Pulmonary fibrosis is a lung disease caused by damage or scarring of the lung tissue. The most common type is idiopathic pulmonary fibrosis or IPF. There is no known cause for IPF hence the term idiopathic. Any damage to the lungs is usually irreversible and progresses over time. Certain medications can slow down the disease progression. In the end, almost everyone will need a lung transplant. Idiopathic pulmonary fibrosis causes scar tissue to build up in one or both lungs. The lungs can’t effectively transport oxygen to the bloodstream when that happens. The disease usually affects adults in their 50s and 70s. The scarring that occurs in the lungs is the same kind of scarring that occurs when you get a wound. As the disease progresses, it becomes harder and harder to breathe. Some people are more prone to IPF than others.

Give Us Your Opinion! – Take Our IPF Survey Today

Risk factors include

• Age: While children can develop IPF, it’s more common in older adults.
• Gender: Idiopathic pulmonary fibrosis affects men more than women. 
• Smoking: Long-term exposure to cigarette smoke can leave the lungs damaged and scarred. 
• Cancer treatments: If you’ve had radiation treatment to the chest, you can develop IPF. Chemotherapy drugs can also damage your lungs over time. 
• Genetic factors: If you have a family history of pulmonary fibrosis, then you are at risk of getting it.
• Occupations: People who work in mines, farms, and construction get exposed to pollutants more. These pollutants in the long run end up damaging the lungs. 

Types of Idiopathic Pulmonary Fibrosis

There are two types of idiopathic pulmonary fibrosis;

Sporadic idiopathic pulmonary fibrosis

When idiopathic pulmonary fibrosis affects only one person in the family it is sporadic. Sporadic IPF is very rare.

Familial idiopathic pulmonary fibrosis

It is the most common type of IPF. With familiar IPF, one or more family members will be affected. One of the risks of IPF is genetics. If a few members of your family have suffered from pulmonary fibrosis, you are also at risk of the same. 

Symptoms of Idiopathic Pulmonary Fibrosis

Liver damage due to IPF is irreversible. After diagnosis, a majority of individuals live for about 3 to 5 years. Most deaths occur due to lung cancers, pulmonary embolism, pulmonary hypertension, and pneumonia. 

Some of the common signs and symptoms of idiopathic pulmonary fibrosis include;

• Dyspnea or shortness of breath in the absence of vigorous physical activity 
• A consistent dry cough
• Unintentional weight loss
• Muscle and joint aches
• Fatigue
• Clubbing or widening of tips of the toes and fingers

The severity of the symptoms varies from one person to another. Some people progress to serious illness within a couple of months. Others take years and even decades to get there. Some individuals may also experience acute exacerbations like severe shortness of breath. These can last for days and even weeks. If you have acute exacerbations, you may need a ventilator to help you breathe. The doctor will also prescribe medications like antibiotics to treat acute exacerbations. 

Diagnosis of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis falls under other lung conditions known as interstitial lung diseases. These are lung diseases that involve scarring and inflammation of the lung tissue. As such, IPF is hard to diagnose because symptoms resemble those of other lung diseases. It usually takes doctors a lot of time and tests to make the right diagnosis. If you always experience shortness of breath, however, you need to see a specialist to rule out IPF. The doctor will first conduct a physical exam. During the exam, they will listen to your lungs through a stethoscope. 

They may also ask follow-up questions about your symptoms, and lifestyle choices. They will also check your family history of IPF and other medical conditions you may have. After that, the doctor will order more tests to confirm IPF diagnosis. 

• Imaging test: A chest X-ray checks if there is inflammation and scarring in the lungs. The doctor may also order a CT scan to get a better detailed picture of the extent of the damage to your lungs. A CT scan will also help pinpoint the exact cause of your IPF. 
• Exercise test: The doctor will have you ride a stationary indoor bike or walk on a treadmill. They will then assess the oxygen levels in your blood. If you have IPF, oxygen levels are lower than normal.
• Lung biopsy: A lung biopsy involves removing a small piece of tissue from your lung. The tissue helps rule out conditions like lung cancer. 
• Arterial blood gas tests and pulse oximetry: These tests help measure the amount of oxygen in your blood. 
• Spirometry: The doctor will ask you to blow into a special device known as a spirometer as hard as you can. The device measures how much air is flowing in and out of your lungs. 

How to Treat Idiopathic Pulmonary Fibrosis

While treatment options for idiopathic pulmonary fibrosis exist, they help manage the symptoms. To prevent further disease progression, the following treatment options can help;

Medications: There are two drugs approved to treat idiopathic pulmonary fibrosis. They are pirfenidone (Esbriet) and nintedanib (Ofev). These drugs slow down the damage and scarring in the lungs. Corticosteroids can also help reduce inflammation during IPF flare-ups. 

Oxygen therapy: This treatment involves breathing through prongs inserted through your nose. Oxygen therapy helps ease shortness of breath by increasing the amount of oxygen flowing into the lungs. 

Pulmonary rehabilitation: This is where you work with specialists to help you cope with the symptoms better. A specialist will focus on things like your diet, exercise, and stress relief. 

Lung transplant: If the lung damage is too severe and progressing quickly, a lung transplant is the best option to save your life. Lung transplant involves replacing the damaged lungs with a healthy lung (s) from a donor. This is a major surgery that will see you spend about four weeks or more in the hospital. You will also be under medications all your life to ensure that your body doesn’t reject the new lung. 

How to Live With and Manage Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis can progress slowly or fast. Sometimes the symptoms flare up requiring you to be on a ventilator for days or weeks. The damage that occurs to the lungs cannot be reversed. The only way to ensure that you live a long healthy life is to have a lung transplant. People who don’t get on the transplant list or whose symptoms progress fast usually die within three to five. 

Here are some of the ways you can live with IPF;

• Make sure you eat a healthy diet full of vegetables, fruits, and whole grains. Eating smaller meals will also ensure that your lungs have enough room to function. 
• Exercising regularly can help strengthen the lungs over time. Physical activity can also be a great way of reducing stress.
• If you smoke, make a hard decision to stop. Tobacco products only increase the damage to your lungs. 
• If you have IPF, your risk of infections like pneumonia and the flu increases. Getting yearly flu shots will help reduce this risk. 
• Find ways of managing stress since it can worsen IPF symptoms and make the disease progress faster. 

FAQs

1. What is the outlook of idiopathic pulmonary fibrosis? IPF affects people differently. For some, the symptoms worsen within a few months while others take years. The average lifespan for someone with IPF is 3 to 5 years if a lung transplant is not an option. 
2. Is COPD the same as for pulmonary fibrosis? While the conditions worsen over time and make breathing hard, they are not the same. With pulmonary fibrosis, the damage is on the cells between the blood vessels and other lung structures. COPD involves damage to the lung tissue, airways, and alveoli. Bronchitis and emphysema are examples of COPD.

Healthier Me Today is intended for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment. Never ignore professional medical advice in seeking treatment, always consult with your healthcare professional. Stay healthy!