases – Multiple pathways targeted (PDGF receptors, VEGF receptors, fibroblast growth factors receptor etc….) – Indirect effects of TGFb/ fibroblasts/ matrix production – INPULSIS study, ~50% reduction in decline of FVC • Some effect of increased time to first exacerbation • Theoretical increased risk of bleeding. Diarrhoea commonest SE – Only 1 tablet bd rather than 3 tds 16
  17. 17. Not just about diagnoses and drugs! • Non –pharmacological interventions – Pulmonary rehabilitation • Symptom control • Managing breathlessness • Anxiety – Cough and GORD • Stop Smoking as treatment • Advance care planning and dying
  18. 18. Pulmonary rehabilitation • Physical deconditioning is common to all chronic respiratory disease • PR recommended in ATS and NICE guidelines for IPF • But few RCTs • Increase in 6MWD up to 81m reported with improved functional status and QoL. Duration of benefit variable (6 or 11 months) • Role of supplemental oxygen uncertain • More studies needed • Anecdotal experience can be very positive • PR is about more than exercise training. • ? Role of ILD focused cohorts • ILD patients not accepted in Camden • Vainshelboim B, Oliveira J, Yehoshua L, et al. Exercise training-based pulmonary rehabilitation program is clinically bene- ficial for idiopathic pulmonary fibrosis. Respiration. 2014;88(5):378–388. • Jackson RM, Gomez-Marin OW, Ramos CF, et al. Exercise limitation in IPF patients: a randomized trial of pulmonary rehabilitation. Lung. 2014;192(3):367–376. • ILD patients not eligible in Camden 18
  19. 19. Oxygen • BTS guidelines 2015 – Same LTOT criteria as COPD (7.3kPa/ 8kPa) – Not to assess for nocturnal oxygen or ambulatory oxygen unless LTOT – Ambulatory oxygen can be used during PR – Some ILD patient may benefit from AOT without LTOT • Frank et al 70 IPF patients, retrospective study – 29 patients not on LTOT- mean 81m increased walk distance – 41 patients on LTOT – mean 16.9 m increased walk distance • NICE: “People with idiopathic pulmonary fibrosis frequently demonstrate a fall in oxygen saturation during exercise even though they are not hypoxic at rest. In such people, ambulatory oxygen is often provided to improve exercise capacity, enhance mobility and enable activities of daily living in order to improve quality of life.” • Remember to assess smoking risk !
  20. 20. Palliative Care  Making time and finding the right setting  Needs assessment tool to ensure we are picking up on patients needs more uniformly and to aid dialogue  NAT:PD-ILD eg. Red flags: • Clinical evidence of RH failure • Repeated unscheduled contact with hospitals • Failure to attend clinic • Deteriorating performance status  Building relationships with local services- overcome geography  Achieving preferred place of death. Challenge of extreme hypoxia  Use of oramorph and lorazepam  Evidence of benefits of early case conferencing in severe disease • Bajwah et al Thorax 2015 20
  21. 21. Integrated/ patient centred care  Achieve continuity across pathway/ work across boundaries  Avoid duplication  Good communication  Generic referral form with better coordination of information transfer  Single visit assessment  Email advice line  Access to a named ILD nurse 3 miles
  22. 22.  NHS England: any help in developing patient centred care?  Partnership with local CCGs and current integrated respiratory services- parity  Support of community respiratory nurses and GPs  Record patient experience  Support groups and charities Community Respiratory Team, Islington and Haringey Integrated/ patient centred care
  23. 23. Thank you Questions? 23