r/pulmonaryfibrosis

My dad was diagnosed in 2022 and he’s had a lot of up and downs and has always kept such a positive attitude.. like truly blows our mind how he overcomes so much with his positive attitude.

But now after 2 strokes, losing his job and his lung disease progressive quickly this year he’s really struggling mentally but really doesn’t like to show it.

I live 3 hours away so I come into town only once a month on the weekend

Last time I was there he was on 3L of oxygen at rest and now he’s up to 12L at rest with my step mom having to plug in two machines when he has to get up to go to the rest room she has to get him on 25L.
She said it’s progressing so fast and he developed the IPF cough that has been miserable for him.

She has so much anxiety at night with him coughing and thinking he’s choking or running out of oxygen.

So scared for him, sad for him that he’s struggling so much and nothing I can do to help.

I saw my Respiratory Consultant today. He confirmed EOL due to the use now of continuous oxygen saying its usually a good indicator of life expectancy of less than 12 months. He showed me the graph of my breathing tests from 2022 when investigation began to date. There is a very steep drop the first 18 months in fvc, then i begin Nintedanib and the deterioration flattens out for 12 months then begins to deteriorate again but at a much slower pace. The conclusion is that the nintedanib is working slowing down the progression and even now I am in EOLC he says it is still worth taking so long as quality of life is there. We agreed on that.

I am to discuss with Palliative Care reducing some of the other drugs that will not be detrimental in my life expectancy timescale.

With my partner, even though we expected the news we felt positive about it and have time to plan to do stuff together.

So, the timeline for those interested is detailed below. For those interested I hope this helps.

2020 August coughing
2021 March GP
2021 April X-Ray - negative
2021 June - CT scan
2021 July - referral to Respiratory Consultant
2021 November - Diagnosis PF/Asbestosis Prognosis 2-5 years
2021 December - Civil case started
2022 May - tlco 64%
2023 August - begin nintedanib
2023 December - FVC 85% tlco 57%
2024 July - begin using ambulatory oxygen
2024 September - FVC 80% tlco 57%
2024 November - referred to Palliative Care Consultant
2025 January - begin using Oramorph
2025 April - Respect form added to CPR/DNR record
2025 June - MST slow release morphine 10mg prescribed
2025 July - Civil case concluded
2026 June - End-of-life-care
2026 July - FVC 56% tlco 36%

FVC - 35% tlco - 37%

My first encounter with black toxic mold in wet plywood was in 2004. I was in ICU and nobody at Yale Medical School could diagnose my condition.

Next in 2005, I was in the ICU for the same symptoms - unknown respiratory infection.

That year, the doctors at Providence prescribed Leviquin, a Johnson and Johnson product that caused my skeletal system to produce cysts on many of my joints.

I had a test for rheumatoid arthritis but the doctors did not diagnose that.

I took some of the old osteoarthritis meds by prescription that were eventually banned.

In 2008, I was teaching in a classroom that had old water damage in the ceiling. Again, I went to ICU. This time, I was put in a medically induced coma.

In 2009, I was diagnosed with hypersensitivity pneumonitis and put on Prednisone.

I was disease free as long as I avoided the black mold.

In 2018, my primary care physician noted that I was being treated for psoriasis by a dermatologist. He suggested I have another test for psoriatic arthritis.

I was diagnosed with that condition and put on a large regimen of Methatraxate. That drug cleared up my psoriasis and my arthritis symptoms.

In 2024, I started noticing allergy symptoms that seemed seasonal. There was no black mold in my environment. But I did develop chronic shortness of breath.

Now, I have been treated by a rheumatologist who tells me that that my Methotrexate is causing my shortness of breath. She wants to put me on Humira injections that cost between $1000 and $4000 per month.

I suggested that I simply need to reduce my Methotrexate to the point that my dosage reduces my shortness of breath but maintains the benefit of treating my arthritis.

Does anyone this sound familiar?

Thought I would post an update on my condition.
Had walk test for Pulmonary Rehab a month ago. Failed on pulse O2 but passed on continuous.

Began using continuous O2 24/7. Oxygen machines installed and O2 piped around the house.

Started Pulmonary Rehab month ago.

Saw my Palliative Care Consultant week or so ago. I see my Respiratory Consultant end of this week.

Moved on to End-Of-Life-Care (status green)17/6

Began to stop taking statins for cholesterol to give my system a rest from as now, unnecessary treatment.

Mentally and emotionally I feel OK. It wasn’t unexpected. Physically, the deterioration feels like it’s noticeable on a weekly sometimes daily basis. Managing ambulatory oxygen is a challenge with deliveries now every three days.

The recent heat in the UK has knocked me about, thankfully we have a/c so that helps. I do have more energy and sleep better with continuous oxygen. There has been some vomiting and digestive problems. Heavy blood and mucus in my nose, I guess from the increase in oxygen.

I am waiting to hear from MacMillan team about care and treatment.

Hi community,

I'm sharing my dad's story here in hope someone can offer some hope, words of wisdom, or share their own experience.

Summary:

Diagnosis: Organising Pneumonia, ILD umbrella, with fibrosis.
Dad's age: 68
Before illness: Incredibly healthy, never smoked, no risk factors, previously would go on 10 mile hikes.
Time in hospital: 3 months
Time in ICU: 17 days
Weight lost in hospital: 16kg
Drug Treatment: The Drug Infusions: Cyclophosphamide. The Steroid Tablets: Prednisolone. The Lung Scarring Prevention: Pirfenidone.

Dad's Story

3 months ago my dad was sick with a cough, exhaustion and fever and was sent to the hospital by his local doctor as they believed he had a nasty bacterial pneumonia and his oxygen saturation was alarmingly low. They tried over 4 different antibiotics but his breathing was not improving and after 2 weeks in hospital he was admitted to intensive care and put on a ventilator for 7 days. When on the ventilator, the doctors pivoted strategy, diagnosing him with Organising Pneumonia and administering industrial level steroids via injection, alongside proning him on his front for 12 hours at a time. He responded to the steroids and proning, and was able to come off the ventilator and onto high flow, and was then given a tablet form steroid called Prednisolone.

10 days after he came off the ventilator, he got moved from icu to the respiratory ward for 3 weeks, before being discharged with oxygen. When he came home he was extremely immobile and weak, needing oxygen and a walking frame to move between rooms. Climbing up stairs could take 10 minutes as he had to have breaks to catch his breath.

After 10 days at home, an ambulance was called as his oxygen sats dropped really low. This was after the doctors told us to reduce the number of Prednisolone tablets he was taking from 6 to 5. I have read that it is common to see a relapse in OP recovery when tapering steroids too early.

He went back to hospital 7 weeks ago, and he is still in hospital today (29/06/26). The doctors believe he has an ILD, with fibrosis, as the CT scans are showing ongoing inflammation, and scarring, which he didn't have when he was discharged from hospital the first time. We are told this is a 10 in a million illness, and my dad has an incredibly rare form of it.

He is now being given two specialist drugs: Pirfenidone, which should halt and prevent further lung scarring, and an immunosuppressant called Cyclophosphamide.

Where we are today

Opinion varies greatly regarding dad's prognosis. The latest CT scan is showing no change since before he was given both specialist drugs, which is a positive sign as it shows the drugs have halted the progression of inflammation and scarring. One doctor actually believes there is a small improvement on the scan.

When he was first readmitted to hospital we were told there's a "50% chance" he has an aggressive pulmonary fibrosis, that he may deteriorate rapidly and the only option would be end of life care. That was 5 weeks ago, and since then, Dad's requirement for oxygen has reduced and he is being weaned off high flow onto a face mask. He is getting into a chair for a few hours every day too. We feel we have beaten the 50% chance odds of rapid deterioration, but what next?

Some doctors feel he may always need oxygen, some feel he will need oxygen but only in the medium term, some feel he could be housebound, some tell us that people with lung scarring can still play tennis, the spectrum of recovery feels incredibly wide. He will need months of rehabilitation to be able to build up his strength again and put the weight back on.

It is also hard to put his breathlessness down to his lung damage vs his extreme muscle wasting and deconditioning as a result of being bed bound for 3+ months.

Please share any words of wisdom
Has anyone experienced something similar, do you have insights to share?