1. Dr Julius Cairn

2. Introduction - update COPD Exacerbations Pulmonary rehabilitation Lung cancer - NSCLC Treatments for patients with limited lung function Tyrosine kinase inhibitors for patients with metastatic adenocarcinoma Treatment of Idiopathic Pulmonary Fibrosis PANTHER-IPF trial

3. Introduction – service developments Non-invasive ventilation Current acute NIV service Planned Domiciliary service Ambulatory diagnostics/ care for pleural effusion

4. COPD – exacerbations and importance of pulmonary rehabilitation Impact of exacerbations Sustained decrease in exercise tolerance due to peripheral muscle dysfunction Loss of lung function and ↓ HRQL in frequent exacerbators Not random events, can cluster – high risk in the 8 weeks after first exacerbation Pulmonary rehabilitation Effective after exacerbation – where high risk of readmission Reduces hospital admission No. pts needed to treat to prevent one admission is 4

5. COPD – management of inflammation, beyond corticosteroids [TORCH - Salmeterol/Fluticasone; Tiotropium] PDE4 inhibitors - Roflumilast Pooled analysis of two large 12 month studies Improves lung function and reduces COPD symptoms Reduces rates of moderate or severe exacerbations Increases time to onset exacerbations in severe to very severe COPD pts who are on LABAs Theophylline – in vitro but not in vivo Macrolides – Azithromycin : 28% ↓ exacerbations ? Statins

6. Lung cancer NSCLC 75-80% SCLC 20-25% For NSCLC Resection rate 10-20% Stages IA – IIB operable ( 5yr survival IA 69%) Some N2 disease patients are suitable for CHART

7. Lung cancer – NSCLC limited stage but medically inoperable Treatment for limited stage ca bronchus in patient with limited lung function Stereotactic radiotherapy – stage I (and II) NSCLC 52-83% survival at 2-5 years Small peripheral lesions, medically inoperable, as good as radical DXT Radiofrequency ablation 3 year disease free survival 53% and cancer specific 80% Limited procedure related morbidity – pneumothorax, pleural effusion; no significant fall in lung function for COPD pts

8. Lung cancer – NSCLC advanced stage – need for detailed pathological and molecular diagnosis Platinum based chemotherapy for stage IIIB and IV Only a 1/3 patients are fit to receive chemo Cisplatin/Pemetrexed for non-squamous Carboplatin/Gemcitabine for squamous ( Bevacizumab + chemo for non-squamous) Advanced adenocarcinoma EGFR mutation status Tyrosine kinase inhibitors eg Gefitinib – as first line treatment –dramatic improvement in progression free survival (PFS), overall response rate 25%

9. Management of idiopathic pulmonary fibrosis 80,000-85,000 people in Europe Median survival between 2 and 5 years 5 yr survival of 20% Pirfenidone Reduces disease progression by 30% (C) Significantly reduces the decline in VC from baseline(C)

10. Management of idiopathic pulmonary fibrosis IFIGENIA trial – evaluate the effects of adding N acetyl cysteine (NAC) to patients receiving Azathioprine and Prednisolone Pts receiving NAC for 12 months slower deterioration in VC and DLCO PANTHER-IPF Triple therapy - excess mortality compared to placebo NAC arm still proceeding

11. Management of idiopathic pulmonary fibrosis Looking for comorbidity Gastro-oesophageal reflux Pulmonary rehabilitation Ambulatory oxygen therapy Pirfenidone NAC Palliative care Specialised clinics Initiation of triple immunosuppressive therapy is now contra-indicated

12. Service development – Pleural effusion Step 1 - Diagnostics – Pleural Clinic consultation and ultrasound guided procedures speed up diagnosis try to avoid admission to hospital avoid day ward attendance link in with surgical services for thoracoscopy Treatment of malignant pleural effusion Ambulatory care tunnelled indwelling drains – managed through the Pleural Clinic or via short-stay beds Step 2 – Diagnostics - Medical Thoracoscopy Diagnosis and treatment with pleurodesis (currently medical/VATS) Need day case/ short-stay beds – reduced length of stay

13. Service development – Pleural Clinic Pleural disease clinic Consultations ( first half of a morning clinic) Diagnostic and therapeutic aspirations – via USS Chest drain insertion – via USS Including pleurx or tunnelled indwelling catheters for recurrent malignant effusion

14. Service development – Non-invasive ventilation Currently Use NIV acutely for decompensated hypercapnic (type II) respiratory failure COPD exacerbation Obesity Hypoventilation syndrome Obstructive Sleep Apnoea with type II respiratory failure Neuromuscular patients

15. Service development – domiciliary non- invasive ventilation COPD Recurrent admissions with exacerbations and type II respiratory failure Persistent respiratory acidosis when at target oxygenation Sleep disordered breathing Obesity hypoventilation syndrome Type II respiratory failure complicating obstructive sleep apnoea Neuromuscular disease

16. Conclusion Update in management of three chronic respiratory conditions – focus reducing morbidity & mortality COPD – reducing exacerbations Lung cancer Idiopathic pulmonary fibrosis Service developments in NIV and pleural disease care Shorten the interval to diagnosis and treatment and reduce length of hospital admission