The story of Pat
Over the last 20 years or so I have had recurrent pericarditis plus other issues, like increasing joint pains.
In 2014 had a harsh cough for a few hours and coughed up a small white stringy mucus but no more and didn’t think anymore about it.
2015 around December I had a few days of coughing up what I now know are bronchial casts. GP diagnosed a chest infection so antibiotics and it all settled again.
2016 I was having increasing bouts of harsh cough and more bronchial casts, one time was really scary as getting short of breath, tired due to harsh cough and more casts. We sent samples of casts and never found any infection. GP asked for advice from respiratory team at hospital who recommended asthma medication as there was a query that I was asthmatic. After trying this with little effect and bouts coming every other week, I was referred to the respiratory team at local hospital.
2017 In January I saw a respiratory consultant who thought maybe a variant of asthma. We send samples again for infection and to histology, which found casts were mainly lymph cells and few other cells, with comment that strange to be coming from lungs.
After getting no clues from tests so far it was decided to do a bronchoscopy when I was actively coughing up casts. Typically I didn’t have any bouts for few months, I started one at end of September.
A bronchoscopy was performed as a day case, they saw casts, took biopsies from lungs and after a few hours came home. I rested and slept okay that night and the next day I was having a quiet day.
After getting up the next day, I was okay initially but mid morning I suddenly had shortness of breath, coughing and fever. I ended up in hospital with pneumonia, joking I was one of the few to get pneumonia after a bronchoscopy.
After 2 days of treatment on the respiratory ward, I was deteriorating so transferred to intermediate care unit for few days. I continued to deteriorate so was transferred to ITU to be ventilated so they could assess more what was happening and why I was not responding to treatment.
A bronchoscopy was done which found my lungs were full of large, multi branched casts and lymph fluid. For the next week I needed one or two bronchoscopies a day, each taking 1-2 hours. I was totally unaware of this plus the transfer to another hospital for ECMO treatment. I also continued to have one or two bronchoscopies each day, but I did eventually start to recover. Plastic bronchitis was diagnosed and I was receiving a zero percent fat fluid via a nasal gastric tube.
After I was well enough to come of ECMO I was transferred back to first hospital, initially on ITU then onto respiratory ward.
That was 14 weeks of being in hospital after going in for pneumonia. We don’t know if the pneumonia and subsequent severe bout of plastic bronchitis are linked or why from coughing up what seemed big casts to me and hard to cough up to my lungs being filled with huge casts that took 1-2 hours to remove via bronchoscopy then fill back again.
I am alive and recovering/moving on from what has been a stressful and distressing time for my family, friends and I.Â
We are now trying to identify what has caused this rare disease, my treatment is very low fat diet and now daily antibiotic due to number of chest infections in last year.
So I am a survivor of rare disease going into apparent overdrive and knowing we may never find out a definitive answer as to why it developed as it did.