One Step at a Time: Kumar’s Journey with Idiopathic Pulmonary Fibrosis

By Dr. Priya Sammani

I’ll never forget the day Kumar walked into my clinic. There he was—Uncle Kumar, as I used to call him, the man who drove me to school every morning, rain or shine, in his old blue van. Kumar had always been full of energy, sharing his big dreams of becoming a tour guide and exploring Sri Lanka’s hidden beauty spots with travelers. And he did just that! For years, he was one of the most passionate tour guides, leading people through breathtaking landscapes and historic ruins. But something was different about him that day; his smile was there, but it was strained, and I noticed how he seemed almost breathless just sitting down.

It was then that he shared his story, and little did we know, it would be a story that so many others with idiopathic pulmonary fibrosis (IPF) would relate to.

The Start of Something Strange

Kumar told me how it all started. At first, it was just a small shortness of breath. He brushed it off, figuring he’d been pushing himself too hard or maybe just needed a break. But as weeks turned into months, the breathlessness grew, and he started feeling more tired than usual. The worst part was the persistent dry cough that would catch him off guard, even when he was talking to his tourists.

“I kept thinking, it’s just age catching up, right, doctor?” he asked, half-smiling.

I could tell he didn’t want to worry, but I knew this sounded more than just age. With his symptoms lining up, it was clear to me that he needed to see a specialist for a closer look. I recommended that he meet a pulmonologist—someone trained in lung diseases—to get to the bottom of his condition. And that’s where Kumar’s journey with idiopathic pulmonary fibrosis began.

What Exactly is Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis, or IPF, is a rare and serious lung disease. To put it simply, IPF causes the lung tissue to become thick and scarred over time, which makes it hard for the lungs to expand and take in air. This stiffening of the lung tissue makes it harder to breathe, and that’s why someone with IPF feels out of breath, even with minor physical effort.

The cause? That’s the tricky part. The word “idiopathic” actually means unknown. Despite advancements in medical research, doctors often can’t pinpoint what causes IPF. There are theories, of course. Some suggest it could be due to environmental factors like pollution or exposure to certain chemicals, while others suspect that genetic factors might be at play. But for most people diagnosed with IPF, no clear cause is found. If doctors can’t identify a reason for the lung scarring, they label it idiopathic.

IPF isn’t the only lung disease that causes breathing issues. For example, chronic obstructive pulmonary disease (COPD) also affects lung function but in a different way. Understanding the differences between IPF and other lung conditions like COPD is essential for getting the right treatment.

Learn more about idiopathic pulmonary fibrosis and its causes here

The Symptoms: More than Just Breathlessness

By the time Kumar came to see me, he was experiencing the classic idiopathic pulmonary fibrosis symptoms: breathlessness, a dry cough, and fatigue. But other symptoms can develop as the disease progresses, such as:

  • Chest discomfort
  • Unexplained weight loss
  • Weakness and tiredness that don’t seem to match the activity level

What really alarmed him was how quickly his breath would vanish with even light activity. Tasks he once breezed through, like guiding tourists up a short hill, now left him feeling exhausted. And unfortunately, IPF is a progressive disease, meaning these symptoms often get worse over time.

The Stages of IPF: A Gradual Progression

Idiopathic pulmonary fibrosis doesn’t progress in neat, clear-cut stages like some other conditions. But, generally, patients go through different phases based on how much their lung function is affected. When Kumar’s pulmonologist explained this to him, he could see his own experience in each phase:

  1. Early Stage: For many people, IPF starts with mild symptoms, like a light cough or slight shortness of breath with activity. Some people might not even notice it much at this point.
  2. Moderate Stage: As the lung scarring increases, the symptoms become more apparent. Physical activities, even moderate ones, lead to shortness of breath, and some patients may need extra oxygen while being active.
  3. Advanced Stage: In this stage, scarring in the lungs is severe. Patients often require oxygen, even while resting or sleeping. For some, high-flow oxygen machines become essential for maintaining normal oxygen levels.

Kumar realized he had already moved beyond the mild phase. He often had to pause and catch his breath on tour, and his dry cough had become an unwelcome companion. As his pulmonologist shared with him, IPF can progress slowly, but it can also have sudden “flare-ups” when symptoms worsen drastically. This, he learned, is why getting the right idiopathic pulmonary fibrosis treatment early is essential.

Discover more about the progression of IPF

Diagnosing Idiopathic Pulmonary Fibrosis

Getting a diagnosis of IPF isn’t always straightforward. Kumar’s pulmonologist explained that IPF shares symptoms with other lung diseases, making it tricky to identify without detailed tests. Kumar went through a series of examinations, including:

  • Chest X-ray and High-Resolution CT Scan: These scans show lung scarring, which is a major sign of IPF.
  • Pulmonary Function Tests (PFTs): These tests measure how well the lungs work and how much air they can hold.
  • Oxygen Level Tests: Checking his blood oxygen levels during physical activity helped understand how much support he needed.
  • Lung Biopsy: In some cases, like Kumar’s, a biopsy is done to confirm the diagnosis and rule out other lung diseases.

Once Kumar received his IPF diagnosis, he realized that although the news was hard to hear, understanding the disease was the first step in managing it.

Treatment Options: Learning to Manage IPF

Kumar was eager to know what his options were. “Can it be cured, doctor?” he asked, hopeful yet aware that IPF sounded serious.

There is currently no cure for IPF. However, treatment can help slow down the progression of the disease and improve quality of life. Here’s what Kumar’s treatment plan looked like:

  1. Medications: Two FDA-approved medications—nintedanib and pirfenidone—can help slow lung scarring. These won’t reverse the damage, but they can delay the disease from worsening too quickly.
  2. Oxygen Therapy: Since IPF affects oxygen intake, many people with IPF need supplemental oxygen. For Kumar, using portable oxygen became essential, especially during physical activities.
  3. Pulmonary Rehabilitation: This program taught him breathing exercises and techniques to improve lung function. The rehabilitation also provided him with strategies to conserve energy, allowing him to still enjoy outings, even if he had to take it slow.
  4. Lifestyle Adjustments: Kumar quit smoking and made sure to stay up-to-date with vaccinations, protecting himself from respiratory infections that could worsen his condition. Maintaining a moderate weight also became important to reduce the strain on his lungs.

Read more about IPF treatment options here

Lifestyle Changes: Finding a New Rhythm

Kumar had to rethink his lifestyle. He still loved nature and took slow, meaningful walks with his camera now in hand instead of his tour guide badge. Photography became his new way of sharing Sri Lanka’s beauty with others. If you’re living with IPF, adjusting your lifestyle might feel overwhelming at first, but small changes can go a long way. These include:

  • Avoiding Smoke and Pollution: Staying away from smoky areas or places with heavy pollution helps prevent additional lung irritation.
  • Regular Health Check-ups: Keeping up with routine check-ups can help monitor the disease and address any new symptoms early.
  • Joining a Support Group: Talking to others going through similar experiences can provide support and reduce feelings of loneliness.

For Kumar, these lifestyle changes became more than just adjustments—they were his way of still living fully, even if life now moved at a slower pace.

Life Expectancy and Outlook

IPF is a progressive disease, meaning it worsens over time. While some people may live with IPF for years, the average life expectancy after diagnosis is typically between three to five years. This varies depending on factors like age, general health, and how quickly the disease progresses. For those in advanced stages, lung transplant surgery is a possibility and the only cure, though it’s a major operation with its own risks.

Kumar understood the challenges but found peace in taking each day as it came. He focused on what he could control—like staying active in his way, spending time with family, and using every moment as a chance to connect.

Explore more about life expectancy and IPF here

The Takeaway

Living with IPF is no easy journey, but Kumar’s story reminds us that even in the face of adversity, life can still be beautiful. If you or someone close to you is experiencing symptoms of IPF, reach out to a doctor. Early diagnosis and treatment can make a real difference, allowing you to take control and live a good quality of life. Kumar’s journey shows that with the right mindset, treatment, and support, one can find purpose, one step at a time.