Frequently Asked Questions About Idiopathic Pulmonary Fibrosis

Last updated on September 28th, 2020

Ever since I started this blog, I had the pleasure to (virtually) meet all of you, mostly living with / having a family member living with Idiopathic Pulmonary Fibrosis.

It’s Magical.

Imagine meeting a lot of people going through the same disease and have a sense of support. That we’re not alone.

Every time I read through the emails that I’ve received and comments you guys left for me in the comment section, it’s a blessing in disguise.

That what my father went through opens up a door for me to connect to the world.

The IPF Series

My father passed away on 17/12/2018.

We still miss him a lot.

I think this is the time that I put an end to this series of articles about living with pulmonary fibrosis in Malaysia. The information I’m providing will be dated compared to you guys who are actively going through it with your family members, and consulting doctors about it.

Before I end this series about pulmonary fibrosis, let’s just compile some of the questions I frequently receive, so that if you know anyone is going through IPF, you can point him / her to this article!

1. What is Idiopathic Pulmonary Fibrosis?

Breaking the phrase down to 3 parts, ‘Idiopathic’ means ‘of no apparent cause’. ‘Pulmonary’ points to things related to the lungs. Fibrosis is a process of scarring, hardening.

In short: scarring, hardening of the lungs with no apparent cause.

The simple way to explain it will be: Let’s say in the beginning the lungs are soft, expanding and recoiling easily as we breathe.

When someone has IPF (or pulmonary fibrosis in general), the lungs are scarred and hardened with time, leaving a stiff lung. When he / she breathes in and out, gas exchange didn’t occur as effectively as it should be.

Hence over time, the patient becomes breathless (and the breathlessness progressively worsened) and blueish (in medical terms, we describe the patient as cyanosed).

Watch this video about IPF to help you understand more!

Additional information about IPF:

• IPF is more prevalent in Europe & North America (3 to 9 cases per 100,000 person-years).
• Patients usually show symptoms of unexplained exertional dyspnea (breathlessness upon strenuous activities), chronic dry cough, or Velcro-like crackles on examination (fine crackles like we remove a velcro strap when the doctor listen to the lungs of a patient using stethoscopes)
• A disease that is easy to be misdiagnosed (Ask the patients whom you know living with IPF, very few get the diagnosis at the first healthcare encounter)

2. What is Interstitial Lung Disease then?

Let’s take a minute and talk about the lungs again. Most of us know lungs are made up of the respiratory tracts (imagine the branches of a tree) and the lung parenchyma (think of the fleshy part of the lung, or the leaves of a tree). At the end of each small tracts named bronchioles are the small balloon-like structures called alveoli.

Imagine that these small little balloons have tissues and space around them, which we call the interstitium.

When there are triggers that cause injury to the lungs, the lungs produce the appropriate amount of tissue to repair the damage.

However, in ILD, the process went wrong and even the little balloons called alveoli is scarred up.

How is this different from IPF? ILD is a term that includes many diseases including IPF. I’ve created a graphic below. As you can see, the Interstitial Lung Disease is the big umbrella term in the center, and IPF is one of the disease under it (located at far left at the top of the image).

3. What is the life expectancy of patients living with Idiopathic Pulmonary Fibrosis?

The median survival is 2 to 5 years from the time of diagnosis. My father passed away 3 years after the diagnosis.

The long answer will be: “It depends.”

I still believe that for IPF patients with fewer events of exacerbation (an event happened that led to fast deterioration of the disease, which usually meant more scarring), the life expectancy might be longer. This presentation about the Step Theory of UIP / IPF progression will tell you more.

4. Which doctor was your father seeing / Do you know of any respiratory physician in _(an area)_?

You’ll be amazed by how many people had asked this to me. My father was previously seeing a respiratory physician in one of the University hospital in Kuala Lumpur, but the doctor is spending more time in private practice for now.

Unfortunately, I have no idea about respiratory physicians in other hospitals. Ask that during the follow-up with your treating doctor and you shall get more information from there!

5. What is the treatment for Idiopathic Pulmonary Fibrosis?

I have to be brutal here – there’s no treatment for IPF at the point of me writing this, other than lung transplant (more on that in the following question). We only have treatment to slow down the progress of the disease.

Let’s break it down to pharmacological and non-pharmacological methods (i.e involving medication or not). For non-pharmacological ways, it involves smoking cessation, supplementation oxygen, pulmonary rehabilitation.

For pharmacological methods, Pirfenidone and Nintedanib had made a lot of advances in controlling disease progression in mild-to-moderate IPF patients since their introduction. So far, there’s no recommendation of one medicine over the other.

As for my father, he was started on Nintedanib, which had a high price of around RM600 per pill (twice a day, you do the math). I am not in a good position to discuss the current price of the medication, your treating physician might give you a better answer!

6. Tell me more about lung transplant in Malaysia.

As of what I know, heart and lung transplantation in Malaysia is still not as common. The most complete statistics that I can find is only from the National Transplant Registry, but the latest stats are only until 2015.

Do download the “Chapter 3 Heart and Lung Transplantation” on the page and have a look starting at Page 12. It’s a little dated, but it’s a very valuable set of numbers we can look at.

First lung transplant in Malaysia is started in 2005, and until 2015, there’s only 10 patients underwent lung transplant (including heart and lung transplant). It’s a valuable resource as it includes the number of patients alive at 31st December of each year.

I’ll advise that you don’t solely decide on whether to go through lung transplant or not based on the figures in the registry, or just some blogs about how people are going through lung transplant – it’s a high-risk procedure.

There are so many other factors that play roles in the recovery of the patient (patient’s risk factor, health condition, the occurrence of rejection towards the new organ, etc). It’s a procedure that requires advance specialty in the operation and post-operative care.

Talk to your treating physician to know more about lung transplant, the benefits and the risks associated, most likely they will refer you to IPR (the Institut Perubatan Respiratori) in Kuala Lumpur for a thorough assessment and discussion (like what my father went through, but in the end decided not to go for it).

Here’s more information about lung transplant in Malaysia by the Ministry of Health.

7. What is your advice for patients living with Idiopathic Pulmonary Fibrosis?

It’s a long journey. It will put you down. Not in a good position to offer advice as I’m not the one living with the disease but in general, it will be helpful if you:

Over to you – what’s your advice for patients living with IPF if you’re caring for one? Let me know!

8. Where can I buy oxygen concentrators or oxygen tanks and refill them?

I wrote a guide about home oxygen therapy in Malaysia! Feel free to leave a comment / let me know more of the suppliers / shops offering the service so that I can update the page and benefit more people living with diseases needing oxygen therapy at home.

9. Where else can I find support?

Living with Idiopathic Pulmonary Fibrosis brings a rare diagnosis, a lot of unknowns and new care-taking responsibilities. Sometimes I felt like I needed some time away.

It’s hard on the patient as well as the caretaker.

I can’t even imagine how my father was feeling living with the disease. Here are some ways we coped with it:

• Talk to family and friends. I always see that my father’s mood lit up after a relative’s visit, a family dinner or a good phone call.
• Ask your treating doctor a question you are keeping. They are seeing patients with a similar condition as yours and will have useful information to offer. (My father was always anxious attending follow-ups so I did a small note of what questions to ask each time we were attending one)
• I’m having this symptom, is this normal?
• What do I have to look out for?
• How’s my disease progressing?
• I’ve always found reading other people’s experience of living with the disease useful. If you’re the same, consider joining an online support group which you can interact in forums like Inspire (a pulmonary fibrosis support group by the American Lung Association). Blogs about patients’ story eg how John survived a lung transplant and how Jacqui is living with pulmonary fibrosis are very helpful too.
• If you’re a caretaker of someone living with IPF, find time for yourself. If another family member is around, take some time off. Speak your feelings out. I found The Caregiver Space Community group on Facebook and it helped to know that there are a lot of other people out there being a caretaker for someone they care for. They offered advice and suggestions that provided support to me through the period I took care of my father.

10. I am / I have a family member who’s living with IPF. How can I help more people living with IPF?

• In the case that you / your family are in the setting of a hospital offering training medical students, do not hesitate to let them examine you / ask you questions. Remember, the more medical students encounter patients, the better they become in picking up the signs and symptoms. The eyes see what the mind thinks.
• Share your experience. Believe this, the experience of living with a rare disease is a very precious piece of information to other patients / their family. If you are not comfortable with writing / blogging, start by answering questions of a new patient whom you met in the clinic.
• After you are being informed of the risks and benefits comfortable with it, consider joining a clinical trial if there’s any. IPF is still at the very early stage of research and could use more patients as study samples. Here’s Why We Need Idiopathic Pulmonary Fibrosis Research Now More Than Ever.
• As for me, my father had inspired me to start writing this series of articles, and we’ve donated out his last box of Nintedanib to a reader whom I connect through this website. We’re planning to donate out his oxygen concentrator and tanks too, but we’re still finding organizations to accept them. (Let me know if you know anyone needing them!)

11. I want to read more about IPF! Can you point me to some source?

Sure thing. Here are some of the useful articles about IPF, which I refer to a lot when I’m writing this post.

1. Idiopathic Pulmonary Fibrosis, a review article from the New England Journal of Medicine
2. Exertional dyspnoea in interstitial lung diseases: the clinical utility of cardiopulmonary exercise testing from the European Respiratory Review
3. An update on the management of Idiopathic Pulmonary Fibrosis, a SlideShare presentation by Dr Sarfraz Saleemi packed with trials conducted for IPF
4. Why We Need Idiopathic Pulmonary Fibrosis Research Now More Than Ever by the American Lung Association
5. Idiopathic Pulmonary Fibrosis, an article from the Orphanet Journal of Rare Diseases
6. Pulmonary Fibrosis Overview, an article by the Pulmonary Fibrosis Foundation
7. Lung Transplantation, a web page by the Ministry of Health Malaysia

Dedicating this series of post to my father – who loved us and inspired me to start this site. Dad, this is for you.

Do you have other questions in mind? Drop your questions in the comment sections below and I’ll try my best to answer.