Mum diagnosed with IPF

Is it too late to get Esbriet (Perfenidone)?  It may not make things better, but it will delay the condition getting worse,

i have been been using it girl 2 1/2 years.

I cannot understand why your mum has not been referred to a specialist consultant in Thoracic Chest Medicine as they may start her on Pirfenidone capsules. This medicine delays progression of the disease and might just might give your mum a better quality of life.

Kind regards

Jazztrain

Hi - 

 

     I have a family member I've been caring for with IPF for almost a year - we live in the US and see a specialist at John's Hopkins. We were told that if sO2 drops below 88 we need to turn up the concentrator. Our dad is now on 10 liters per minute. 79% and below is where we were told brain damage can begin to happen. I would turn up the oxygen and inquire about Esbriet as others have said. 

Recomend you read my latest blogs on this page. I believed, and for me anyway it was true, that oxygen over a period of time made my condition worse. So I bought a finger oxygen/pulse meter from Walmart for $50 and started monitoring my oxygen levels....I am doing so very much better now. I also, on the advice of a naturopath cut out eating refined sugar in any form....for me a very difficult thing as I LOVE choclate and sugar in my tea and coffee....It has been very hard but I am feeling so much better and feel I am going to live for a few years now...an incredible transition from what I was feeling 6 months ago....Hope this all helps. 

My mom had firbrosis issue 15 yr ago.  She first presented with difficulties in breathing.. and unable to sleep [due to oxygen insufficiency].. and when we brought her to the ER, she was put on supp.oxygen, then only she was able to sleep...her symptoms gradually worsened and about a year after that, diagnosed with pulmonary fibrosis with scleroderma.  Her fibrosis was not limited to the lung. She passed away at the end of 2007.  She was on steroid drug prednisolone and another drug which I cant recall.

Prior to the first crisis, she had backpain [upper left quadrant] which she complained about. She was also sometimes seen grasping for breath.  IPF is a mystery, but it is known only as a disease with abnormal tissue scarring ie lung.  While scleroderma is said to be an autoimmune disease.  It is said that some cases of scleroderma are familial ie there is genetic predisposition.  I also had upper back pain which can go up or down in intensity; i didnt make any correlation prior to this, but now I think I should.  I went to see the speacialist ie ortho and they just dont know whats wrong with my back.  I couldnt make the link, but now I think there could be some things that I should monitor.  

About 15 yr ago I had major issue with breathing ie I easily get tired and was wheezing for air at times.  I think maybe the disease at the early stage, is a rather silent creeping thing ....for most ppl .....as the disease steals away your stamina bit by bit, so that in due time, you eventually get used to the heavy feeling, and did not think there was any major issue with your  body.  However, a crisis can cause a drastic precipitation [like my mom] and thats when things markedly become worse

When I had the heavy lung issue, I tried  various supplements.  There was once when I took RIBOSE sugar, it was like a steroid shot.  I felt completely light and energetic [this is where I think ppl do not realised, that they are actually having a major issue with their own body].  RIBOSE is  a sugar that helps build ATP/energy.  However, the effect dissipated after that and the back upper pain returned [although it seems with lesser intensity]

The second "eye opening" thing / supplement that made me feel so energetic was Ubiquinol.  The first intake was another eye opener.  IMO, ppl with this abnormal sclerosis disease have an underlying oxidative stress ie not enough oxygen is supplied to the cells.  This causes stress in the cells, eventually, that causes tissue injury and abnormal tissue repair, followed by fibrosis. What causes the stress is still a mystery

After I took Ubiquinol, my breathlessness disappeared.  But my back upper quadrant pain was never resolved ..it come and go and I am only due to meet a rheumatologist in August.  

So, my opinion is that, if Ribose and Ubiquinol [first use] gave a great big uplifting of energy, there must be sth that is not balanced in the body and requires monitoring.  

I have dig quite a number of websites/books and discovered that TLR4 maybe involved in inflammation and fibrosis.  This issue is still being debated as the observations are not concordence [animal models] in the reports.  However, in fibrosis, it is observed that this receptor is abnormally upregulated eg liver fibrosis.  Searching PubMed for "tlr4 and er stress", I noted that activation of TLR4 can cause ER stress (ER is a labyrinth of compartments in the cell, that processes many cellular proteins for proper folding, synthesis and maturation].  When ER stress occurred, it can lead to fibrosis [google ER stress fibrosis].  While I am no expert in IPF / fibrosis disease, the facts are there.  

What actually causes TLR4 upregulation or stimulation, is still a mystery, it could be viral bug like EBV or CMV or bacterial, or environmental contaminant.  It could be a combination of these agents.  

Coincidentally, I noted that one herbal tea that I took recently, which contained Clinacanthus nutans herb, made the pain disappear and my body felt like a heap of burden being lifted off.  Initially I thought the herb might have somekind of painkiller active components.  However, searching PubMed, I noted a very recent publication showed that its anti inflammatory activity is achieved by TLR4 inhibition [google pubmed 26869924].  This is definitely different than typical pain killer like aspirin or celebrex [which both didnt help me]. I dont know if this info is significant, but it seems the herb might stop the excessive TLR4 signalling and may possibly prevent the fibrosis or progression?

 

Hello All,

Thanks, for the support and advise.

It is with the heaviest heart that I send you this message regarding my mother who passed away last Friday with advanced fibrosis. May god rest her soul.

I pray and hope that one day there will be a cure for PF. So no one will have to bear this pain of losing a loved one to PF.

Once again thanks!

Regards,

Osman