1. Dealing with Idiopathic Pulmonary Fibrosis (IPF)
Webinar for patients with IPF

2. Declaration of interest
Dr. Marlies Wijsenbeek
Dr. Marlies Wijsenbeek has received speaker honoraria, advisory board fees and unrestricted research grants from Boehringer Ingelheim, Intermune and Hoffmann-La Roche. All fees were paid to her institution.
Ron Flewett
Ron Flewett has received speaker honoraria and travel grants from Roche Pharma and Boehringer Ingelheim.
The webinar is supported by Boehringer Ingelheim.

3. A person living with IPF
Today‘s speakers
Dr. Marlies Wijsenbeek
Pulmonologist, MD, PhD
Centre of Interstitial and Vascular Lung Diseases Erasmus Medical Centre, University of Rotterdam,
The Netherlands
Ron Flewett
A person living with IPF

4. How does IPF progress?

5. How does IPF progress?
Early IPF
Alveoli
In some patients IPF gets worse quickly and in others the disease evolves slowly
New medications might help slow the disease progression
If you notice any change in symptoms, don’t wait until your next appointment. Contact your doctor right away
Leathery tissue
Small blood vessel
Advanced IPF
Alveoli
Leathery tissue
Small blood vessel
Kim DS, et al. Proc Am Thorac Soc 2006;3:
Ley B, et al. Am J Respir Crit Care Med 2011;183:
Raghu G, et al. Am J Respir Crit Care Med 2011;183:

6. If you notice any changes, contact your doctor right away.
How does IPF progress?
As IPF progresses and lung damage becomes worse, some people have difficulty breathing during everyday tasks
Some people feel their difficulty for breathing worsens quite rapidly in weeks, months or years, while others have periods of stability (symptoms remain the same).
IPF may also include periods of acute deterioration in respiratory function, which are termed “acute exacerbations of IPF” when a cause cannot be identified
Notice a change in your breathing or symptoms?
Could it be a cold or infection?
If you notice any changes, contact your doctor right away.
Kim DS, et al. Proc Am Thorac Soc 2006;3:
Ley B, et al. Am J Respir Crit Care Med 2011;183:

7. What is an acute exacerbation of IPF?
If there is a sudden, severe worsening of symptoms (or lung function), it can be an “acute exacerbation of IPF.”
Acute
sudden onset
Exacerbation
considerable or rapid worsening
Collard HR, et al. Respir Med 2007:101:
Collard HR, et al. Am J Respir Crit Care Med 2016;194:265–75

8. Is it an acute exacerbation of IPF?
Fever or flu-like symptoms .
Like body aches
Coughing
Difficulty breathing
A cough that gets worse
It becomes significantly more difficult to breathe than usual and it doesn’t seem to get better
If you start to feel a worsening of symptoms or shortness of breath, contact the doctor right away
Collard HR, et al. Respir Med 2007:101:
Collard HR, et al. Am J Respir Crit Care Med 2016;194:265–75

9. Acute exacerbations of IPF
To diagnose an acute exacerbation, the doctor may perform different tests and/or HRCT to see if there is additional scarring on the lungs
An acute exacerbation can lead to hospitalization and may impact life expectancy.
Collard HR, et al. Respir Med 2007:101:
Collard HR, et al. Am J Respir Crit Care Med 2016;194:265–75

10. How could the risk of having an acute exacerbation of IPF be reduced?
Take measures to prevent respiratory infections (e.g. hand washing)
Keep up-to-date on recommended vaccinations, e.g. against influenza and pneumococcus
Take antiacid medications if prescribed by your physician
Avoid air pollution
Richeldi L. Ann Am Thorac Soc 2015;12(Suppl 2):S181–5; 2.
Luppi F, et al. Intern Emerg Med 2015;10:401–11

11. How can patients manage chronic cough?
A chronic, dry, hacking cough is a common symptom of IPF
May be embarrassing for patients
Other diseases may contribute to make worse the cough (e.g. Gastro Esophageal Reflux)
Discuss treatment options that could relieve/alleviate chronic cough with your doctor
No relief after coughing
Occurs when talking for long periods
Duck A, et al. J Adv Nurs 2015;71:1055–65
Lee JS, et al. Curr Opin Pulm Med 2011;17:348–54
Swigris JJ, et al. Health Qual Life Outcomes 2005;3:61
British Lung Foundation. 9 things people with lung disease want you to know. Available at (Accessed Dec 2016)

12. How can IPF be treated?

13. Raghu G, et al. Am J Respir Crit Care Med 2011;183:788-824.
How can IPF be treated?
Talk to your doctor about the treatment options that are best for you.
Oxygen Therapy
Medicine
Rehabilitation
Lung transplant
Raghu G, et al. Am J Respir Crit Care Med 2011;183:

14. Raghu G, et al. Am J Respir Crit Care Med 2011;183:788-824.
How can IPF be treated?
The lung scarring (fibrosis) in IPF is permanent.
Various therapies can help to alleviate symptoms and slow down disease progression.
Treatment decisions for IPF are highly personal for each patient and may be affected by additional factors such as other diseases the person may have and the history of acute exacerbations.
Talk to your doctor to determine the best IPF therapy for you
Raghu G, et al. Am J Respir Crit Care Med 2011;183:

15. A B C D E The ABCDE of IPF care Assess the needs
Backup and information for the patient
to help him make decision together with the doctor C
Give comfort
Alleviate symptoms and treat the comorbidities D
Disease modifying drugs E
Talk about issues that concern end of life

16. Raghu G, et al. Am J Respir Crit Care Med 2011;183:788-824.
How can IPF be treated?
Therapeutic agents
Doctors may prescribe an anti-fibrotic medicine.
Pulmonary rehabilitation
Involves exercise like physical training and aerobic conditioning, as well as nutritional guidance and education about managing IPF.
Oxygen therapy
Provides extra oxygen, typically through a nasal tube connected to a portable oxygen container.
Your doctor will determine if you need oxygen and how often you should use it.
Raghu G, et al. Am J Respir Crit Care Med 2011;183:

17. How can IPF be treated? Lung transplantation
• The damaged lungs are replaced with those from a donor.
• May be required if the IPF is quickly worsening or very severe.
• A lung transplant is a major intervention and has its own risk, but could improve your quality of life and help you live longer.
• Medicines will be required for the rest of your life to reduce the risk of organ rejection.
• Not every patient is a lung transplant candidate, this will need to be assessed with the treating physician.
Orens JB, et al. J Heart Lung Transplant 2006;25:
George TJ, et al. Arch Surg 2011;146:
Mason DP, et al. Ann Thorac Society 2007;84:
Raghu G, et al. Am J Respir Crit Care Med 2011;183:

18. What are the potential benefits of oxygen therapy?
Reduced breathlessness
Reduced fatigue
Sustained ability to exercise
Sustained ability to perform everyday activities
Due to these benefits, the use of supplemental oxygen therapy is
strongly recommended in patients with IPF and low oxygen saturation (less than 88%)
in the blood at rest (resting hypoxemia).
Managing IPF symptoms. Learn about oxygen therapy. Available at: (Accessed Dec 2016)
GB Healthwatch. Oxygen Therapy. Available at (Accessed Dec 2016)
Raghu G, et al. Am J Respir Crit Care Med 2011;183:788–824;
Raghu G, et al. Am J Respir Crit Care Med 2015;192:e3–e19

19. Why is pulmonary rehabilitation important for patients with IPF?
Feeling breathless
Patient fearful that activity makes them breathless
Patient avoids activity that makes them breathless
Patient reduces levels of activity
Patient’s muscles become weaker
Weak muscles use more oxygen and are less efficient
The cycle of breathless-
ness
Pulmonary rehabilitation can help break the cycle of breathlessness in
patients with IPF
British Lung Foundation. Idiopathic Pulmonary Fibrosis. Disease-modifying treatments. Available at: (Accessed Dec 2016)

20. What can patients expect during pulmonary rehabilitation?
Physical exercise
Psychological support
Nutritional advice
Education about IPF
Social support network
Breathing exercises
Pulmonary rehabilitation may help manage breathing problems, improve patients’ fitness, coping skills, daily function and quality of life.
NHS Choices. Idiopathic pulmonary fibrosis. Available at: (Accessed Dec 2016)
British Lung Foundation. Exercise with a lung condition. Pulmonary rehabilitation. Available at:
(Accessed Dec 2016)
Garvey C, et al. J Thorac Dis 2013;5:343–8

21. Most pulmonary rehabilitation programmes only last a few weeks/months
How can beneficial effects of pulmonary rehabilitation be sustained long term?
Most pulmonary rehabilitation programmes only last a few weeks/months
To sustain the benefits achieved during pulmonary rehabilitation, patients are encouraged to continue with exercise, breathing exercises and healthy lifestyle changes they have learned even after completing rehabilitation programme.
Pulmonary Rehabilitation. National Heart Lung and Blood Institute. Available at: (Accessed Dec 2016)

22. Living with IPF

23. Stay in contact with peers
Living with IPF can be challenging at times and you may find yourself faced with changes in your daily life. Talking with others may be helpful: Visit LifewithIPF.com for a list of some helpful online resources.
Benefits of support groups
Share experiences and get helpful information
Talk openly about your concerns
Find emotional and mental support
Develop competence in coping with your disease
Finding your support group
Ask your doctor or health care provider for advice
Ask fellow patients for suggestions
Internet search
Contact national organisations and local community centres for suggestions
Check the local newspaper

24. Please take your time and explore LifewithIPF.com further!
Thank you for watching the webinar for patients with IPF “Dealing with Idiopathic Pulmonary Fibrosis (IPF)“ with Dr. Marlies Wijsenbeek from the Erasmus Medical Centre, University of Rotterdam, The Netherlands and Ron Flewett, a person living with IPF.
Please take your time and explore
LifewithIPF.com further!