Treatment of Idiopathic pulmonary fibrosis and Transplantation
Idiopathic pulmonary fibrosis or IPF is a disease of scarring in the lungs and it’s called idiopathic
Because we don’t fully understand how it occurs or whether the processes that accelerated or slow it down and for a long time there hasn’t been any effective treatment from a medical standpoint.
People with IPF usually present with an abnormal x-ray, or they may come in with cough or more shortness of breath, and they usually have a CT that shows some degree of scarring in their lungs
Patience may have what we call hypoxemia that just means low oxygen levels in the blood and that is treated with supplemental oxygen and the goal is to maintain an acceptable oxygen level in your blood when you’re sitting at rest
But with the “improve quality” activity in general, most people will benefit to a certain degree from pulmonary rehabilitation and rehabilitation program.
The idea is to get the most out of the lungs that you have,one of the problems with chronic obstructive “lung disease” is that people will become progressively more debilitated, because they simply sit around.
Because they get short of “breathing problems” due to “chronic obstructive pulmonary” respiratory so just even that anticipation of exercise is a big barrier to actually participating any sort of “rehabilitation programs” activity together with some “pulmonary rehabilitation toolkit”
There are several medical “exercise education” therapies that have been used in the past with different levels of success, so some patients may end up on a small amount of steroids,while others patients are treated with n-acetylcysteine or mucomyst which is an antioxidant therapy.
So recently in late 2014 the fda approved two new drug therapies specifically for treatment of IPF, the two drugs are prevented own or esperan and in tetons it oro PHEV and both of these drugs are designed to slow down the rate at which chronic lung function is lost due to scarring.
So the exciting thing about these two new drugs is that for the first time we have a medical therapy that slows down the rate at which pulmonary fibrosis progresses.
However they don’t reverse the fibrosis that’s already occurred and in some patients if their diseases advanced and or progressive despite medical therapy then lung transplantation may be an option.
Lung transplant is really a life changing event for patients because they are now completely dependent on medications to prevent rejection, and they can be more susceptible to infection because of these anti rejection medications so long-term, those two complications rejection and infection are what we struggle with.
We usually follow patients very closely especially with into that first year after transplantation because that’s where a lot of the complications tend to arise and so this from a patient’s perspective.
They can expect frequent visits at the clinic, frequent monitoring of their lung function
and lab tests and also a very intensive obstructive pulmonary rehabilitation program disease rehab program that’s designed to help them recover from the transplant and get back on their lungs and due to this, “quality life” has been cut off their agenda.