What is Idiopathic Pulmonary Fibrosis?
Idiopathic pulmonary fibrosis (IPF) affects mostly the elderly, usually those older than 70, and is rarely diagnosed in people under 50. So, what is idiopathic pulmonary fibrosis? With increasing shortness of breath and a constant dry cough, IPF is a debilitating, progressive lung disease.
IPF involves scarring of lung tissue but, as indicated by the medical term “idiopathic,” the exact cause of this scarring is unknown. There is also currently no effective treatment.
In this article you can learn more about the symptoms that might point to idiopathic pulmonary fibrosis. There is also brief explanation of what happens in the lungs and which factors might trigger this condition.
Because the symptoms of IPF are common to many lung conditions, the diagnosis is often missed and needs to be confirmed by a full medical workup.
Symptoms of IPF
The exact symptoms experienced by those with idiopathic pulmonary fibrosis, and how rapidly it develops, vary from person-to-person.
The following are the common and most significant symptoms of IPF.
1. Persistent Dry Cough
A persistent dry, hacking cough is usually the first symptom of IPF and can occur for months before the other symptoms become severe. Many patients and their caregivers describe the cough as the most distressing symptom.
Around 80% of those with IPF experience the dry cough. It is not relieved by any of the usual cough medication, and although there are many theories, scientists have been unable to explain what triggers it.
One study found that, on average, people with IPF cough around 14 to 25 times per hour during the day and 1.9 to 9 times per hour at night. It has even been suggested that the constant coughing could cause further lung damage, contributing to disease progression.
The coughing spells can have a serious impact on quality of life. Physical consequences include chest pain, lightheadedness, incontinence and poor sleep. It could also be stressful and embarrassing and interfere with work and social activities.
2. Shortness of Breath
As normal lung function in those with IPF deteriorates, they experience an increase in shortness of breath.
Initially, the person might become breathless only while exercising and it could be blamed on being unfit and getting older. Eventually, even light activity, like washing dishes or dressing, could leave the person breathless.
The changes in the structure and the function of the lungs can be confirmed by a lung function test, or sprirometry, a chest x-ray and a lung biopsy.
It is not surprising that 95% of those with IPF report extreme fatigue — physical, mental and emotional. Fatigue impacts on their overall quality of life and it is often not taken seriously by caregivers and others because it cannot be seen.
Various factors contribute to the fatigue:
- Constant poor oxygen supply to all parts of the body.
- The extra work the body needs to do just to breathe.
- The effort of the constant coughing.
- Poor sleep quality as a result of low oxygen and coughing.
- Side effects of medication.
- Anxiety, fear and depression, contributed to by both by the lack of oxygen to the brain and distress about the condition.
Interestingly, it has been found that people with IPF experience less fatigue if they do some form of regular exercise.
4. Loss of Appetite and Weight Loss
Unintended weight loss often accompanies IPF. This could be the result of loss of muscle mass due to physical inactivity or not eating properly. The symptoms, medications and depression could all contribute to loss of appetite and malnourishment.
Poor nutrition and weight loss is often linked to more rapid progression of the condition. So, it is important to remain as active as possible and to ensure proper nourishment by eating nutrient-dense foods.
The effects of IPF on the body may eventually cause other serious conditions. These include pulmonary hypertension, blood clots in the lungs, lung infections and heart failure. This is why regular monitoring the person’s condition by a medical practitioner is essential.
What is Known About IPF?
The air you breathe in fills millions of tiny sacs, or alveoli, in your lungs. From here the oxygen from the air passes into tiny blood vessels surrounding the alveoli.
In idiopathic pulmonary fibrosis, the thin walls of the alveoli become damaged and thick, which causes stiff scar tissue to develop. As the amount of alveolar damage increases, the lungs become stiff and don’t expand properly with each breath. Not enough oxygen is absorbed into the body, and this is what causes the breathlessness.
Symptoms of IPF can worsen slowly over years and might be interpreted as a normal part of aging until they become debilitating. For others the condition can become severe within a few months.
Idiopathic means that the cause of the condition is unknown. Research is ongoing and there are many theories. So far, it appears that IPF is probably caused by a combination of biological and environmental factors.
In a small number of cases, IPF runs in families. Furthermore, the condition has been linked to certain environmental pollutants, viral infections, acid reflux, smoking and specific autoimmune diseases. There are already signs that a severe COVID-19 infection might increase the incidence of IPF in the population.
Managing Idiopathic Pulmonary Fibrosis
IPF is a severe disease, characterized by progressive breathlessness, a constant dry cough and fatigue. Lung scarring cannot be reversed and to date there is no treatment to stop its progression.
The condition has to be properly diagnosed by a medical practitioner and the person diagnosed should remain under their care for regular monitoring. There is now one medication that appears to slow down fibrosis, and the complications which may arise can often be treated.
Furthermore, there are a number of interventions that can help to relieve the symptoms, such as oxygen therapy and pulmonary rehabilitation, which includes exercises to expand lung capacity. Self-care, like a nutritious diet, regular exercise and stopping smoking, will help those with IPF to remain active for longer.
For valuable support and advice, if you have IPF you can join the Living with Pulmonary Fibrosis group, recommended by The American Lung Association.