Interstitial lung disease: end of life care Professor Joanna Porter, - PowerPoint PPT Presentation

RCGP accredited until 1 st April 2020 RCN accredited until 2 nd July 2020 Accreditation applies only to the educational content and not to any product RCN cannot confirm competence of any practitioner Interstitial lung disease: end of life care Professor Joanna Porter, Consultant in Respiratory and General Medicine University College London Hospitals and Senior Lecturer in the Department of Medicine at UCL In conversation with Dr Steve Holmes, GP, Park Medical Partnership, Shepton Mallet and Evercreech Education Lead and Executive, Primary Care Respiratory Society UK This video has been developed and funded by Boehringer Ingelheim Date of preparation: October 2019 | SC-UK-00619 This non-promotional material is for medical-to-medical use only

By the end of this module you should be able to: ▪ Outline the impact of ILD on life expectancy in patients with a diagnosis of ILD ▪ Identify appropriate palliative care for symptom control and when to consider: – Oxygen therapy – Opiates – Benzodiazepines ▪ Describe which patients may be suitable for a lung transplant ILD, interstitial lung disease

IPF has the worst prognosis of ILDs with a survival rate of 20 – 30% over 5 years 1 100 90 90 5-year survival rate 80 (% of patients) 65 70 60 60 47 50 40 20 – 30 30 18 20 10 0 1 3 Lung and IPF Ovarian PAH Colorectal Breast cancer 2 2 bronchus cancer 2 cancer 2 cancer 5-year survival rate of IPF in comparison with other conditions IPF, idiopathic pulmonary fibrosis; ILD, interstitial lung disease; PAH, pulmonary arterial hypertension 1. Ley, B. et al. (2011); 2. American Cancer Society: 5-year relative survival, 2007 – 2013; 3. Benza, R.L. et al. (2009).

The overall goals of treatment in ILD are to improve quality of life, maintain activity levels, control symptoms and prolong survival Adult with ILD Assess for pulmonary rehabilitation Offer best supportive care and Offer pharmacological symptom relief treatments Explore lung transplantation Review and follow up Care at the end of life ILD, interstitial lung disease Adapted from 5. NICE clinical guideline 163. June 2013 (last updated May 2017).

Summary: end of life care ▪ Many patients will need palliative care to manage their symptoms ▪ Discussing this with patients at an appropriate time is important; good communication with patients and their families is key ▪ Oxygen therapy, opiates and benzodiazepines may be helpful ▪ For some patients, a transplant may be an option ILD, interstitial lung disease 5. NICE clinical guideline 163. June 2013 (last updated May 2017).

References 1. Ley, B., Collard, H.R. and King, T.E. Jr. (2011). Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med, 183(4), pp. 431 – 440. 2. American Cancer Society: 5-year relative survival, 2007 – 2013. Available at : https://cancerstatisticscenter.cancer.org/#!/data-analysis/SurvivalByStage (accessed October 2019). 3. Benza, R.L., Miller, D.P., Gomberg-Maitland, M., Frantz, R.P., Foreman, A.J., Coffey, C.S., Frost, A., Barst, R.J., Badesch, D.B., Elliott, C.G., Liou, T.G. and McGoon, M.D. (2009). Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation, 122(2), pp. 164 – 172. 4. Ley, B., Ryerson C.J., Vittinghoff, E., Ryu, J.H., Tomassetti, S., Lee, J.S., Poletti, V., Buccioli, M., Elicker, B.M., Jones, K.D., King, T.E. Jr and Collard, H.R. (2012). A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med , 156(10), pp. 684 – 691. 5. National Institute for Health and Care Excellence. Clinical guideline 163. June 2013 (last updated May 2017). Idiopathic pulmonary fibrosis in adults: diagnosis and management. Available at: www.nice.org.uk/guidance/cg163 (accessed October 2019). 6. Travis, W.D., Costabel, U., Hansell, D.M., King, T.E. Jr, Lynch, D.A., Nicholson, A.G., Ryerson, C.J., Ryu, J.H., Selman, M., Wells, A.U., Behr, J., Bouros, D., Brown, K.K., Colby, T.V., Collard, H.R., Cordeiro, C.R., Cottin, V., Crestani, B., Drent, M., Dudden, R.F., Egan, J., Flaherty, K., Hogaboam, C., Inoue, Y., Johkoh, T., Kim, D.S., Kitaichi, M., Loyd, J., Martinez, F.J., Myers, J., Protzko, S., Raghu, G., Richeldi, L., Sverzellati, N., Swigris, J. and Valeyre, D.; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. (2013). An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med, 188(6), pp. 733 – 748. 7. Weill, D. (2018). Lung transplantation: indications and contraindications. J Thorac Dis , 10(7), pp. 4574 – 4587. 8. Kärkkäinen, M., Nurmi, H., Kettunen, H.P., Selander, T., Purokivi, M. and Kaarteenaho, R. (2018). Underlying and immediate causes of death in patients with idiopathic pulmonary fibrosis. BMC Pulm Med , 18(1), pp. 69.