Understanding IPF

IPF is a rare and serious lung disease


How IPF affects the lungs

Normal lungs have no scarring and this means oxygen can pass easily from the environment into the air sacs of the lungs and easily pass into the bloodstream. This allows for oxygenation of the blood. In pulmonary fibrosis, the lungs lack elasticity and are stiff due to scarring or fibrosis. This scarring makes it harder for oxygen to pass through the alveoli (air sacs) and reduces the amount of oxygen that can be passed into the bloodstream. The fibrosis stiffens the lungs, reducing their size and capacity. This reduction in lung capacity is reflected in breathing tests, also known as pulmonary function tests, which may be used to diagnose or monitor pulmonary fibrosis progression. As the scarring or fibrosis progresses it may become harder to breathe and some patients may need oxygen to help them breathe.

  • IPF is progressive, which means that over time the scarring becomes more widespread and lung function declines. Doctors can’t predict how quickly this will happen. IPF progresses rapidly for some people and slowly for others

IPF permanently damages lung function

Currently, there is no cure for IPF, but there are treatment options that may help delay disease progression.

Why Lung Function Matters 

  • IPF is progressive. This means that the scarring caused by IPF will continue to happen, and lung function will continue to get worse
  • IPF is unpredictable. It is difficult to know how slowly or quickly IPF will progress. Each person’s experience is different
  • IPF is irreversible. Once there is scarring in the lungs, it cannot be reversed. Today, there is no cure for IPF, but there are treatment options that may slow its progression

How IPF impacts lung function

  • “Lung function” refers to:
    • How well the lungs can breathe in and out and exchange gases such as oxygen
    • Oxygen is vital to the body
  • IPF reduces lung function by causing permanent scarring around parts of the lungs. This scarring keeps the lungs from breathing in and transporting as much oxygen as they used to
  • It’s important to talk to your doctor about your lung function and how it impacts your body

How your doctor may test lung function

There are a few different types of tests that your doctor might do to check how your IPF is progressing. They include:

Pulmonary function tests

There are tests that measure lung function. Your doctor can give you more information. One test your doctor may do is a forced vital capacity (FVC) test:

  • FVC is a test that measures the amount of air you can exhale with force after you inhale as deeply as possible. It measures how well the lungs work
  • A device called a spirometer measures FVC. You take in a big breath and blow out as hard as you can to generate a reading
  • Your treatment team may check your FVC at every visit. If your FVC measurement goes down over time, it may mean that lung function is decreasing and IPF may be progressing
  • Remember that no matter what your test results say, both you and your healthcare team want what’s best for your health. You’re both working toward the same goal

Exercise tests

Your doctor may have you do physical activities. For example, one type of test looks at how far you can walk in a set amount of time. Another test checks your oxygen saturation while you walk. A third one measures how quickly you recover after walking.

Lung scans

There are scans your doctor can do to see what is happening inside your lungs. One type of scan is called an HRCT scan (High Resolution CT scan).

Since everyone is different, your doctor will decide which tests are best for measuring how your IPF is progressing. Ask your doctor about which tests may be right for you.

Excerpts from CHEST Foundation on how to improve your life:

Starting a New Medication

Specialty medications are different from regular prescriptions, as you may not pick them up from your local pharmacy. Specialty medication acquisitions require more steps than a regular prescription. The process is as follows:

  • The prescription is filled out on a form called a Referral. The physician will sign the form and it gets faxed to the manufacturers’ “Hub”. You may be asked to sign the form, as well.
  • Once the Hub receives the referral, they will verify your benefits with your current insurance. This helps them know which Specialty Pharmacy is in network with your insurance. The Hub will fax our office a Prior Authorization for the medication. Every Specialty Medication requires a Prior Authorization.
  • Prior Authorization is submitted to your insurance and they will review to see if it meets their criteria for approval. The process can take up to a couple of weeks depending on your insurance. If for some reason the insurance decides not to approve your medication, we will look to see why they made this decision and try to appeal this decision.
  • It is possible that your insurance will require a copay. In the case the copay is significant and you cannot afford the amount, you can ask special foundations for copay assistance. Non-Medicare patients can directly apply for assistance from the drug company manufacturing the drug. You will find more information in the Financial Assistance tab of this web page. Your physician’s office can also guide you in this matter. In spite of a sometimes hefty copay, through a variety of channels, patients usually are able to afford these specialty drugs- it does take some work by you to apply. You may receive phone calls during this time from the hub, the pharmacy, the drug company or assistance foundations to verify your income and assets. Please cooperate with them, they just want to help!
  • Once the medication is approved, the Specialty Pharmacy will be ready to ship it to you. You will receive a phone call from the Hub and/or from the Specialty Pharmacy. These will likely be 1-800 numbers. You have to answer the phone in order for them to be able to ship your medication to you. Please call them back. The medication will be mailed to your home address. Some medications require that a nurse from the Specialty Pharmacy visit you while you are taking the medication, your clinician will discuss this in detail if it applies to you.
  • You should work with all members of your Care Team during this process. This includes your physician, office nurse, Specialty Pharmacy staff (Pharmacist and Nurse).


Idiopathic Pulmonary Fibrosis is a serious condition. The medications designed to fight the disease typically show benefit slowly, over months. Only a portion of patients improve their lung function. In most it prevents further loss of lung and poor outcomes. The adverse effects (Side-effects) of these medications, however happen upfront. Your body typically will get used to them either with time, or with additional natural remedy or drugs. This may also take 2 to 3 months. It is necessary that you have patience, and talk to your doctor or nurses about dealing with them. Sometimes slowing the planned increase or cutting down on medication for a while will allow your body to adjust. Discuss matters with your doctors’ office before taking action on your own. Patience is a virtue!

Financial Assistance

Non-profit charitable assistance funds:

For Medicare and Medicaid patients, the following foundations may be able to assist with copay support. It may take some work by you to apply. Your physician’s office, the Hub or specialty pharmacy contacts may help you through this. (See Starting a New Medication Tab)

We maintain an updated list and verify if funding is available on a weekly basis.

Click here to see foundations.


FDA Approved Agents

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