Living with Idiopathic Pulmonary Fibrosis in Malaysia

Last updated on August 28th, 2019

Ever notice that the ability to walk around freely and breathe in fresh air whenever you want is a blessing?

It is, because there’s a small fraction of people, for example, the patients living with Idiopathic Pulmonary Fibrosis – they just don’t have that privilege.

My dad was one of them.

Living With Idiopathic Pulmonary Fibrosis in Malaysia

It all started from the end of 2015

One day, my mom pulled me to a corner of the house and whispered in my ears.

“Hey. Is it common for someone at dad’s age to like…”

“Like what?”

“Having difficulties to pee?”

“What difficulties are you talking about?” I asked. My dad was 56 years old that time. I started recalling that male patients will slowly have prostate enlargement as they age. Everything is possible in medicine, but wouldn’t benign prostate hyperplasia in a 56-year-old man a little early?

“He always wets his pants a little after going to the toilet. He said it felt like he lost the ability to pass all the urine into the toilet bowl. It spilt out a little. So, some of it wets his pants..” Her voice trailed off, finding it a bit embarrassing to continue.

I told mom that we’ll help dad to arrange a urology clinic consult.

Dad refused. We sort of dropped the issue after a while.

What is causing the cough?

My brother was working in a hospital across the South China Sea. I was in University juggling with my medical studies. My sister was in the UK for her college.

Things looked normal. We were busy. We see dad and mom once every few weeks.

It looked normal, but something didn’t feel quite right.

Mom started giving us extra phone calls, telling us that dad was coughing more than ever. We asked mom to bring dad to a physician. We knew my dad smoked a lot during his younger days, so we thought it was COPD.

COPD is the Chronic Pulmonary Obstructive Disease, where there is a long-standing obstruction of lung airflow, very commonly due to smoking.

“Mom, it’s something we call the COPD. It’s a common disease that affects someone who smokes. It’s kind of like asthma, but not really asthma.”

I remember showing dad and mom YouTube videos of explanation for COPD, and how the medication prescribed by the doctor he went to will help. I explained that inhaling the medication will sort of open up the passageway in dad’s lungs for breathing. Those passageways might be narrowed in dad’s lungs.

“Hey Jenne, look at this,” Dad happily showed his inhaler to me. “See how this thing creates a hole in the capsule, and when I suck at the mouthpiece, the medicine will be sucked into my body.”

Dad followed the prescription religiously, sucking in bronchodilators that should help with his coughing.

Except that it didn’t.

And so we were wrong?

Few months passed by.

Strange, the medication didn’t seem to work, at all. It even looked like it aggravated his cough.

We went home and was shocked to find out dad was constantly wanting to sleep. Neglecting his meals, he’s saying that he’s too tired for everything. 

After taking a few bites of his regular meal, he told us that he would want to go to bed. He wanted to sleep after talking to us briefly. He didn’t even feel like walking to the bathroom to take his shower. He was tired all the time.

Something was off. We brought dad to another physician.

The true diagnosis

I wouldn’t forget that day. It still hurts me as I’m typing it out.

I was sitting in front of the CT Scan room with my dad and mom, waiting for my dad’s turn for a scan. Mom was crying. Dad was quiet in thoughts. This was the third doctor that we’ve gone to for my dad’s cough.

The doctor just told us a few minutes ago that it’s hard to say anything about it, and urged my dad to get a scan on that day itself. It’s likely not COPD. It’s likely something more serious.

The doctor kept quiet while he’s looking at the films from the scan when we’re back in his consultation room.

“I’m writing a referral for you,” he said. “I’m not an expert in treating this kind of lung disease, but I know someone who does.”

And while we were relieved that it’s not something nasty like cancer, it’s something almost equivalent – a disease with no apparent cause and medication to treat. A disease which can only be treated by a lung transplant.

Idiopathic Pulmonary Fibrosis

The diagnosis was so hard to accept. A disease that I have only learned in pathology lectures. A disease that we’ve learned to offer as differential diagnosis as a medical student when we heard fine crepitations at the base of lungs of the patient.

And suddenly, I had to accept that this rare disease is the cause of my dad’s symptoms?

I wouldn’t forget what my dad said to me that day, when he finally had supplementary oxygen connected to him via a nasal canula.

“Thank you, thank you so much,” he said to the doctor. His grayish face was finally turning red. His dark red lips were slowly turning to pink. He took in deep breaths, deep breaths, and more deep breaths.

We finally understood everything – the cough, the tiredness, the reason why the inhaler wasn’t working. His lung is simply hardened and lost some ability to utilize oxygen from normal room air. He needed more oxygen.

It’s pulmonary fibrosis, and even the treating physician didn’t know the exact cause of it.

The journey of living with Idiopathic Pulmonary Fibrosis in Malaysia

Here is some information about how my dad was living with Idiopathic Pulmonary Fibrosis in Malaysia, since his diagnosis. Again, this is not intended to be a substitute for professional medical advice, diagnosis, or treatment. This is solely shared for informational purpose only.

My dad was:

  • Put on an oxygen concentrator since diagnosis. He required an oxygen flow rate of 5 liters per minute.
  • Using portable oxygen cylinders for driving / eating out / outing. We had three at home back then, requiring refills every 2 to 3 weeks. Here’s a separate post I wrote about Home Oxygen Therapy in Malaysia.
  • On prednisolone and OFEV (Nintedanib). Nintedanib is extremely pricey. He was on it for about a year before he left us.
  • On follow-up for IPF with a respiratory physician. He had 6-minute walking tests and peak flow spirometry during his visits to monitor his disease progression.
  • Having bouts of cough (characteristic: loud, difficult to stop once initiated) that severely impaired his quality of life. We’ve tried everything that could work to stop his cough, but only certain cough lozenges and water boiled with ginger is providing temporary relief. (Again, not medical advice, for sharing purpose only)
  • Breathless upon walking short distances, and it was worrying that the distance that he was comfortable in walking gets shorter and shorter as the disease progress.
  • Turning down the option of lung transplantation because we were explained by the doctors that lung transplantation is not without risk (a lot of issues must be taken care of after transplantation as well, eg. graft rejection*, being put on immunosuppressants* that will bring other problems, etc) and that lung transplantation is just not that common in Malaysia (source). We’ve heard of successful lung transplant cases too, but we respect my dad’s decision to not going for it.
  • Living with idiopathic pulmonary fibrosis for 3 years and passed away on December 17th, 2018. It’s a 3-year-journey that taught my family a lot.

Read the questions that were frequently asked about idiopathic pulmonary fibrosis on this site here.

My message to you

  • If you found this article because you are living with Idiopathic Pulmonary Fibrosis, or that you have a loved one who is living with it, I want to give you a hug. Accepting it is difficult. Living with it requires so much strength that no one will know.
  • In Asia, IPF is a somewhat rare disease with an incidence rate of 0.6 to 3.4 per 100,000 person years* (source). It’s important that physicians keep this in their mind, but this might be a rare disease which some doctors never encounter in their whole practice.
  • A lot of IPF patients didn’t get their diagnosis right the first time. Nonetheless, spend more time with our parents and truly pay attention to what is happening around them.
  • If you are a caregiver to a patient living with Idiopathic Pulmonary Fibrosis, you have to buck up. Learn more about the disease in any way you can. Ask questions. Research. Comfort the patient. Make his life after the diagnosis a memory to cherish.
  • If you are living with Idiopathic Pulmonary Fibrosis in Malaysia – information might be scarce and difficult to find. Do not fret – Talk to your physician or other patients. Utilize the internet. Participate in online forums who talk about the same disease. Find support.
  • This article is last updated in August 2019 and will not be updated in the future. Information written here will be out-dated, but I’ll keep this few articles about IPF on my site as it connected me to many other patients living with IPF in Malaysia. Thanks for the comments and e-mails guys. Please take good care of yourself.

Dedicating this series of articles to my dad (1959 – 2018). Love you.

Are you taking care of a patient living with Idiopathic Pulmonary Fibrosis in Malaysia, or are you currently living with Idiopathic Pulmonary Fibrosis? Do share how you’re doing in the comment section below.