Pulmonary Fibrosis, Op-ed

Pulmonary fibrosis is a serious, lifelong lung disease. It causes lung scarring (tissues scar and thicken over time), making it harder to breathe.

It can occur in a wide range of clinical settings and can be precipitated by a multitude of causes. The estimated prevalence ranges from 25 to 74 per 100,000 populations.

Idiopathic Pulmonary Fibrosis

Idiopathic is a term used when we can’t determine what caused a condition. Idiopathic pulmonary fibrosis (IPF) is the most common type of pulmonary fibrosis.

About 5 million people are affected globally. The disease newly occurs in about 12 per 100,000 people per year.

The incidence of IPF appears to be increasing and is due in part to the aging population, increased awareness of IPF among patients and clinicians, and the improved ability to diagnose IPF due to advances in CT imaging.

A number of comorbidities are associated with IPF, including pulmonary hypertension, emphysema, diabetes, lung cancer, gastroesophageal reflux disease, depression, obstructive sleep apnea, and cardiovascular disease.

Causes

There are over 200 known causes of non-IPF. The list includes significant acute insult to the lungs as adult respiratory distress syndrome , a significant pulmonary infection, diffuse alveolar damage from any source, inhaled substances as coal/silica, asbestos, radiation , congenital conditions as cystic fibrosis , autoimmune conditions, connective tissue disorders, sarcoidosis, tuberculosis and ageing.

Risk Factors of Pulmonary Fibrosis

Certain risk factors, like smoking, may make it more likely you could develop pulmonary fibrosis. But even having one or more risk factors doesn't mean you’ll definitely get the disease one day.

Older age, most people who get pulmonary fibrosis develop it in the second half of life, between ages 50 and 70.

Pulmonary fibrosis affects more men than women. However, cases in women have risen in recent years.

Cigarette smoking increases the risk of getting pulmonary fibrosis. Cigarette smoking is one of the most recognized risk factors for development of IPF.

Furthermore, recent work suggests that smoking may have a detrimental effect on survival of patients with IPF. The prevalence of tobacco use in IPF ranges from 41% to 83%, depending on the case definition used in the studies .

The mechanism by which smoking may contribute to the pathogenesis of IPF is largely unknown. However, accumulating evidence suggests that increased oxidative stress might promote disease progression in IPF patients who are current and former smokers.

Working around dust or fumes as regularly breathing in chemicals or hazardous substances can damage the lungs. Farmers, ranchers, hairdressers, stone cutters/polishers and metal workers may be at increased risk for pulmonary fibrosis.

Other medical conditions: in some cases, another medical condition (such as the autoimmune disease rheumatoid arthritis or a viral infection) leads to pulmonary fibrosis.

Other factors: radiation exposure, such as radiation therapy to treat cancer, can damage lung tissue. So can some medications, including chemotherapy and certain heart medications.

Inheriting pulmonary fibrosis is very rare. Researchers still have much to learn about how (and which) genes may cause pulmonary fibrosis.

Post COVID Pulmonary Fibrosis

After the COVID-19 outbreak, increasing number of patients worldwide who have survived COVID-19 continue to battle the symptoms of the illness, long after they have been clinically tested negative for the disease.

As we battle through this pandemic, the challenging part is to manage COVID-19 sequelae which may vary from fatigue and body aches to lung fibrosis.

Various mechanisms of lung injury in COVID-19 have been described, with both viral and immune-mediated mechanisms being implicated.

Cytokine storm caused by an abnormal immune mechanism may lead to initiation and promotion of pulmonary fibrosis.

The reason remains unknown as to why certain inpiduals recover from such an insult, whereas others develop progressive pulmonary fibrosis due to accumulation of fibroblasts and myofibroblasts and excessive deposition of collagen.

Symptoms of Pulmonary Fibrosis

Pulmonary fibrosis doesn’t affect everyone in the same way. Many common, easily treatable conditions can cause similar symptoms. Sometimes these symptoms are signs of the common cold or an upper respiratory infection.

Pulmonary fibrosis symptoms include: breathing in short, shallow spurts, dry cough that doesn’t go away, fatigue (extreme tiredness, no matter how much you sleep), shortness of breath, especially during or soon after you exercise, weight loss that’s not on purpose or easily explained.

As the disease progresses, some people experience clubbing, fingertips or toes that look different, such as wider or more round and cyanosis, bluish skin (in fair-skinned people) or grey or white skin around the mouth or eyes (in dark-skinned people) from too little oxygen in the blood.

Treatment

The lung scarring that occurs in pulmonary fibrosis can't be reversed, and no current treatment has proved effective in stopping progression of the disease.

Some treatments may improve symptoms temporarily or slow the disease's progression. Others may help improve quality of life.

Using oxygen can't stop lung damage, but it can: make breathing and exercise easier, prevent or lessen complications from low blood oxygen levels, reduce blood pressure in the right side of your heart and improve your sleep and sense of well-being.

You may receive oxygen when you sleep or exercise, although some people may use it all the time.

Pulmonary rehabilitation can help you manage your symptoms and improve your daily functioning. Pulmonary rehabilitation programs focus on: physical exercise to improve your endurance, breathing techniques that may improve lung efficiency, nutritional counseling, counseling and support.

Lung transplantation may be an option for people with pulmonary fibrosis. Having a lung transplant can improve your quality of life and allow you to live a longer life. However, a lung transplant can involve complications such as rejection and infection.

Complications

The following are complications that can be seen in patients with idiopathic pulmonary fibrosis pulmonary hypertension, acute exacerbation of pulmonary fibrosis, respiratory infection, acute coronary syndrome, thromboembolic disease, adverse medication effects and lung cancer.

Tips for Prevention

Some cases of pulmonary fibrosis may not be preventable. Other cases are linked to environmental and behavioral risk factors that can be controlled.

Follow these tips to lower your risk of getting the disease: avoid smoking, avoid second-hand smoke, and wear a face mask or other breathing device if you work in an environment with harmful chemicals.

If you’re having trouble breathing, make an appointment with your doctor. Early diagnosis and treatment can improve the long-term outlook for people with many lung diseases, including pulmonary fibrosis.

Eat well. People with lung disease may lose weight both because it's uncomfortable to eat and because of the extra energy it takes to breathe.

Yet a nutritionally rich diet that contains adequate calories is essential. Try to eat smaller meals more often during the day.

Aim to eat a variety of fruits and vegetables, whole grains, low-fat or fat-free dairy products, and lean meats. Avoid trans-fat and saturated fat, too much salt, and added sugars.

Get moving, regular exercise can help you maintain your lung function and manage your stress. Aim to incorporate physical activity, such as walking or biking, into your daily routine.

Take time to rest; make sure to get enough rest. Taking time to rest can help you have more energy and cope with the stress of your condition.

Get vaccinated, respiratory infections can worsen symptoms of pulmonary fibrosis. Make sure you receive the pneumonia vaccine and an annual flu shot. It's important that your family members also be vaccinated. Aim to avoid crowds during flu season.