Treating Pulmonary Fibrosis

Treating Pulmonary Fibrosis
Dr Stephen M. Bianchi

‘Mechanics’ of breathing
Airways as conducting and clearing system (bronchi) O2 O2 O2 O2 Adequate volume of gas movement Gas exchange at alveoli (leaves) Cardiac/Heart Output Efficient/fit muscles O2 Pulmonary blood vessels

Why do I get breathless? O2 O2 O2 Irritated widened airways
Increased mucus O2 O2 Crumpled lungs Thick ‘leaves’ Bad heart Bad muscles O2 Thick tubes

? Things to ‘fix’ Crumpled lungs & reduced gas exchange
Drugs to slow it’s progression; medications to reduce impact; oxygen Increased mucus – Medicines to make sputum clearance easier & physiotherapy to help with clearance exercises Thicker pipes and bad heart Drugs to improve cardiac function and reduce lung blood vessel pressure Bad muscles Pulmonary rehabilitation Multi-facetted care

Slowing the fibrosis; treating symptoms
Antifibrotic Drugs Pirfenidone Nintedanib Both ? Symptomatic treatments Fans Lorazepam Oromorph/opiates Mucolytics? Chest clearance? Treating cough Reflux? Cough suppression Oxygen therapy Concentrators Ambulatory Pulmonary Rehabilitation Hospital courses Local courses Maintenance courses Heart and blood vessel treatments Optimise cardiac status (comorbidity) Treat high pulmonary blood pressure Psychological support (pt & family) Patient support groups Advanced care planning Clinical research Transplantation

IFIGENIA (2005) Control/Placebo = Pred/Aza vs Pred/Aza/NAC

Triple therapy abandoned
PANTHER-IPF (2012) Nil vs Pred/Aza/NAC Triple therapy abandoned

? NAC alone NAC monotherapy no survival benefit; no improvement in lung function. ? Palliative benefit (mucolytic) Consider carbocysteine ? Some responders – lung function. Possible genetic driver (Muc5B)

Balancing treatment effects
Benefit Reduction of progression of disease by approximately 50% compared to no treatment based on group trial data No promises that this works in an individual patient Cost Medication burden (at least 2-9 tablets per day) Side effects – in at least 40-80%; rare for nil Treatments don’t reverse disease Heavily medicalised life – monitoring/medication Financial

Symptomatic treatments Fans Lorazepam Oromorph/opiates Antifibrotic Drugs Pirfenidone Nintedanib Both ? Mucolytics? Chest clearance? Treating cough Reflux? Cough suppression Oxygen therapy Concentrators Ambulatory Pulmonary Rehabilitation Hospital courses Local courses Maintenance courses Heart and blood vessel treatments Optimise cardiac status (comorbidity) Treat high pulmonary blood pressure Psychological support (pt & family) Patient support groups Advanced care planning Clinical research Transplantation

Looking outside the lungs
IPF is a condition found more commonly in smokers or ex-smokers IPF is a condition found more commonly in older persons Consider other diseases related to smoking and age Most common are heart disease (ischaemic) & emphysema (COPD) IPF affects pulmonary blood vessels and can lead to pulmonary hypertension This seems more common in those with other lung diseases e.g. emphysema

Treatment options Optimise other lung diseases
Stop smoking Inhaler therapies Pulmonary rehabilitation Screen for heart disease and pulmonary hypertension (ECHO) Treat ‘left’ heart disease Consider pulmonary hypertension treatments Sildenafil/bosentan INSTAGE study (nintedanib/sildenafil) Oxygen therapy

Oxygen Little/No good evidence
BTS guidelines based around evidence from COPD Some debate amongst clinicians Number driven treatment Only give LTOT if PaO2 <7.3kPa and treat to PaO2>8kPa Only give AOT if demonstrate desat on 6MWT and improved oxygenation and walking distance on oxygen Only give POT if sats <94% Symptom driven treatment No such thing as LTOT and AOT All treatment is POT Give to anyone who is symptomatic Treatment success is if patient feels benefit in self defined QoL

Pulmonary rehabilitation
Little/No good evidence BTS guidelines based around evidence from COPD Some debate amongst clinicians Standards of care suggest that all patients with IPF should be referred to a PR programme if they wish. Unclear what a PR course for IPF should involve Ideally in a convenient local location. Usually 8-10 weeks. Maintenance programme Aims? Maintaining activity; promoting activity; doing activity differently Oxygen as a supportive measure

Treating symptoms directly
Breathlessness Fans (air movement) esp at night, hot weather Position (avoid lying flat) Rapid breathing/dysfunction/panic – lorazepam Sensation of breathlessness – oromorph/opiate Staged exercise (pulmonary rehab) Chest clearance Cough Treat reflux if symptoms (and maybe even if no symptoms) – upcoming research! Codeine linctus/tabs/oromorph/morphine Thalidomide Can be more challenging that other therapies

Ensuring a persons wishes are respected
IPF is a serious condition and for many will be the condition that leads to the end of their life Frank, honest but kind discussions with patients and families about resuscitation, preferred place of care, treatments that are appropriate and acceptable (e.g. critical care) Open discussion about the value of palliative care team involvement and the changing face of palliative care – Clifton House, Sheffield. Patient support groups – supporting each other, sharing experiences, sharing coping strategies and ‘tricks of the trade’. Forming different relationships with health care providers, charities, ‘non-medical’ activities

Adult Lung Transplants Number of Transplants by Year and Procedure Type
NOTE: This figure includes only the adult lung transplants that are reported to the ISHLT Transplant Registry. As such, this should not be construed as representing changes in the number of adult lung transplants performed worldwide. 2017 JHLT Oct; 36(10):

Adult Lung Transplants Distribution of Procedure Type for Major Indications by Year
Year of TX A1ATD COPD CF IIP ILD-not IIP Double (%) Single (%) 2001 59.9 40.1 30.2 69.8 94.5 5.5 31.6 68.4 36.2 63.8 2002 57.0 43.0 39.1 60.9 97.1 2.9 35.2 64.8 54.2 45.8 2003 66.4 33.6 43.2 56.8 96.1 3.9 42.9 57.1 44.1 55.9 2004 74.8 25.2 45.6 54.4 96.5 3.5 45.1 54.9 59.4 40.6 2005 76.9 23.1 49.5 50.5 97.5 2.5 46.8 53.2 57.4 42.6 2006 71.8 28.2 58.4 41.6 98.5 1.5 47.9 52.1 69.1 30.9 2007 82.5 17.5 63.5 36.5 97.9 2.1 49.1 50.9 64.5 35.5 2008 79.8 20.2 65.4 34.6 98.8 1.2 52.4 47.6 62.3 37.7 2009 87.2 12.8 99.8 0.2 52.0 48.0 64.7 35.3 2010 85.8 14.2 70.3 29.7 99.6 0.4 56.0 44.0 65.8 34.2 2011 89.9 10.1 73.3 26.7 69.4 30.6 2012 91.5 8.5 74.4 25.6 98.3 1.7 60.2 39.8 70.0 30.0 2013 89.8 10.2 76.5 23.5 99.2 0.8 59.3 40.7 67.8 32.2 2014 87.1 12.9 79.4 20.6 61.6 38.4 67.4 32.6 2015 93.2 6.8 77.2 22.8 98.9 1.1 62.0 38.0 73.8 26.2 2017 JHLT Oct; 36(10):

Adult Lung Transplants Kaplan-Meier Survival by Diagnosis
(Transplants: January 1990 – June 2015) Median survival (years): A1ATD: 6.7; CF: 9.2; COPD: 5.8; IIP: 4.9; ILD-not IIP: 6.0; Retransplant: 2.9 Survival was calculated using the Kaplan-Meier method, which incorporates information from all transplants for whom any follow-up has been provided. Since many patients are still alive and some patients have been lost to follow-up, the survival rates are estimates rather than exact rates because the time of death is not known for all patients. The median survival is the estimated time point at which 50% of all of the recipients have died. Survival rates were compared using the log-rank test statistic. Adjustments for multiple comparisons were done using Scheffe’s method. Results of log-rank test should be interpreted with caution when curves cross. Only pair-wise comparisons statistically significant at p < 0.05 are shown on the slide. All pair-wise comparisons were significant at p < 0.05 except A1ATD vs. ILD-non IIP and COPD vs. ILD-non IIP 2017 JHLT Oct; 36(10):

Adult Lung Transplants (2003-6/2015)
Recipient age Risk Factors For 1 Year Mortality with 95% Confidence Limits Adult Lung Transplants (2003-6/2015) p < Multivariable analysis was performed using a proportional hazards model censoring all patients at 1 year. Continuous factors were fit using a restricted cubic spline. Analyses were limited to transplants having essentially complete information regarding risk factors. 2017 (N = 38,983) JHLT Oct; 36(10):

Transplantation Referral when gas transfer <40% Age <60 (to 65)
No significant other major disease IHD reviewed (coronary angio) No kidney impairment Bone density scan BMI <30 Pred <10 Visit to transplant centre for assessment of suitability, in patient detailed assessment including pychosocial aspects, listing and waiting. High physical and psychological burden. Not everyone listed gets a transplant

Antifibrotic Drugs Pirfenidone Nintedanib Both ? Symptomatic treatments Fans Lorazepam Oromorph/opiates Mucolytics? Chest clearance? Treating cough Reflux? Cough suppression Oxygen therapy Concentrators Ambulatory Pulmonary Rehabilitation Hospital courses Local courses Maintenance courses Heart and blood vessel treatments Optimise cardiac status (comorbidity) Treat high pulmonary blood pressure Psychological support (pt & family) Patient support groups Advanced care planning Clinical research Transplantation

Treating Pulmonary Fibrosis

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