13 Pulmonary Fibrosis Stage 4 Symptoms (and Finding Relief)

Pulmonary fibrosis is a condition in which the tissue of the lungs becomes damaged and scarred. Many things can lead to pulmonary fibrosis, although doctors often cannot identify a specific cause. 

If you’re living with a chronic lung disease like pulmonary fibrosis (PF)interstitial lung disease (ILD) or chronic obstructive pulmonary disease (COPD), you’ve likely heard your doctor talk about stages.

For people with COPD, the GOLD System and the BODE Index are often used to place COPD into stages, ranking it by disease severity.

However, the pulmonary fibrosis stages are not as well defined for people living with pulmonary fibrosis.

While there is no official or formal system for identifying stages, the medical community may divide pulmonary fibrosis into four stages:

  • Stage 1 – Mild
  • Stage 2 – Moderate
  • Stage 3 – Severe
  • Stage 4 – Very Severe

This pulmonary fibrosis staging system uses a patient’s forced vital capacity (FVC) score from a pulmonary function test to determine a patient’s pulmonary fibrosis stage.

Generally, a predicted FVC score of more than 75 percent is mild-stage pulmonary fibrosis. An FVC score of 50 percent to 75 percent is moderate-stage pulmonary fibrosis.

Severe-stage pulmonary fibrosis has an FVC score of 25 percent to 49 percent, and scores less than 25 percent are considered very severe.

People with severe or very severe pulmonary fibrosis may also be considered in end-stage pulmonary fibrosis.

Stage 4 pulmonary fibrosis is very painful, often requiring high-flow oxygen. While this can be a difficult adjustment, it’s not a death sentence. 

Many symptoms accompany stage 4 pulmonary fibrosis, but there are ways to find relief from these symptoms.

Here are 13 common pulmonary fibrosis stage 4 symptoms.

1. Chest Pain

Chest pain is a common side effect of end stage pulmonary fibrosis. The primary cause of this chest pain is frequent coughing, which can cause the muscles around your lungs to become sore, strained, and pulled. Coughing may be more severe in certain situations, like talking a lot or during exercise. 

How To Find Relief

Sometimes, you can find relief at home by managing your cough and taking over-the-counter pain medications such as ibuprofen. In some cases, your doctor may prescribe stronger pain medication.

2. Depression

Living with a chronic disease is complex, and many chronic disease patients experience depression at some point. For patients with stage 4 pulmonary fibrosis, shifting from portable oxygen to high-flow oxygen can trigger or increase anxiety and depression. 

Many of the symptoms of depression are similar to symptoms of stage 4 pulmonary fibrosis, such as weight loss, sleep disturbances, or fatigue. Symptoms that are more specific to depression include:

  • Apathy or numbness
  • Difficulty concentrating
  • Feeling worthless
  • Feelings of guilt
  • Loss of interest in typically enjoyable activities
  • Thoughts of death or suicide

How To Find Relief  

Depression is a multifaceted illness, and treatment options can vary from person to person. 

Speaking with a therapist, especially one trained to work with patients with chronic disease, can be beneficial. These therapists can often provide tools to help you process the complex feelings you may have about your condition and help you work through them and find a resolution.

In some cases, antidepressant medications may be helpful. There are many types of antidepressants, and you may not find success with the first one you try. 

Preventing yourself from becoming isolated can help prevent depression. Talk to your doctor about portable oxygen systems that can handle high-flow oxygen. Consider hosting friends and family at your home so you can access oxygen but still visit with the people you care about.

3. Increased Anxiety

Along with increased depression, stage 4 pulmonary fibrosis can often lead to increased anxiety. Like depression, this can be caused by feeling stuck at home and by fear of the future. Depression and anxiety often occur together.

Some symptoms of anxiety are similar to symptoms of pulmonary fibrosis, and some are similar to symptoms of depression. Anxiety is characterized by feelings of panic, fear, or impending doom. 

How To Find Relief

Anxiety is usually treated with therapy and medication. 

A therapist trained in working with chronic disease patients can help you navigate the stress and uncertainty of a stage 4 pulmonary fibrosis diagnosis. They may recommend different forms of therapy to help with your anxiety.

Some types of medications can also help with anxiety. Like antidepressants, these can affect each person differently, and you may need to try a few types before you find one that you’re comfortable with.

4. Poor Appetite

A lack of appetite from pulmonary fibrosis can be caused by many things, such as medications, depression, or not feeling well. Poor appetite can lead to difficulty maintaining your body weight, low quality of life, and difficulty pushing through your disease. 

How To Find Relief

There are a few things you can do at home to help manage a poor appetite:

  • Eat multiple small meals throughout the day if big meals seem like too much
  • Add calories to your meals with oils, butter, milk, and yogurt
  • Try taking a walk or getting in some movement before you eat to increase your appetite

Certain types of medication therapy may also help. Your doctor may be able to give you medicine to increase your appetite.

If your lack of hunger is caused by a medication you’re taking for pulmonary fibrosis, consider asking your doctor if there’s an alternative treatment option available.

Poor appetite caused by depression can sometimes be resolved through therapy or antidepressant medications.

5. Bothersome Cough

Coughing is a common symptom of end stage pulmonary fibrosis and can lead to discomfort and chest pain. Pulmonary fibrosis itself can lead to a cough, but so can accompanying symptoms like acid reflux, post-nasal drip, emphysema, and sleep apnea.

Coughs can be triggered by talking a lot, physical exertion, or environmental factors.

How To Find Relief

Treatment for your cough may include:

  • Avoid environmental triggers like cigarette smoke or heavy perfumes
  • Cough drops or cough medication
  • Drink plenty of fluids
  • Add honey to your drinks to help soothe your cough
  • Try breathing exercises to help control your cough
  • Check with your doctor about prescription medications for your cough
  • Use antacids or other medicines if your cough is caused by acid reflux
  • Use a decongestant if your cough is caused by post-nasal drip

6. Becoming Housebound

For many patients, moving into stage 4 of pulmonary fibrosis means they will need high-flow oxygen. High-flow oxygen is considered above four liters, and patients in stage 4 usually need this oxygen 24 hours a day.

Portable oxygen tanks that can accommodate that can be expensive and hard to find.

As a result, becoming housebound is a genuine fear for patients with stage 4 pulmonary fibrosis. Becoming housebound can lead to social isolation, depression, and anxiety.

How To Find Relief

One of the best ways to avoid becoming housebound is to speak to your medical provider about your options for a high-flow portable oxygen system. An oxygen system will allow you to spend some time outside your home.

Be sure you know how long this system will last, and use your pulse oximeter to check your oxygen saturation while you’re out.

If a portable high-flow oxygen system isn’t an option for you, consider working with family and friends to host get-togethers and events in your home. If your loved ones can come to you, that will help reduce social isolation.

Over the last few years, video calling has become much more common. If hosting in your home isn’t an option, consider setting up weekly or monthly video calls with your loved ones to help you stay connected.

7. Reduced Lung Function

Pulmonary fibrosis’s characteristic damage and lung scarring often result in reduced lung function. This can make breathing difficult and getting enough oxygen into your body.

Your body needs oxygen to survive. Signs that you aren’t getting enough oxygen include confusion, headache, shortness of breath, and restlessness. 

How To Find Relief

By the fourth stage of pulmonary fibrosis, your doctor has probably gone over many of your treatment options with you. If you continue to struggle to get enough oxygen, you should speak with your doctor. 

If you’re at stage 4, you’ve likely become familiar with oxygen systems by now. Oxygen therapy helps your lungs get the oxygen they need.

It can also help reduce blood pressure and improve your overall well-being. If you feel that your current oxygen therapy is no longer effective, speak with your doctor.

Some methods of pulmonary rehabilitation can also help increase lung function. This may include breathing techniques and exercise.

8. Fluid Retention

Fluid retention, also called edema, is a side effect of your body not getting enough oxygen. When you don’t have enough oxygen, your heart has to work extra hard, decreasing the amount of blood pumping through your body. Your brain may tell your kidneys to retain water to compensate. 

This fluid usually settles in the lower areas of the body as the day goes on, like your feet and legs. It can also lead to fluid retention in the abdomen, which may cause reduced appetite and weight loss.

How To Find Relief

You and your doctor should consider a few things when treating fluid retention.

One of the easiest things to adjust is your diet. Excess salt can increase your likelihood of swelling and edema. Your doctor may recommend reducing extra salt or fluids depending on your diet.

It’s generally recommended that you don’t exceed 2,000mg of salt per day and not exceed 64 ounces of fluid daily.

Some medications can also result in fluid retention. Your doctor may decide to look at other medication options for you. Nerve pain medications and some opioids are common culprits of edema.

Your doctor may also consider increasing your oxygen intake. They may recommend you spend more time on your oxygen system or may want to upgrade you to a stronger system.

This helps reduce the stress on your heart, which reduces overall fluid retention. 

9. Disturbed Sleep Patterns

Many of the symptoms of pulmonary fibrosis can disrupt your sleep. Coughing, chest pain, and shortness of breath can make it difficult to fall into a deep sleep, no matter how tired you are.

Patients with pulmonary fibrosis are also more likely to have sleep apnea, a condition where you either stop breathing or do not breathe deeply during sleep. 

While it’s unclear what the relationship between sleep apnea and pulmonary fibrosis is, having the two together can make it difficult for your body to get enough oxygen while you’re asleep.

Patients with sleep apnea often have difficulty staying asleep and may be tired and sleepy during the day.

How To Find Relief

Treating the underlying cause of your disrupted sleep is one of the best ways to get your sleep patterns back on track. Consider medications or therapies for cough and chest pain, and use your oxygen as directed. 

If you’re feeling excessively sleepy during the day, taking a nap is okay. Short power naps can help rejuvenate your body and reset your sleep cycle.

Your doctor may recommend a sleep study to rule out sleep apnea. If you are diagnosed with sleep apnea, your doctor may recommend nighttime oxygen or a CPAP machine.

10. Need for Intensive Home Support

Many patients with stage 4 pulmonary fiborosis will need intensive home support. This can be difficult, as it may feel to patients that they are losing their independence. 

How To Find Relief

For patients needing home support, there is no “cure.” However, there are ways to make things easier:

  • Surround yourself with a strong support team. If your loved ones are offering to help, allow them.
  • Find a home support nurse or provider that you trust and can build rapport with.
  • Talk to a therapist about the mental struggles you may have around needing intensive home care.

11. Frequent Flare-Ups and Hospitalizations

Patients with stage 4 pulmonary fibrosis symptoms may deal with increasing flare-ups and hospitalizations. This can be difficult both mentally and physically. 

How To Find Relief

Often, flare-ups can come on suddenly, but sometimes they’re triggered by environmental toxins like cigarette smoke, chemicals, or perfumes. If you notice your flare-ups have a trigger, do what you can to avoid that trigger to reduce the frequency of flare-ups.

When flare-ups and hospitalizations happen, lean on your support system. Allow loved ones to visit, help you with tasks, and be there for you to vent. Speaking with a therapist can also be helpful.

12. Increased Severity in Shortness of Breath

As your pulmonary fibrosis worsens, so will your shortness of breath. This can make it challenging to get enough oxygen and cause extreme discomfort.

How To Find Relief

There are a few different things you can try to relieve your shortness of breath:

  • Practice breathing techniques
  • Use your oxygen
  • Don’t over-exert yourself
  • Avoid triggers like allergens, cigarette smoke, or perfumes
  • Ask your doctor about medications like inhalers to help you breathe

13. Difficulty Maintaining a Healthy Body Weight

Patients with pulmonary fibrosis often lose weight. This can be due to a lack of appetite or because some pulmonary fibrosis medications can cause nausea, vomiting, and diarrhea. 

How To Find Relief

If your weight struggle is due to loss of appetite, nausea, vomiting, or diarrhea, consider the following:

  • Asking your doctor to adjust your medication
  • Eating small meals throughout the day
  • Eating foods that are easy on your stomach
  • Drink peppermint or ginger tea or ginger ale

New Developments in Pulmonary Fibrosis Staging

Doctors and researchers have been developing a new staging system for pulmonary fibrosis. The new pulmonary fibrosis stages system currently in development is called the GAP Index.

More sophisticated than the traditional pulmonary fibrosis staging system, the GAP Index comprises four predictors: age, recent respiratory hospitalization, baseline FVC and 24-week change in FVC. 

The sum of the individual scores for each factor is then used to obtain a score.

More recently, doctors and researchers have continued to develop the GAP Index, using a multidimensional risk prediction model and staging system. 

This Gap Index model also consists of 4 baseline variables: gender, age, and two lung physiology variables (FVC and diffusing capacity of the lung for carbon monoxide). 

They developed a GAP Index calculator to help doctors and patients better understand pulmonary fibrosis stages, where a patient might fall within those stages and the individual risk for each patient.

There are GAP Index calculators available online. Remember to discuss your GAP Index results with your doctor as well as any changes to your symptoms.


Stage 4 pulmonary fibrosis can be a difficult diagnosis. While there are medications that can slow the progress of the disease, there is no cure. In some cases with severe stage 4 pulmonary fibrosis, patients may be eligible for a lung transplant, which can relieve many of the above symptoms.