Idiopathic pulmonary fibrosis (or IPF) is characterized as an interstitial lung disease that develops due to unknown causes. With roughly 50,000 new diagnosis yearly, IPF, impacts both male and female patients in their middle and older ages, typically between 50- and 70-years of age. Like other forms of pulmonary fibrosis (PF), IPF causes the gradual scarring, thickening, and stiffening of the deep lung tissues. The formation of scar tissues is what’s known as “fibrosis”, which affects patients at different rates. However, the average patient lives between 3 to 5 years following an official IPF diagnosis.

Following an IPF diagnosis, medical professionals aim to slow the progress of the disease and improve life expectancy and quality of life through prescription medication. However, early diagnosis is key for the best chance at treating and extending lifespan as many patients aren’t  properly diagnosed until serious lung damage is already done.

These common symptoms of idiopathic pulmonary fibrosis tend to develop gradually over time, and include:

1. Shortness of breath

Labored breathing (or dyspnea) is the most telltale sign of IPF is shortness of breath that gradually gets worse with time or physical activity, until breathing becomes difficult and disabling even when the patient is at rest.

2. Fatigue

Extreme tiredness often occurs gradually and without cause, and can be explained as general malaise that affects the entire body (i.e., stiffness and aching joints).

3. Chronic cough

IPF shortness of breath is often accompanied by a stubborn, hacking, and uncontrolled cough that doesn’t improve over time.

4. Widening fingertips and toes
   Patients with idiopathic pulmonary fibrosis often suffer clubbing, which is the widening of the fingertips and toes due to excess fluid pooling at the ends of soft tissue in the fingers and toes.
5. Unexplained weight loss

IPF patients often describe gradual yet unexplained weight loss prior to diagnosis.

The scarring from IPF eventually impairs the ability of the lungs to transfer oxygen through the bloodstream, leading to oxygen deficiency of the organs and brain. The most common causes of death due to IPF are:

• Lung cancer
• Heart failure
• Pulmonary embolism
• Pneumonia
• Respiratory failure
• Pulmonary hypertension

Treatment options for IPF patients

Sadly, there is no cure for IPF. However health care providers aim to treat patients with methods meant to improve breathing and overall quality of life, including:

1. Pulmonary rehabilitation

Rehab for IPF patients takes many different forms—meditation and relaxation, breathing exercises, stress-relieving therapies, and healthy diet.

2. Oxygen therapy

Increasing oxygen flow in the bloodstream is essential for IPF patients. This means many patients are giving oxygen therapy via a mask worn while sleeping or exercising to decrease shortness of breath and make everyday life more comfortable.

3. Prescription medication

The most common drugs prescribed by doctors to slow down lung scarring and damage are Esbriet (pirfenidone) and Ofev (nintedanib).

4. Clinical trials

Scientific researchers are continuing to make strides as far as advanced treatments for IPF, which means patients may take part in clinical trials to test new IPF medications that aren’t yet available to the public.

5. Lung transplant

In severe and extremely fast progressing cases of IPF, doctors may recommend a lung transplant if it’s the patient fits the criteria and it’s the only hope to extend life.