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What is pulmonary fibrosis?
1
Pulmonary fibrosis (PF) is a condition in which lung tissue becomes scarred,
thickened, and stiff. The scarring is called fibrosis and makes it hard for
a person to breathe.
FIGURE A
How does pulmonary fibrosis affect the lungs?
1
The air sacs and blood vessels in the lungs work to deliver oxygen to the body.
In people with PF, the tissue inside and between the air sacs becomes scarred and stiff.
The scarring and stiffness make it hard for oxygen to pass through the walls of the
air sacs into the bloodstream.
As a result, the brain, heart, and other vital organs may not get the oxygen
they need to work properly.
What causes pulmonary fibrosis?
PF can be caused by many things, some of the
known causes of PF include:
Job-related and environmental factors. Being
exposed to toxins and pollutants over the long term
can damage the lungs. These toxins and pollutants
may include silica dust, asbestos fibers, grain dust,
and some bird and animal droppings.2,3,4
Radiation treatments. Radiation treatment for
lung or breast cancer may also damage the lungs
in some people.3
Certain medicines. Some chemotherapy medicines
To request a copy of the Pulmonary Fibrosis Patient
Information Guide, contact the PFF Patient Communication Center at 844.Talk PFF (844.825.5733) or
pcc@ pulmonaryfibrosis.org.
What is IPF?
IPF is a specific form of PF; it is also reported
to be the most common type of PF.2
to treat cancer, some heart medicines, and some
antibiotics are known to cause lung damage.1
Genetics may play a role since IPF may occur
in several members of a family.2
Certain medical conditions. Lung tissue can also
Some researchers have also suggested that
gastroesophageal reflux disease (GERD) may
have a role in the development of IPF.2
be damaged by conditions such as tuberculosis,
pneumonia, lupus, rheumatoid arthritis, sarcoidosis,
and scleroderma.3,5,6,7,8
Genetics. Genes can play a role in the development
of PF.2
When there is no known cause for PF, the disease
is called idiopathic pulmonary fibrosis, or IPF.1
For more information about the causes of PF,
visit www.pulmonaryfibrosis.org/causesofpf.
Who gets idiopathic pulmonary fibrosis?
If you have been diagnosed with IPF, you are
not alone.
As many as 132,000 people in the United States
have idiopathic pulmonary fibrosis.9
IPF is most common in people 50 years or older.2
FIGURE D
FIGURE B
O2
It is important for people living
with a form of IPF to work closely
with their health care providers
to find ways to try and preserve
their overall health.
inside of
alveolus
alveolus wall
CO2
inside of capillary
FIGURE E
O2
inside of
alveolus
alveoli
alveolus wall
alveolus wall with IPF
capillary
CO2
FIGURE C
inside of capillary
How does idiopathic
pulmonary fibrosis make
you feel?
IPF symptoms develop
over time. The most common
symptoms of IPF are: 10
• Shortness of breath
• A dry, hacking cough
Other symptoms that may
occur over time include:
• Fast, shallow breathing
• Fatigue and weakness
• Aching muscles and joints
• Weight loss that cannot
be explained
• Clubbing (widening and
rounding) of the tips of
the fingers and toes
FIGURE A shows the location of
the lungs and airways in the body.
alveolus wall with IPF
FIGURE B shows a cross section
of a normal alveolar-capillary unit.
What can happen to your body
over time when you have idiopathic
pulmonary fibrosis?
IPF can progress slowly or quickly. It varies
from person to person and is hard to predict.2
In some people, IPF remains stable for years.
In others, symptoms get worse over time
and it becomes harder to breathe even when
you are not active.2
IPF can also lead to other medical problems.
As the disease gets worse, it can lead to respiratory (lung) failure, infections, high blood
pressure in the lungs (pulmonary hypertension),
and heart failure.3
FIGURE C shows a cross section
of an alveolar-capillary unit with
thickening of the alveolar wall.
FIGURE D shows normal oxygen
exchange across the alveolus wall.
FIGURE E shows how oxygen
exchange across the alveolus wall
is impaired by fibrosis.1
How are PF and IPF treated?
The treatments used for various forms of
PF will depend on its cause. Specifically, for IPF,
there are US Food and Drug Administration
(FDA) approved medications that can be used
to help patients affected by the disease. These
medications are taken orally.
Additionally, some treatments may help relieve
the symptoms of PF, and may help maintain
your activity.
These treatments include:
Oxygen therapy. Oxygen may be prescribed
if the level of oxygen in your blood gets too low.
Using oxygen can help make breathing easier
and help you try to stay active.
It can also prevent or reduce problems from low
oxygen levels in your blood, reduce the blood
pressure in your heart, improve your sleep, and
help you feel better.11
Pulmonary rehabilitation. Pulmonary rehabilita-
tion programs help people with PF. They focus
on physical exercise and breathing techniques.
They also provide nutritional counseling and
emotional support.12,13
Lung transplantation. For some people with PF,
lung transplantation may be an option.14
Another option to consider is to enroll in a
clinical trial. Your health care provider will talk
with you about your options and which steps
might be right for you.
Who treats pulmonary fibrosis?
Finding a health care provider who has
experience treating people with PF is important.
Pulmonologists (lung and breathing specialists)
familiar with PF can often be found at major
medical centers.
To find a medical center near you with
expertise in treating PF, contact the PFF Patient
Communication Center at 844.825.5733 or
+1 312.587.9272 (from outside the US) or visit
www.pulmonaryfibrosis.org.
Can you benefit from participating in a clinical trial?
A clinical trial is a research study to learn more about investigational
treatments or new ways to use existing treatments. Some clinical
trials are performed to see if a new medicine is safe and effective for
people to use. Other clinical trials compare existing treatments to
find out which one is better.15
The FDA and other regulatory agencies such as the European
Medicines Agency (EMA), have regulations and guidelines to make
sure people are provided with reliable information to help them
decide whether working with their doctor to join a clinical trial is
an appropriate option for them.16,17 Please be sure to talk to your
provider about all of your options.
Where can you find support?
IN-PERSON SUPPORT GROUPS
Participating in a support group may help individuals with PF and
family members better manage the challenges of living with PF.3
To find a support group near you, visit www.pulmonaryfibrosis.org/
life-with-pf/support-groups.
If there is no support group in your area and you would like
to start one, contact the PFF Patient Communication Center at
844.Talk PFF (844.825.5733) or +1 312.587.9272 (from outside
the US) or visit www.pulmonaryfibrosis.org/life-with-pf/
support-groups/support-group-leader-network.
ONLINE SUPPORT GROUPS
Inspire. These online support groups are for people with PF
and their caregivers. Learn more about the Pulmonary Fibrosis
Foundation’s Inspire community at www.inspire.com/partners/
pulmonary-fibrosisfoundation.
RareConnect. This international online community is designed
to connect people with IPF who speak different languages. Learn
more about RareConnect at www.rareconnect.org/en/community/
idiopathicpulmonary-fibrosis.
How can you live better with pulmonary fibrosis?
Living with PF is not always easy. But there are some things that you can do to help manage
your condition and try to maintain your quality of life.18,19
TAKE AN ACTIVE ROLE WITH
CONSIDER MAKING SOME
YOUR CARE TEAM
LIFESTYLE CHANGES
Your care team includes your providers, your
supporters, and YOU. Being actively involved in
your treatment is important for individuals
with PF.
Stay active and keep in shape.
Work closely with your health care providers
and others on your care team.
Call your health care provider if you do not feel
well or notice any unusual symptoms.
Take all of your medications exactly as directed
by your health care provider.
Follow your health care provider’s dietary and
exercise recommendations.
Get your vaccinations. Respiratory (lung)
infections like the flu can make your PF symptoms worse.20 It is important to talk with your
doctor about getting a flu shot every year and be
up to date with your pneumonia vaccine.
Eat well and maintain a healthy body weight.
A dietitian can help you create a healthy eating
plan that works for you.
Get plenty of rest.
Stop tobacco use and avoid secondhand smoke.
Learn and practice relaxation techniques
to help manage stress.
Join a support group. Family members and
caregivers can join too.
Try to keep a positive attitude. If you feel
depressed or anxious, be sure to tell your provider
or another member of your care team.
Keep all of your medical appointments.
Speak up for yourself. Talk with your providers
about your questions or concerns.
Be prepared for your medical visits. Prepare a list
of questions to take to your appointments. Visit
www.pulmonaryfibrosis.org/yourappointment
for a list of questions.
Take notes at your appointments. Or have
a friend or family member come with you to
take notes.
Get support and stay informed. Go to educa-
tional meetings held by your local medical center,
join a support group, and connect with the
Pulmonary Fibrosis Foundation.
SUPPORT FOR YOU FROM
THE PULMONARY FIBROSIS FOUNDATION
The Pulmonary Fibrosis Foundation (PFF)
provides support for the entire PF community.
Our signature programs include:
• PFF Patient Communication Center
• Disease Education Materials
• International network of support groups
and online communities
• PFF Care Center Network and
the PFF Patient Registry
• PFF Summit
• PFF Ambassador Program
To learn more about the Pulmonary Fibrosis
Foundation contact the PFF Patient Communication
Center at 844.TalkPFF (844.825.5733) or
pcc @ pulmonaryfibrosis.org.
REFERENCES
1 NHLBI, NIH. What Is Idiopathic Pulmonary Fibrosis?
Accessed at: http://www.nhlbi.nih.gov/health/health-topics/
topics/ipf/. Accessed July 2013
2 Raghu G, Collard H, et al. An Official ATS / ERS /JRS / ALAT
Statement: Idiopathic Pulmonary Fibrosis: Evidence-based
Guidelines for Diagnosis and Management. Am J RespirCrit
Care Med. 2011; 183: 788–824, 2011
3 Schwarz MI and King TE. Interstitial Lung Disease, 5th
Edition. (Shelton, CT: People’s Medical Publishing HouseUSA). 2011.
4 Chan AL et al. Clinic Rev Allerg Immunol (2012) 43:69–83.
DOI 10.1007/s12016-011-8282
5 Dheda K et al. Lung Remodeling in Pulmonary Tuberculosis.
J Infect Dis. (2005) 192 (7): 1201–1210. doi:
10.1086/444545
6 Cheema GS, Quismorio FP Jr. Interstitial lung disease
in systemic lupus erythematosus. Curr Opin Pulm Med.
2000 Sep;6(5):424–9.
7 Tanoue LT. Pulmonary manifestations of rheumatoid arthritis.
Clin Chest Med. UID 9917959.
8 Le Pavec J et al. Scleroderma lung disease. Clin Rev
Allergy Immunol. 2011 Apr;40(2):104–16. doi: 10.1007/
s12016-009-8194-2
9 Raghu, G. Incidence and Prevalence of Idiopathic
Pulmonary Fibrosis. American Journal of Respiratory
and Critical Care Medicine, Vol. 174, No. 7 (2006),
pp. 810–816. doi: 10.1164/rccm.200602-163OC.
Available at: http://www.atsjournals.org/doi/full/10.1164/
rccm.200602-163OC. Accessed August 2013
10 Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis.
Orphanet J Rare Dis. 2008 Mar 26;3:8. doi: 10.1186/
1750-1172-3-8. Accessed at: http://www.ojrd.com/
content/3/1/8. Accessed July 2013
11 Wells AU. Interstitial lung disease guideline: the British
Thoracic Society in collaboration with the Thoracic Society
of Australia and New Zealand and the Irish Thoracic Society.
Thorax 2008;63(Suppl V):v1–v58. doi:10.1136/thx.
2008.101691
12 Jastrzebski D et al. Dyspnea And Quality Of Life In Patients
With Pulmonary Fibrosis After Six Weeks Of Respiratory
Rehabilitation. Journal Of Physiology And Pharmacology
2006, 57, Supp 4, 139 148
13 Spruit et al. An Official American Thoracic Society/European
Respiratory Society Statement: Key Concepts and Advances
in Pulmonary Rehabilitation. Am J Respir Crit Care Med
Vol 188, Iss. 8, pp e13–e64, October 15, 2013
14 International Guidelines for the Selection of Lung Transplant
Candidates: 2006 Update — A Consensus Report from the
ISHLT Pulmonary Scientific Council (Consensus Document).
J Heart Lung Transplant 2006;25(7):745–755
15 Learn About Clinical Studies. ClinicalTrials.gov.
Available at: http://clinicaltrials.gov/ct2/about-studies/learn.
Accessed November 2013
16 About FDA — What Does FDA do? FDA.gov. Available at:
http://www.fda.gov/AboutFDA/Transparency/Basics/
ucm194877.htm. Accessed November 2013
17 What we do. European Medicines Agency. Available at:
http://www.ema.europa.eu/ema/index.jsp?curl=pages/
about_us/general/general_content_000091.jsp&mid=
WC0b01ac0580028a42. Accessed November 2013
18 Florian J et al. Impact of pulmonary rehabilitation on quality
of life and functional capacity in patients on waiting lists
for lung transplantation. J Bras Pneumol. 2013 Jun;39(3):
349–56. doi: 10.1590/S1806-37132013000300012
19 Holland A, Hill C. Physical training for interstitial lung disease.
Cochrane Database Syst Rev. 2008 Oct 8;(4):CD006322.
doi:10.1002/14651858.CD006322.pub2
20 Rothberg MB et al. Complications of Viral Influenza.
The American Journal of Medicine (2008) 121, 258–264
ABOUT THE PULMONARY FIBROSIS FOUNDATION
The mission of the Pulmonary Fibrosis Foundation (PFF)
is to serve as the trusted resource for the pulmonary fibrosis (PF)
community by raising awareness, providing disease education,
and funding research.
PULMONARY FIBROSIS FOUNDATION
230 EAST OHIO STREET, SUITE 304
CHICAGO, ILLINOIS 60611-3201
PFF PATIENT COMMUNICATION CENTER
844.TalkPFF ( 844.825.5733)
+1 312.587.9272 (from outside the US)
www.pulmonaryfibrosis.org
© 2015 PULMONARY FIBROSIS FOUNDATION. ALL RIGHTS RESERVED.
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