Patient Priority Day

January 30, 2018 | Author: Anonymous | Category: Science, Health Science, Immunology
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Patient Support Day Nottingham March 2012

Contibutors • • • • • • • • • •

Dr Gisli Jenkins Dr Vidya Navaratnam Professor Simon Johnson Carole Mallia David Cashman Dr Sanjay Agrawal Geraldine Burge Dr Helen Parfrey Annette Duck British Lung Foundation.

• All funding from the Nottingham Respiratory Biomedical Research Unit

Program •

The Science of IPF – How much IPF is there and how bad is it? – Why does IPF happen? – What have clinical trials in IPF taught us?

Practical Management of IPF – – – –

Lung Transplantation Best Supportive/Palliative Care Oxygen Therapy Travelling, flying and exercising with IPF

The Future for IPF – Developing an IPF support network – Potential New Clinical Trials – Lung Tissue Research Proposals

Question Time

Aims of Today • To inform patients of what we know. • To offer practical advice. • To answer questions. • To find out what’s important to you. • To develop a strategy to improve the care of people with IPF throughout the UK

What is IPF?

It's a new killer baffling doctors. And the only warning sign is feeling out of breath... Read more:

Interstitial Lung Diseases Idiopathic Pulmonary Fibrosis • Average age > 60 • Male > Female • No Known Cause • No proven therapy • Median survival 3 years • Also known as Cryptogenic Fibrosing Alveolitis

Other Stuff • • • • • • • • • • • •

Sarcoidosis Connective Tissue Disease Asbestosis Hypersensitivity Pneumonitis Non Specific Interstitial Pneumonitis Desquamative Interstitial Pneumonitis Respiratory Bronchiolitis ILD Cryptogenic Organising Pneumonia Acute Interstitial Pneumonitis Idiopathic Pleuroparenchymal Fibrosing Elastosis LAM HX

ILD Olympics CTD




HSP Drugs The rest

Why do people get Idiopathic Pulmonary Fibrosis? • It is NOT infectious • It is genetic in a small minority of cases

Causes of IPF • Idiopathic (and cryptogenic) means “we don’t know” • Genetics plays a role – Surfactant protein D – Muc5b – Telomerase

• Other hypotheses include: – Viral infection – Gastro-Oesophageal Reflux Disease – Inhaled dust/smoke – Immune system going wrong

• Problem is distinguishing cause from association

What about pulmonary fibrosis generally? • Immune reaction to birds • Immune reaction to drugs • Inhaled dusts leading to injury of the lung • Inhaled chemicals injuring the lung

Interstitial Lung Disease Unit

Goodwin and Jenkins Biochem Soc Trans 2009

Interstitial Lung Disease Unit

Wrong place, wrong time, and then some, hypothesis • If you have the wrong genes (?Muc5b polymorphism) • If you have the wrong exposure (?Metal dust) • And then your repair process breaks down! (epigenetics) • You develop IPF

What happens when your cell gets injured?

Cell dies (apoptosis)

Neighboring cell divides • Risky time for cells • DNA taken apart and then put back together • This can lead to the introduction of genetic mistakes • This can lead to reprogramming of cells

Sometimes just the DNA gets damaged • Again risky time for cells • Complex repair process can lead to errors • Even small mistake can have profound consequences • Average gene contains thousands of DNA molecules

Just one mistake can lead to an amino acid change which can completely change the function of a protein

Complex disease pathogenesis • You need to be on the road to have a RTA • BUT NOT ALL ROAD USERS WILL HAVE AN RTA. • The more you use the road the higher your chance of an RTA • A car is more likely to kill a pedestrian than a cyclist • A motorcyclist is more likely to die in RTA than any other road user

Complex disease pathogenesis • The more often your cells divide the more likely they are to acquire errors • The more often your cells get injured the more likely they are to acquire errors • Some things will injure certain cells/genes over others • Certain cells/genes are more prone to injury than others

So why do people get lung fibrosis? • They get older (more cell divisions) • Their lungs get injured (cigarette smoke, gastroesophageal reflux.) • They have susceptible genes

Lung transplantation

Interstitial Lung Disease Unit

IPF is the second commonest indication for lung transplantation COPD IPF CF Alpha1-AT deficiency

34-38% 17-23% 17-19% 9%

Registry Data ISHLT lung transplantation 22nd report 2005. LAIA 2002

Interstitial Lung Disease Unit

ISHLT Guidelines for lung transplant in people with IPF Referral • Histologic or radiographic evidence of UIP irrespective of vital capacity. • Absence of major CI Transplantation - Histologic or radiographic evidence of UIP and any of the following: - A DLco of less than 39% predicted. - 10% or > FVC during 6/12 follow-up. - O2 Sats < 88% during a 6-MWT. - Honeycombing on HRCT (fibrosis score of > 2).

JHLT, July 2006 Interstitial Lung Disease Unit

Lung transplant leads to improved survival in IPF

Thaboot et al 2003

Interstitial Lung Disease Unit

The number of patients with IPF being transplanted are increasing.

Interstitial Lung Disease Unit

The downside? High operative mortality (15% in first 3 months) No. of donor organs available
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