Previously I had mentioned that my dad was going through an acute excerbation of IPF before he passed away. None of the doctors really identified it as such but I do not have any other words to describe his sudden decline in breathing capacity.
What exactly is an acute exacerbation ? Well, to put it simply, it is the sudden worsening of one's lung condition. This happens quite commonly with IPF patients at the end stage.
My dad was a pilot and he flew his last flight on 10th December 2012. He used to take O2 while sleeping at night but he could carry on with most day to day activities quite well. At this point he was taking just pirfenidone and some NAC for his cough. (the cough was the most irritating part!)
However, since we learnt about lung transplants at Global Hospital in Chennai, my dad decided to go there (we live in New Delhi) for an evaluation. My parents went to Chennai on 17th December 2012 for the evaluation. They did a full workup and also an angiography to check his cardiac health. They found two blockages, one insignificant and another was about 70%. The doctors at Global Hospital advised my dad that he needed to have a bypass surgery to undergo a lung transplant. His report said "Bypass surgery followed by Lung TX after 6 weeks".
He did not enjoy the Angiography. It's a rather painful process where they cut an artery in your arm (or leg) and put a catheter through it all the way to your heart. They check everything and take the catheter out. Then, they seal up your artery but it takes a few days to heal and can be quite painful. Unfortunately, he was upset with the result as he now had not one but two problems (IPF and a heart issue).
Furthermore, with a clear heart issue my dad could not fly any more. So he called up his employer and informed them of the news.
We took my dad to Apollo Hospital in New Delhi on 22nd December where the doctor ordered an echo, looked at the report from Global and said that he didn't need any intervention. Unfortunately, the doctor was busy so when we asked him about the lung transplant requiring a heart procedure first, he said "They will do it if they want to do it, but in my opinion, it's not required." It's always difficult when you get such answers because while dealing with such a serious sickness you often do not have the strength to deal with ambiguity.
So we decided to seek a second opinion. We went to Fortis Escorts Heart Insitute where the doctor advised us that the blockage doesn't look that serious (we had a DVD of the angiography that Global Hospital had given us). However, he said that he would like to do a procedure called an FFR (it's an angiography that measures some sort of pressure difference between arteries to see if there is a significant blockage). He said that if the FFR showed that the blockage was significant, they would immediately put in a stent. The stent would go in on the same catheter so there would be no need for another procedure. We scheduled the procedure for 8th Jan 2013.
Through this time, my dad used to take the dog for short walks, he could drive and go to the market, and while he was always a little breathless - he could manage things reasonably well.
On 8th Jan 2013 my dad was admitted to Fortis Escorts for the stenting. On the morning of the 9th, they performed the FFR and put in his stent as the blockage was above the margin (just by 0.5%). My dad spent another day in the hospital and then came home. The next day, he felt like a new man so he went for a big walk with the dog. The day after that he was flat out on the bed and didn't have any energy at all!
This is where my dad's serious decline started. He had increased his nightly O2 from 2.5L to 4L/min. He still didn't use O2 during the day but felt increasingly breathless and could walk only short distances before needing rest. He couldn't walk the dog anymore. The stenting had apparently caused some adverse reaction because now he seemed much worse than before.
We went back to the doctor who had done the stenting. He ran a few tests and everything was fine. He said the stent was working properly and my dad's heart was fine. Everytime I mentioned that his condition was worse after the stenting the response was "Oh well, he has IPF so it's normal to get worse..". It's strange that doctors in India have very less experience with IPF. One of the first heart doctors we met in Apollo asked my dad "Oh, how did you get this IPF ?". I wanted to say "Do you know what Idiopathic means ?" but I refrained myself. Several doctors have asked similar questions along the way including some pulmonologists! (shocking, I know!) I tried to explain to the doctor that my dad wasn't declining at such a rate prior to the stending, but the doc was already busy thinking about the next patient who was going to pay him Rs. 1000 + tax for a 15 minute consultation.
So we went back home and tried to deal with the situation. We decided to go see another pulmonologist in Apollo, New Delhi. One of the heart specialists had recommended him as "the best in the country". When we asked him about whether we should get a lung transplant he seemed to not know about the procedure happening in India. Not only that, but he recommended against it. He said "Oh sir, when your DLCO falls below 35% only then we'll look into it".
In all of these days, no one had mentioned the words "Acute Exacerbation". It was only through my research online that I found out about this. I was so scared. Did we mess up my dad's health more by performing the stenting ?! Why didn't the doctors say that something like this could happen ?
Well, the short answer is "the doctors wouldn't know". This is the worst thing about IPF - we do not truly understand it's nature, progress, and speed. In my dad's case, he was going through an acute exacerbation. This became evident when in February I had to buy a portable O2 concentrator just so that he could walk around comfortably. But by the end of February not even the 2.5L of pulse flow portable oxygen was enough for him. He needed 5L of continuous O2 to walk around - we got him a long tube for his big concentrator. In December, my dad had walked into Apollo hospital to see the doctor. In February, I had to take him in on a wheelchair. Yet, the doctor looked at his X-Ray and said "There's no acute exacerbation - it looks okay". I don't think he knows how to read an X-Ray or maybe an X-Ray is not the right tool for the job. The days running up to my wedding were interlaced with visits to incompetent and impatient doctors in New Delhi. Unfortunately, it was all for nought.
On 5th March 2013 my dad passed away and even in the ICU in the Army Hospital R&R, the pulmonologist was not sure of whether this was an acute exacerbation or not. He was not sure if it was an infection or something else. They had run several tests since my dad was admitted there for the past 2 days. They had cleared his heart of any issue. He needed 10L of continous flow O2. His condition had worsened a lot over the night of the 4th and on the 5th even with 11L of continous flow O2 he could not maintain over 70% saturation. When I saw him in the ICU on the morning of the 5th, he was delirious due to the lack of O2. When I went up to him, he just opened his eyes momentarily before he went back to groaning and trying to breathe. The doctor in charge of the ICU asked us if we wanted to try mechanical ventilation.
This was the moment that I had to make the toughest decision of my life. The decision to let my father go. I had read everything online. IPF patients respond poorly to mechanical ventilation. The procedure is traumatic and the outcome is poor. After all, it's not like my father will take mechanical ventilation and suddenly be cured of his IPF. He would still struggle to breathe, even if he managed to make it out of the ICU. The doctor in the ICU also agreed that IPF patients should not be mechanically ventilated. There's no point.
My mother was with me and already crying. I asked the doctor what we can do to make him comfortable. She said that we can give him morphine and he will be peaceful and comfortable. I told her to do it. I asked her if we had to sign some sort of consent form but she said verbal consent from family is enough.
I have no doubt in my mind that my dad's sudden and sharp decline was due to an acute exacerbation of IPF. This phenomenon is well documented in IPF research and I have read a lot of things about it. Unfortunately, it appears that medical professionals in India seem to be quite unaware of it. They are bogged down treating regular varieties of pulmonary disease (TB, Pneumonia, etc.) that IPF doesn't fly too high on their radar. So what caused my dad's acute exacerbation ?
- Was it the chart busting pollution in New Delhi ? (my parents were living in Coimbatore until September 2012)
- Was it the initial angiography in Global Hospital ?
- Was it because of periods of hypoxia while my dad was not on O2 ?
- Was it the stenting procedure ?
- Was it something else ?
Unfortunately, we will never know why these things happened the way they did. However, the ultimate truth is that my dad went from walking a few kilometers in the trade fair in November 2012 to not being able to walk on 10L of O2 in March 2013. He knew he had IPF for 6 years but his decline in the end was very sharp.
It's important for IPF patients to understand that acute exacerbations can happen at anytime and to take proper care. It's also important to find a competent doctor, if you know anyone, please leave his info in the comments - it will help other people IMMENSELY. I could not find a good doctor in the top hospitals of New Delhi (I only tried Fortis and Apollo).