Hi, folks, welcome to Demystifying Medicine
Channel. In this video,
We're going to have an overview on the
idiopathic pulmonary fibrosis disease or IPF
for short. And we want to discuss what do we
really know about this disease?
In this video, we will answer the questions,
including what is pulmonary fibrosis, how
does it occur, and basically what are the
possible symptoms and treatments associated
with this disease? Pulmonary fibrosis is a
chronic, restrictive fibrotic lung disease.
Well, I know it's a little bit confusing,
but in simpler words, pulmonary fibrosis is
an inflammation and a scarring to the lung
tissue. So far, there are more than 200
different types of this disorder, but a
variety of causes.
And basically the word idiopathic was
stemmed from the Greek language with the
meaning of unknown.
Therefore, the exact cause of this disease
is still unknown.
This chronic disease primarily occurs in
older adults within the age range of 40 to
70 years old and mostly in male smokers.
Doctors usually suspect individuals with
pulmonary fibrosis disease when they show
symptoms such as chronic dry cough,
shortness of breath.
Loss of appetite, tiredness, weight loss and
a diffuse pain in the chest.
In advanced stages of this disease, one can
experience a shortness of breath without
physical effort and bluish coloration of the
lips, nails and sometimes the whole skin.
Traditionally, doctors rated the diagnosis
as early, mild, advanced, or severe.
However, researchers are working to narrow
down the precise measurements of the disease
in order to better identify it.
The stages of IPF include stage one or mild
stage when the symptoms are not shown or are
presenting as mild cough or shortness of
breath. Stage two or moderate happens when
the symptoms has become severe enough that
the patient probably needs to use breathing
medications or oxygen machines.
And basically, the oxygen levels is low
throughout the whole body in advanced stage
or stage three after disease, the severity
of the symptoms with barely a low doctors to
treat the patient except a lung
transplantation. However, not everyone can
be a proper candidate for the long
transplantation procedure.
In a person with healthy lungs.
The gas exchange occurs in a way that we
inhale oxygen and exhale.
Carbon dioxide.
Are small air sacs within the lungs, which
are called alveoli, are surrounded by
important cells such as epithelium,
macrophages, and fibroblasts.
When oxygen is inhaled, it goes into the
bloodstream and gets carried by red blood
cells. When the small particles of the smoke
or air pollution get inhaled.
They can cause micro injuries to the alveoli
or epithelium cells.
And these injuries would trigger the cells
to produce inflammatory molecules.
Therefore, the inflammatory molecules would
cause a fibroblast cell, which are located
at the surrounding of the alveoli cells, to
do multiple differentiation.
And it would finally lead to damage or loss
of lungs, capillary surface area and gas
exchange units.
Consequently, it would result into poor gas
exchange and shortness of breath and other
complications associated with this disease.
It has been identified that genetics is an
important risk factor in the idiopathic
pulmonary fibrosis.
Usually when two or more members within a
family have IPF, it is called familial
pulmonary fibrosis.
However, family members can be secondary as
well, such as aunts, uncles, cousins and
etc. In some families with familial
pulmonary fibrosis or FPF.
Not every affected family member has IPF.
Some of them can be diagnosed with IPF and
others could have another type of
restrictive lung disease.
The average survival rate from the time of
diagnosis to the advanced stage of this
disease is about 3 to 5 years.
Currently, there are two drugs approved by
FDA for the treatment of this disease, which
include prevented and entered in it.
These medications would basically help the
patients to decrease the decline in their
breathing test results.
However, these medications would not
necessarily make the patients to feel
better, but they decrease the spread of the
breathing problems in IPF patients.
The side effects associated with these
medications include impacting liver
function, but not severe enough to lead to
major health problems.
However, it is recommended to perform blood
tests for liver function every month for the
duration of six months and
The other side effects for both medications
include GI side effects, which means the
digestive system doesn't feel right, and it
usually comes with the symptoms such as
nausea, vomiting and diarrhea.
Therefore, it is recommended to take the
pills with food and evenly spread the doses
throughout the day. Another side effect
associated with these medications are
photosensitivity rash, which are rashes that
appear with exposure to the direct sunlight.
Therefore, patients who take these
medications must be cautious while being in
the sun by wearing long sleeves, outfits,
sunglasses, hats and, more importantly,
sunscreen. Finally, I must say that despite
the fact that IPF is a chronic progressive
disease, but IPF patients can manage their
disease as fully as possible.
In order to do that, there are some steps to
take. Step one is to learn as much as
possible about IPF and their specific
diagnosis diagnosis by referring to
accredited medical resources.
The second step is having family members or
friends to attend their medical appointments
as they can be a second set of ears and help
the patient with their daily activities.
Step three and the most important step is to
remember that you are not alone and reach
out to your doctor or pulmonary fibrosis
support organizations for more help.
Thank you everyone for watching.
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