Last updated on September 27th, 2020
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. 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! Click to skip to the following questions directly:: #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: 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). 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. 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! 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! 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. 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! 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. 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: Sure thing. Here are some of the useful articles about IPF, which I refer to a lot when I’m writing this post. Dedicating this series of post to my father – who loved us and inspired me to start this site. Dad, this is for you.Frequently Asked Questions About Idiopathic Pulmonary Fibrosis
The IPF Series
#2 What is Interstitial Lung Disease then?
#3 What is the life expectancy of IPF?
#4 Do you know any respiratory physician near me?
#5 What is the treatment for IPF?
#6 Tell me more about lung transplant in Malaysia.
#7 What is your advice for patients living with IPF?
#8 Where can I buy oxygen concentrators or oxygen tanks and refill them?
#9 Where else can I find support?
#10 I am / I have a family member who’s living with IPF. How can I help more people living with IPF?
#11 I want to read more about IPF! Can you point me to some source?
1. What is Idiopathic Pulmonary Fibrosis?
2. What is Interstitial Lung Disease then?
3. What is the life expectancy of patients living with Idiopathic Pulmonary Fibrosis?
4. Which doctor was your father seeing / Do you know of any respiratory physician in _(an area)_?
5. What is the treatment for Idiopathic Pulmonary Fibrosis?
6. Tell me more about lung transplant in Malaysia.
7. What is your advice for patients living with Idiopathic Pulmonary Fibrosis?
8. Where can I buy oxygen concentrators or oxygen tanks and refill them?
9. Where else can I find support?
10. I am / I have a family member who’s living with IPF. How can I help more people living with IPF?
11. I want to read more about IPF! Can you point me to some source?
Do you have other questions in mind? Drop your questions in the comment sections below and I’ll try my best to answer.
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