Here in the UK in the year I was told I have IPF (Idiopathic Pulmonary Fibrosis) 5,000 others received the same news. Somewhere between 3 and 4,000 have already died. They didn’t last long enough for a cure which will probably come from genomic developments – so said my consultant.
*If you know of anyone in a relevant field please let me know.*
This may or may not be genomic but it is the first ray of hope (I think) that I’ve seen for a long time;
An intriguing article that cuts through the fluff and talks about the realities of finding a cure for IPF. Both Esbriet and Nindetab are mentioned.
The IPF-associated protein that Elias’s team found—CHI3L1—“has been retained over species and time…We knew Mother Nature doesn’t do this unless it is very important.” When his team looked into it, they found CHI3L1 helps “stop cell death, while stimulating fibrosis and repair. It is a primordial, and fundamental, defense mechanism.”
Hunninghake wrote in the NEJM a revolution may be afoot. The new IPF drug trial results alone are “a major breakthrough….We may soon have choices in the medical management of pulmonary fibrosis.” It is even “reasonable to shift our understanding of the pathogenesis of this disease. It is now clear that (IPF) is a disease perpetuated by aberrant wound healing, rather than primarily by chronic inflammation.”
In part it says: “Via further experiments, Elias’ team found IPF is, in part, caused by fibrotic healing run amuck, trying to keep up with IPF’s ceaseless lung injury. Why is it ceaseless? “The injury process is still enigmatic.” But it is real. Doctors find, in autopsy, IPF patients’ lungs possess old scarring over old injuries—and fresh scarring over new ones.
Elias, and the team of Yale Translational Lung Research Director Erica Herzog, are further investigating IPF mechanisms. They are also screening for drugs. They have identified an antibody they will turn into a drug via the above (CADET) grant for lung drug design.”
“We are very excited about this whole area. We plan to stay in it for a while.”
Idiopathic Pulmonary Fibrosis: Hope, At Last
There have been stunning “firsts” in research on idiopathic pulmonary fibrosis (IPF), a mysterious disease that stiffens and stills the lungs, killing half its victims in three years. In May, results of Phase 3 clinical trials on the first two effective drugs for IPF were published in the New Englan…