What is IPF?
Pulmonary fibrosis is a form of lung disease where scar tissue, technically known as fibrotic tissue, forms in the space around the air sacs. As the scar tissue builds up, the sacs cannot take in air effectively. This leads to shortness of breath, that can make any physical exertion difficult and reduces oxygen in the blood, which can cause fatigue. The disease is progressive, meaning once it takes root, it usually worsens, although at different rates in different people.
Idiopathic pulmonary fibrosis is diagnosed when the pulmonary fibrosis has no apparent or known cause. The majority of IPF patients are older men who have a history of smoking.
What is AE-IPF?
Acute exacerbation of pulmonary fibrosis occurs when the disease suddenly and drastically worsens. The onset of acute events is worse in cases of IPF since the actual cause of the original PF is unknown, so often, the reason for acute exacerbation is unknown and difficult to control.
Acute exacerbations are the most common cause of fatality from the disease, which is why we need to pay attention to their causes. AE-IPF is an area where considerable research is ongoing with the aim to identify curative therapies.
How Does Pulmonary Fibrosis Occur?
Fibrosis or scarring is usually the body’s defense mechanism, a form of bandage for injury. Just like a scar forms when our skin is cut, fibrotic tissue forms inside our bodies when parts are injured. In the case of idiopathic pulmonary fibrosis, that defense mechanism is in overdrive and is totally out of sync, causing scarring to occur on undamaged, healthy tissue.
Some researchers theorize that the unusual scarring is attributable to the body’s autoimmune system not working as intended. Therefore, much of the current research into IPF is focused on finding appropriate autoimmune response treatments.
Natural scarring is caused by a protein called fibrin. Some treatment options, such as systemic enzyme therapy, look at reducing fibrin in the body to slow the rate of scarring.
In an acute exacerbation episode, the scarring is suddenly accelerated. New treatment options involve high dosage autoimmune suppressants. Natural anti-fibrosis nutrients too are showing some benefits in clinical trials, in reducing fatality rates in AE-IPF.
Symptoms of IPF and Acute Exacerbation IPF
IPF patients typically first present with shortness of breath. This progresses to other symptoms which include:
- Chronic dry, hacking cough
- Fatigue
- Chest pain
- Loss of appetite
- Unexplained and unexpected weight loss
- Aches and pains in muscles and joints
- Digital clubbing (widening and rounding of the tips of extremities such as the fingers and toes)
When acute exacerbation of IPF occurs, these symptoms may seem to progress with greater speed and ferocity. For example, the initial shortness of breath might suddenly progress to severe difficulty breathing, the chronic dry cough can turn into bouts of extreme coughing, and the fatigue might become more unrelenting and pronounced.
What Causes Acute Exacerbation of IPF?
The exact causes of acute episodes are not well understood. In true idiopathic acute episodes, there is, in fact, no known cause. However, acute exacerbation can sometimes be caused by a known trigger such as an infection, exposure to contaminants or GER.
Infection
Lung infections can be serious for PF sufferers. Lung infections are a leading cause of acute exacerbation in pulmonary fibrosis, idiopathic or otherwise. Common illnesses such as influenza and pneumonia can preempt an acute episode.
Environmental Pollutants
Exposure to dust, smog, pollen or smoke can be a trigger for exacerbations. Those who smoke put themselves at an extreme risk if they do not abstain following a diagnosis of pulmonary fibrosis.
Gastroesophageal Reflux (GER)
There is a strong link between GER and AE-IPF, with studies showing that around 80-90 percent of patients with IPF also have GER. There is speculation that the acid that enters the esophageal system during a reflux event, transfers into the lungs and exacerbates the scarring.
Prognosis for IPF When Acute Symptoms Appear
It is difficult to determine the prognosis for people with IPF for a variety of reasons. To start, it can take several years to get diagnosed with IPF even if symptoms began years prior. At that point, it can be difficult for people with IPF to find or access the right care, to make lifestyle changes, and to feel hopeful about the future. IPF survival rates used to be 3-5 years on average, with 20 percent of those affected surviving past the five-year mark (and often managing to control acute exacerbations of the disease). Now, however, the prognosis is much more hopeful thanks to advances in research, medicine, and treatment options.
Acute exacerbations of IPF are historically highly fatal, preceding approximately 40 percent of IPF deaths. Most patients admitted to the hospital with AE-IPF do not survive. A mean survival rate once an acute episode occurs is reported to be less than six months. Avoiding the known triggers of AE-IPF can help improve the long-term prognosis.
What Can You Do to Help Your Prognosis?
While there is no cure, there are some treatments that can slow the progression of the disease and improve prognosis. Avoiding acute episodes and slowing the rate of scarring are key factors in improving survival rates.
Treat GER
As gastroesophageal reflux is a risk factor for AE-IPF, control of the symptoms via proton pump inhibitor (PPI) medication and dietary change is recommended. Maintaining an alkaline diet combined with enzyme and probiotics therapy have shown positive results in reducing the occurrence of GER as well as in cleansing the lungs of mucus. Some examples of alkaline foods include squash, lettuce, chickpeas, basil, and spinach. Avoid acidic foods like sugar, meat, white rice and pasta. Other sources of acidity to stay away from include tobacco and caffeine.
Protect Against Infections
A high occurrence of acute exacerbation IPF episodes is linked to lung infections such as colds, flu or pneumonia. To protect against infection, it is essential to apply stringent hygiene controls.
In addition to ensuring strict hospital standards of hygiene, avoid exposure to cold or situations where germs spread easily. Such situations include enclosed offices with communal air conditioning, public transport, air travel or other occasions where germs can circulate easily due to the higher number of people in a confined space.
Vaccinations for common influenza or pneumococcal viruses are strongly recommended to avoid infection.
Perform Breathing Exercises
Breathing exercises that help strengthen your lung capacity and improve your oxygen intake are considered beneficial in slowing the rate of progression in IPF. Helpful exercises include:
- Deep inhalations through your diaphragm
- Controlled inhalation, pausing, and exhalation on a count
- Certain controlled coughing exercises
Discuss with your doctor or therapist about using a lung strengthening device that can help measure and improve lung capacity over time.
Practice Sudarshan Kriya Yoga (SKY)
While all forms of yoga can provide excellent health benefits by encouraging improved breathing and circulation, Sudarshan Kriya Yoga focuses specifically on cyclical breathing. The cyclical breathing exercises performed in this type of yoga are highly beneficial to pulmonary fibrosis patients.
Avoid Environmental Exposure
Avoid stressors such as dust, pollen or smoke that may promote an acute exacerbation. If the natural environment where you live is particularly dusty or polluted, consider using a face mask or breathing device. Alternatively, use supplemental oxygen when outdoors.
Systemic Enzymes
Certain natural enzymes have been found to be fibrinolytic. This means that they may help reduce the amount of the scar-tissue-forming protein, fibrin, in the body. Decreasing fibrin, in turn, may reduce the progression of scarring.
Two enzymes which have shown positive effects on reducing fibrin are nattokinase and serrapeptase. While there is clinical evidence that serrapeptase and nattokinase enzymes are fibrinolytic, they are usually recommended to be taken in limited doses. No studies have been conducted looking at the effects of high doses.
Medication Treatment Options
There is unfortunately little in the way of curative treatment for fibrosis to counteract or repair the scarring. Treatments aim to reduce symptoms, slow deterioration and improve the comfort of the patient.
The cause of cell damage itself is not understood and there is no way to stop or reverse it. However, two key methods are currently leading research hypothesis: antifibrotic remedies that reduce development of fibrin proteins; and immune-suppressant medications that inhibit the immune response causing scarring, similar to those used by organ transplant recipients.
Anti-Scarring (Antifibrotic) Medication
Two medication options that have been effective in reducing the rate of progression of scarring from pulmonary fibrosis are nintedanib and pirfenidone.
Nintedanib, marketed under the brand names Ofev and Vargatef, is an antifibrotic drug that blocks the scarring process by inhibiting tyrosine kinase activity. Tyrosine kinase is a naturally occurring enzyme that is responsible for activating certain proteins, including fibrin.
Pirfenidone, which is marketed under the names Esbriet, Pirfenex and Piresp, is both antifibrotic and anti-inflammatory. The antifibrotic action of pirfenidone is exerted by reducing the rate of fibroblast growth, thus reducing fibrosis.
Immune-Suppressant Therapy
Recent breakthroughs in immune-suppressant therapies have shown early indications of improved survival rates for AE-IPF. One trial that used rituximab, which attacks autoantibody-producing B cells, combined with plasma exchanges to reduce the number of autoantibodies in the blood indicated improvements in survival as compared to the historical control group. Studies looking into the effects are still ongoing and not yet conclusive, as there are some rare extreme negative reactions to rituximab reported.
Corticosteroids are no longer recommended as the fibrosis is not considered an inflammatory action. However, those that provide additional immune-suppression properties are being studied.
Researchers have mixed feelings about Prednisone use for the treatment of AE-IPF since it assists in suppressing immune system function and reduces inflammation but also has significant side effects.
Azathioprine (marketed under the brand name Imuran) or mycophenolate (marketed under the brand name Cellcept) have been tested and shown to be effective for suppressing immune system function in AE-IPF cases. Studies are at the early stages and are not conclusive. There are some significant warnings and side effects associated with prolonged use of azathioprine, such as links to certain skin cancers. Mycophenolate, an autoimmune suppressant, is more expensive to manufacture but has shown positive results without the inherent risks of azathioprine. An antibiotic derivative, it has previously been used long-term for organ transplant recipients to prevent rejections and may have significant potential for AE-IPF.
Antibiotic Treatment for Infective Causes
If any lung infection is contracted in patients with IPF, it is imperative the infection is treated immediately to avoid an acute illness, usually by broad-spectrum antibiotics. Any lung condition that occurs must be treated with professional medical assistance at the earliest, and if needed, after-hours emergency treatment should be sought.
Palliative Care
It is important that IPF patients get palliative support to improve their quality of life. This can be in the form of physical and emotional support.
Supplemental oxygen can help improve general health considerably, since the reduction in breathing capacity often leads to an oxygen deficiency in the body. Additional oxygen can help provide more energy and mental clarity.
Nervous system repression or anti-anxiety medication can assist sufferers to alleviate the discomfort felt, by relaxing the central nervous system and improving the ability to breathe as well as easing mental concern.
Finding an IPF support group is a form of palliative self-care, as it can help patients deal with the feelings they have and learn from others about available options and strategies that have worked for them in coping with the disease.
Track Your Progress
Measuring levels of blood oxygenation and lung function are essential to monitoring the progression of IPF and in identifying AE-IPF events before they become too difficult to control.
A spirometer or lung function measurement device can assist in providing a quantitative measurement of your lung capacity. Spirometers measure the exhalation output of lungs after one deep inhalation.
A pulse oximeter is a non-invasive tool for measuring blood oxygen levels. It is a highly recommended way to track your progress, and most models are inexpensive. You can purchase a pulse oximeter for around $15 from pharmacies or health shops. Some smartphones also come with built-in oximeters. Record your levels regularly, especially if trying new exercises and supplements.
An exercise test, like the six-minute walk test, can be used to chart your fatigue levels. In these tests, a short exercise program–for example, walking for six minutes–is conducted with a post-exercise evaluation of heart rate, breathing and general well-being.
AE-IPF and How to Cope
IPF is a difficult diagnosis. While researchers are still working to understand the mechanisms behind it, which hopefully will lead to curative treatments, there are ways to reduce the chance of having an acute event.
Exercising, avoiding exposure to triggers and using antifibrotic enzyme supplements are some of the primary strategies to improve prognosis. Antifibrotic medication has been proven successful in slowing progression, and trials of autoimmune suppressant therapy are both promising and ongoing.
Maintain fitness and avoid triggers as much as possible, and commit to a palliative care regimen to improve your quality of life. Additionally, discuss treatment options with a medical professional, ideally a pulmonologist, about specific medical treatments available to you, as advances may occur at any time. Research and read about other complementary therapies that may help with your condition.
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