What is Idiopathic pulmonary fibrosis?


The following information is not based on any particular scientific fact but rather on Robert’s experiences with the disease, from his understanding thru info searches, and from personal communications with medical staff.

Idiopathic Pulmonary Fibrosis (IPF)

In December of this year (2013), I will be 62 years of age. Up until November of 2012, when I was first diagnosed with the disease; I had never heard the name “Idiopathic Pulmonary Fibrosis (denoted by the acronym IPF)”. When my doctor gave me my initial diagnosis, he told me “this is not good news”. My first knee jerk thoughts were that this long worded disease sounded like it was very specifically defined meaning that the medical community had a specific and treatable cure for it. My thoughts were wrong.

In breaking apart the words of the name; idiopathic refers to an unknown origin or cause, pulmonary refers to the lungs, and fibrosis refers to scar tissue. In other words it is a disease of an unknown cause that creates scar tissue in the lungs. IPF is more specifically defined by many international lung societies as “a specific form of chronic progressive fibrosing interstitial pneumonia of an unknown cause”. As I understand this the disease began at some point in time and is recurring from time to time at undeterminable and inconsistent rates creating scar tissue from pneumonia (inflammation, not bacterial pneumonia) in the interstitium (the space around the alveoli). The alveoli are tiny sac like structures in the lungs that are essential to the complex exchange of gases in our bloodstream; carbon dioxide leaves the bloodstream and oxygen enters the bloodstream. Over time the scar tissue displaces the soft moist pliable interstitium and alveoli with a thickened stiff leather-like tissue that alters the functions of the lungs as a whole. Breathing becomes harder and organs receive less oxygen as the body is exerted even to the point that additional sources of oxygen are required. The end results are a loss of total volume and function of the lungs causing the brain, the heart, and other organs to become negatively impacted. IPF is terminal; it has no cure and there are currently no effective medications to alleviate or alter symptoms of the disease.

According to the Pulmonary Fibrosis Foundation (http://pulmonaryfibrosisfoundation.org) ; In the US, IPF affects between 132,000-200,000 persons. Approximately 50,000 new cases are diagnosed each year and as many as 40,000 Americans die from IPF each year.

Some common symptoms are:

  • Shortness of breath
  • Clubbing of fingertips- a condition due to lack of oxygen affects the flesh under the fingernails causing the nails to curve downward. This condition is not specific to IPF.
  • Chronic, hacking dry cough
  • Fatigue, weakness
  • Discomfort in Chest
  • Loss of appetite
  • Unexplained weight loss

The symptoms that I am currently exhibiting are shortness of breath when I exert myself or do outside activities. When I work outside, I will wear an oxygen backpack to insure that my oxygen stays 92% or above. Some light headedness occurs when I over exert and some fatigue. I also have some discomfort in the chest which may be either from the biopsy or the disease or both. I also have the crackles, a sound that can be heard with a stethoscope on the back in the lower area of the lungs. I was told that this sound is the results of the fibrous stiffened lung tissue rubbing together when I breath. Some say it sounds like Rice Krispies when you pour milk over them. others say it's like the sound made when wading up saran wrap in your hand. My wife said she can hear it with the unaided ear by placing her head on my back. I also have a constant feeling of phelgm in my throat with an urge to constantly clear it. If I try to cough it is a non productive, dry wasted cough. To offset this feeling, I constantly chew gum and use a slight clearing of the throat that seems to work.