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My IPF Journey

19 March, 2021

by Karen Simmons

At the end of 2013 my father passed away from Lung Cancer.  My mum, sister and I granted his dying wish that he may pass away at home and not in a hospice, however during that journey my mother had collapsed and was taken to hospital.  My sister and I assumed it had been brought on by the stress of caring for my dad, but aged 74 she was diagnosed as having Idiopathic Pulmonary Fibrosis.  We had never heard of it and although mum was sent home with leaflets, she ‘filed’ them and we didn’t really discuss it any further at that time as she recovered and carried on.  She was prescribed Omeprazole for acid reflux which she found helpful, but nothing else.

Then in February 2014 when I was 52, I took myself to the GP with what I thought was a chest infection.  I felt ‘low’ following the death of my Dad and assumed I had picked up an infection as a result.  My GP told me it wasn’t an infection but by the sound of my voice, he determined that I had acid reflux and also prescribed me with Omeprazole. I hadn’t realised how bad my indigestion/heartburn was until I took these ‘wonder’ pills!

I should say at this point that my work colleagues had noticed that I was coughing….… a lot! It was a dry, harsh, non-productive cough and very annoying for me and especially them.  I called it my “silly cough”.  I saw the GP again, this time about the cough.  He was due to retire shortly and said my chest sounded clear and as I had no other signs of infection, he suggested co-codamol for the cough.  I declined and went away.

A year later, I needed to be referred for a surgical procedure on my bladder which required me to attend for some physiotherapy beforehand.  The Physiotherapist was quite concerned that in the 45-minute consultation, I had coughed incessantly, and she suggested that the surgery could not proceed until the cough had been addressed. So back to the GP I trekked. 

I underwent spirometry tests with a healthcare assistant at the surgery and was given an FEV1 of 86% and told this was perfectly normal, but to be sure I was referred for a walk in chest x-ray at the hospital. Again, ‘normal’.  So, missed markers so far were acid reflux, dry persistent cough, and a couple of chest infections, something I had never suffered from following everyday colds up until now.

In October 2015, my “silly cough” was very annoying, so I went back to the GP again.  My original GP had retired by this time and a new, younger chap had taken me on.  He listened to my chest and decided that, although he couldn’t hear anything, perhaps it should be investigated further and his exact words were, “I’m just a GP so we’ll refer you to the Respiratory Department at the hospital”.  What followed was another chest x-ray and a CT Scan, and on 13 December 2015, I was told that the CT Scan showed scarring of the lungs consistent with IPF (like Mum has).  I was told there are two medications available to slow the progression of the disease but to qualify, I would need to have a lung biopsy.  The Consultant said “which, of course you will agree to won’t you in order to get the drug”! I agreed. 

In my naivety, I had assumed it would be a needle biopsy carried out under a local anaesthetic.  Wrong! The paperwork arrived for Video Assisted Thoracic Surgery (VATS).  So, in February 2016, I had the surgery and then spent 5 days in the Norfolk and Norwich Hospital.

I then attended an appointment with the Consultant to be told that following my VATS Biopsy, my diagnosis had been changed from IPF to Hypersensitivity Pheumonitis which meant that I did not qualify for one of the two drugs but would be put on a high dose of steroids.  I didn’t understand.  I was confused.  I was disappointed.  I had agreed to the biopsy to get this wonder drug that would help.

According to the NICE guidelines Pirfenidone or Nintedanib is prescribed only for IPF if your FVC is between 50 and 80.  As I had a diagnosis of Hypersentitivity Pneumonitis I did not qualify.  My brain was fogged.  If my fibrosis was idiopathic, in other words ‘cause unknown’ that would allow me to have the drug.  So, they must know what has caused the hypersensitivity pneumonitis and therefore treat it.  Wrong again!  He said we know that the pneumonitis is causing the fibrosis, so the fibrosis is not idiopathic, we just don’t know what is causing the pneumonitis.  I was even more confused.  To my mind then, by definition, surely that meant it was ‘idiopathic’.  However, I agreed to the steroids. 

For six months, I was like a woman possessed.  I now knew what it meant to be “on steroids”.  40Mg per day and I was a whirlwind.  Constantly on the go.  My work colleagues said I made them feel worn out just watching me, it’s just a shame the steroids didn’t get rid of the annoying cough!  After 6 months, I had to conclude that apart from the euphoria from the steroids, they didn’t seem to be helping my condition.  My Consultant suggested we up the steroids to 60mg per day.  I was unsure and phoned my mum with the bad news.  By coincidence, she had an appointment at Papworth Hospital and mentioned my case to her Consultant.  He offered to give me a second opinion if required.  I discussed this option with my GP and he was unhappy for me to be put on such a high dose of steroids.  He felt it would be detrimental to my other vital organs so, at my request, he referred me for a second opinion at Papworth.

So, once again a full history was taken, and I was also referred to Addenbrooke’s 100,000 Genome Project looking into the genetics of rare diseases.  The pathology from my biopsy and test results were requested from the NNUH and following a Bronchoscopy at Papworth, on my Mum’s 78th birthday, I was finally given a diagnosis of IPF in the lower lungs and hypersensitivity pheumonitis at the top. Disappointingly, I had both, but it meant that I was eligible for those elusive anti-fibrotic drugs.

In the meantime, during March of 2017, having been quite well without any real effects from her condition, mum had become quite breathless and was prescribed ambulatory oxygen therapy and a CT scan was ordered.  By the end of April, when she came to stay with me, she was really starting to struggle, and two admissions followed to the NNUH.  She subsequently returned home to Cambridge and a further 999 call gave us the news we didn’t want to hear.  Finally, following the CT scan, it was discovered that mum had a significant number of clots on her lungs and after 9 weeks on the respiratory ward, mum was transferred to the Arthur Rank Hospice where she spent her 78th birthday.  The very same day I got my diagnosis from Papworth. 

She cried.  She was so upset to think that I had what she had but I was 21 years younger than her.  She said it was so unfair.  Two weeks later my mum passed away peacefully with me, my sister and husband by her side.  The hospice nurses were absolutely wonderful.  They were aware of my diagnosis and gave me an extra hug knowing that I was now “walking in my mother’s footsteps”.

It was hard initially, to not only come to terms with the loss of my mum, but to also know first-hand  the journey I was now on.  I struggled.  Why us? Why Me? What had caused it?  Mum never smoked, only drank occasionally and had no exposure that we were aware of.  I was similar (apart from the fact that I had smoked in my younger years).  We were both fit before diagnosis and led a healthy lifestyle.  The one thing we both had in common was premature whitening of our hair.  I was 21 when I started to go white and was completely white by the age of 30.  Apparently, this has been noted by Consultants previously as a possible factor.

I broke down at my appointment with the Respiratory team at Papworth in the September.  Everything was so raw, but my wonderful nurse reminded me that whilst there was no treatment available for my mum, as my FVC was 78, I could start the anti-fibrotic drugs.  I chose Pirfenidone. She also reminded me that because of my age and current state of health, I could possibly be considered for a lung transplant, should I decline.

In that moment, my mindset changed.  I will start the drugs. I will keep myself as fit and healthy as possible.  I will do everything in my power to help myself.  I decided my mantra would be “Accept the diagnosis.  Fight the prognosis!”

I decided to resign from my job in November 2017.  I walked our Cocker Spaniel 5 miles every day, come rain or shine, snow or wind, even the ‘Beast from the East’ didn’t stop me going out.  I was determined to spend as much time as I could with my husband, two girls and 5 grandchildren and to live life.  My husband and I had the cruise we had always promised ourselves in September 2018 and took a train journey to Italy in May 2019.

However, by the end of 2019, I was starting to struggle and finally, and reluctantly, I contacted my respiratory team at Papworth in January 2020 to say I felt I was beginning to struggle.  That prompted an emergency appointment and I came home with ambulatory oxygen and a referral to the Transplant Team at Papworth.  Following my initial appointment, I was required to have an angiogram and pH test (of the reflux) prior to transplant assessment. Then came COVID19.

Needless to say, those tests were delayed.  However, in August 2020 I had the angiogram and pH tests and went for my two-day transplant assessment in September.  Following all the usual tests which were conducted with military precision and such understanding and kindness, I was told I was a suitable candidate to go on the transplant list.  My FVC had declined to 42 from 58 in the February.  I went live on the transplant list on 16 October 2020. 

Since then, my oxygen needs have increased significantly.  I can no longer walk Tilly on my own, and only manage a short stroll around the village whilst needing 8 litres of oxygen.  I am trying to keep myself as fit as possible given the constraints of my breathlessness and have been doing Pulmonary Rehabilitation online.  I just hope and pray that I will be lucky enough to be given the chance of a transplant.  I am aware that, due to my lungs being so small, the possibility of a double transplant will be “challenging” so I will be considered for a single lobe should one become available.  My life is now in the hands of fate, but I will never give up hope and will do everything within my power to keep going.  Accept the diagnosis, but fight the prognosis!

UPDATE: Since originally writing this, Karen received a right lung transplant on 1st February. She returned home on 27th February but has since been re-admitted to hospital where she is currently in ICU fighting an infection.

Our thoughts are with Karen and we wish her a swift recovery.