Lung transplantation is an option for certain people with idiopathic pulmonary fibrosis (IPF). It is one form of treatment currently available for IPF, but not everyone is eligible, and finding a donor lung isn’t guaranteed. Lung transplants have been shown to improve both life expectancy and quality of life in people with IPF, but there are serious risks involved.

In order to qualify for a lung transplant, you must meet certain criteria. For one, you must have no other significant health problems, such as cancer; heart, liver, or kidney disease; or chronic incurable infections. This can be a challenge in people with IPF, as many suffer from other lung conditions, coronary artery disease, or other problems that make them ineligible to receive a transplant. Your doctor will be able to tell you if you are eligible for a transplant based on your overall health status.

In the past, individuals over the age of 65 rarely received transplants. However, because surgical techniques and results have improved, more centers are performing transplants in older patients.

Risks of Lung Transplantation

Lung transplantation is a highly risky procedure and there is no guarantee that it will be successful. Risks include:

Only your doctor can say whether or not you are a possible candidate for a lung transplant. Medical guidelines recommend that the option of lung transplant be brought up at the time IPF is diagnosed if the patient meets all the criteria for transplant. Before pursuing the option of a lung transplant, you should discuss all the potential risks and benefits with your doctor.

Waiting is Now Reduced for Eligible Patients

The new lung allocation system (LAS) used by the United Network for Organ Sharing (UNOS, www.unos.org) has set new standards for how they evaluate transplant candidates. LAS is now based on the severity of lung disease. As a result, wait times have been reduced considerably for patients with severe lung function issues, including people with IPF.

If you’ve been diagnosed with IPF, your doctor will likely use tests to track how the disease progresses over time. Knowing how your condition is changing over time can help you and your doctor make informed treatment decisions.

You can find a list below of some of the tests your doctor may use. Note that this is just for your information. Your doctor may not use all of these. They may even employ tests not listed here.

Medical Tests Used to Track the Progression of IPF

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You can also take part in tracking your IPF by keeping a record of your symptoms over time. Use our Symptom Tracker to document how you’re feeling each day to be shared with your doctor.

There are things you can do to help manage idiopathic pulmonary fibrosis (IPF) symptoms and try to sustain your ability to perform daily activities for as long as possible.

53% of patients and 55% of caregivers believe a better understanding of managing IPF symptoms can help improve overall well-being

The approaches used to manage the symptoms of IPF are designed to meet each patient’s unique needs. Every person’s medical history is different. In addition, people with IPF frequently suffer from other medical conditions. These other conditions may have an impact on the course of IPF. (See "Managing other conditions" below.)

It’s also important to remember that each patient experiences IPF differently, and while some people with IPF don’t live long after getting their diagnosis, others may live longer than the often-quoted averages. Working together, you and your doctor can develop a plan to help you manage your symptoms in an effort to sustain your ability to participate in daily activities for as long as possible. Common approaches to managing IPF symptoms are listed below.

Summary of Options for Managing IPF Symptoms

Managing Other Conditions

As mentioned above, it is common for people with IPF to also have other medical conditions (called “comorbidities”). These may include obesity, diabetes, pulmonary hypertension, obstructive sleep apnea, coronary artery disease, and emphysema.

These conditions will often require their own treatments and medicines. They may even have an impact on the course of IPF. Remember to always take your medicines as prescribed by your doctor.

If you have any questions about other health conditions you have, or the medicines you are taking for them, be sure to talk to your doctor.

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If you've been diagnosed with idiopathic pulmonary fibrosis (IPF), then you're sure to have a lot of questions. People with IPF can experience a variety of physical and emotional challenges throughout the day, ranging from fatigue to powerful emotions and frustration. Understanding how IPF affects you as an individual will help you to better manage your symptoms and anticipate what lies ahead. The good news? People with IPF can play a big role in managing their IPF symptoms.

Customizing a Plan That Works For You

Because IPF disease affects each person differently and progresses at different rates, prescribed treatment plans are tailored to meet each person’s individual needs.

Create a personal action plan and set daily goals

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Getting Involved in Your Own Treatment Makes a Difference

You are the biggest factor in managing your IPF treatment. As with all diseases, it is important to live a healthy lifestyle. Activities such as physical conditioning, breathing exercises, nutritional counseling, and anxiety, stress, and depression management may all be a part of your personal disease treatment plan your doctor may create for you.

There Are a Number of Things You Can Do to Help Manage Your IPF Symptoms:

Quit Smoking	If you're still smoking, the most important thing you can do is to seek help and stop smoking as soon as possible. Remember, additional potential benefits to quitting include: increased oxygen levels in your blood, lowered blood pressure, and improved circulation.
Eat Well	A healthy diet plays an essential role in your overall physical and mental health. A diet rich in nutrients and low in saturated fat, trans fat, cholesterol, salt, and added sugar, along with a healthy balance of fruits, vegetables, whole grains, lean meats, poultry, fish, beans, and fat-free or low-fat dairy products, will help you get the fuel you need throughout the day. Remember, eating several smaller meals (rather than a few large meals) may prevent feeling too full, which can make it hard to breathe.
Exercise	A lack of exercise can work against people who have IPF, as inactivity weakens the muscles, making even the simplest activities more difficult. Developing an active lifestyle is often key in decreasing ‘air hunger' and strengthening the muscles to make them more resistant to fatigue. Regular exercise can help you develop the energy you need to accomplish daily tasks with less shortness of breath, and also help you to ease feelings of anxiety and depression.
Oxygen Therapy	Supplemental oxygen can make a substantial difference in a person’s ability to manage many of the lifestyle-altering effects that can accompany IPF. Oxygen therapy may provide the additional oxygen the body needs to help people with IPF maintain an active lifestyle, and can also decrease shortness of breath and the additional strain on the heart due to low oxygen saturation levels.
Get Rest	Getting at least eight hours of sleep every night can boost your overall sense of well-being. Rest can also help you deal with the stress of living with IPF.
Connect with Others	Whether it's friends and family or support and advocacy groups, staying connected to others who can provide good company and a positive outlet for what you're going through can make a powerful difference in adding meaning to your life and helping you cope with living with IPF.
Manage Emotions	Psychological and spiritual support is often an important factor in keeping a positive outlook, reducing fears, and enhancing the daily lives of people with IPF. Keeping a positive attitude and staying relaxed may conserve valuable oxygen by avoiding its excessive use that can come with tension.

Create a personal action plan and set daily goals

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Pulmonary rehabilitation (or “rehab”) is a treatment that covers many different approaches. The goals are to decrease the severity of breathlessness as much as possible during daily activity and to increase energy and exercise levels in general. Pulmonary rehab is recommended for patients with idiopathic pulmonary fibrosis (IPF), but most studies of its effectiveness have been done in patients with chronic obstructive pulmonary disease (COPD). Your doctor may prescribe pulmonary rehab as an addition to other types of care. Most, but not every patient is a candidate. It’s also important to understand that pulmonary rehab has not been shown to prevent the loss of lung function that occurs over time with IPF.

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“If pulmonary rehab has not been recommended or discussed with your physician, you may want to ask your physician about the therapy. It is extremely important to participate in pulmonary rehab if recommended by your doctor.”

Trina Limberg, Director of Pulmonary Rehabilitation at UC SD Medical Center

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Pulmonary rehab includes activities such as physical conditioning, exercise training, and breathing exercises. It may also include anxiety, stress, and depression management, as well as nutritional counseling, education, and other programs.

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“Your rehabilitation therapist will be able to help you figure out a solution that works best for you, as well as answer any questions you may have.”

Chris Garvey, FNP, MSN, MPA, MAACVPR University of California San Francisco

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The Possible Benefits of Pulmonary Rehab

As mentioned above, pulmonary rehab has been studied as a treatment in several different lung diseases and shown to have benefits for many patients who use it. Although there are not as much data on pulmonary rehab for people with IPF, a number of studies have shown it to provide benefits in IPF. These include:

Pulmonary rehab has been shown to be safe in people with IPF. There are few risks associated with pulmonary rehab that are generally related to the exercise part of the program. As with any physical training activity, there may be a very slight increase in muscle soreness. Also, physical activity can increase the risk of chest pain or arrhythmia (irregular heartbeat). Working in collaboration, your rehab specialist and your doctor will determine how much additional oxygen you may need as your exercise program is developed.

Although leading medical guidelines recommend that most patients with IPF receive pulmonary rehab, it may not be right for everyone. You should discuss the option of pulmonary rehab with your doctor to see if your condition allows it and if it might be of benefit to you.

A Team Effort

If your doctor prescribes pulmonary rehab, you should know it involves working with a team of healthcare providers. This team may include nurses, respiratory therapists, exercise physiologists, physical therapists, social workers, dieticians, and others. It may also include several exercises and lifestyle changes at home. Working closely with your treatment team will help you to know what is expected of you.