Coughing.
Shortness of breath.
These conditions don't seem overly serious but are the only
symptoms found in people with Idiopathic Pulmonary Fibrosis.
Idiopathic Pulmonary Fibrosis or IPF
is a progressive disease caused by scarring of lung tissue.
IPF is an interstitial lung disease,
meaning that it affect the tissue and space around the
alveoli,
the air sacs of the lung.
Damage to the alveoli will
interfere and reduce gas exchange with the blood preventing oxygen
deliveries to other parts of the body area of the body.
Disorder the cause or causes of IPF is unknown.
However,
Studies have identified numerous factors that may increase one's risk
of developing IP.
This includes genetic predisposition and possibly environmental triggers
such as cigarette smoke.
One way or another, individuals with IPF will experience
damage to the lungs and their body undergoes
excessive wound
healing, making the lungs thickened, stiff and heavily scarred.
Normally when the lungs are injured,
the immune system recruits many styles to the side of
damage,
including a first offense line,
called macrophages.
Macrophages are immune cells that prevent further damage and infection
by removing dead cells, debris and infectious agents by
engulfing.
They also dry the body's immune response by releasing chemical
signals called cytokine.s
The cytokines will signal fibroblast cells to begin with healing
in the lungs.
Fibroblast ourselves building the scaffolds of all organ.
They do this by releasing of structural protein called collagen
and other ECM proteins to re-establish,
the structure of the connective tissue,
and the context of tissue damage fibroblasts will transform
into myofibroblast to contract and close up the wound and
IPF fibroblasts and myofibroblast tend to remain active
and proliferate, forming cells,
and clusters known as fibroblastic foci.
The accumulation of these cells means ongoing production of ECM
component,
which is why the lungs of IPF patients become scarred
and stiff. To diagnose IPF,
your doctor will further collect information about your medical history,
to rule out other possible condition,
and determine potential causes of your symptoms of physical exams
performed using a stethoscope.
It
is estimated that 15,000
Canadians have IPF. In general,
there is no geographic
distinction for incidence of IPF and cases are reported globally
in both rural and urban areas.
IPF tends to affect individuals above the age of 55
and is more prevalent in men than women.
Currently.
There is no cure for IPF and patients usually die
after 2 to 6 years. In October 2014, two new
drugs, Ofev and Esbriet were approved by the FDA to treat
IPF.
It is not fully understood how these drugs work but
both drugs have been seen to slow the progression of
tissue scarring.
Lung transplants are also available for patients with more severe
physiological deterioration,
but requires patience to register on a waitlist,
and wait-time, They can take more than two years. Early
death may be due to infection, rejection or heart failure.
Patients can also be given supplemental oxygen to combat hypoxia,
or the lack of oxygen delivered to your body due
to lung damage.
Finally,
doctors,
encourage patients to be involved.
In clinical trials,
for new drug treatments,
being research,
for more information on research about IPF.
Visit Doctor,
Martin Kolb's
lab website from McMaster University.
You can also learn more at
www.demystifyingmedicine.ca