Fibrosis File


Robert Craig “Evel” Knievel

Pulmonary Fibrosis has been in the news recently. Ironically, we just lost death-defying Evel Knieval and also singer Robert Goulet to the disease.

Dr. Steven Idell of Tyler, TX,

has discovered an enzyme that carefully eliminates scar tissue and blood clots that inhibit lung function. It’s called single-chain urokinase plasmogen activator, or scuPA. Idell said scuPA doesn’t act like other agents. It doesn’t eat everything away, but instead is slow-acting and only reacts with tissue it is meant to destroy. Inhaling scuPA could be a potential treatment for pulmonary fibrosis. Dr. Idell is applying for clinical trials to be conducted by the National Institute of Health.

The Journal of Clinical Investigation reports “Telomerase, a protein most notable for its connections to aging and cancer, has been shown to have increased activity in mice with lung fibrosis. University of Michi- gan researchers have identified telomerase in the progression of pulmonary fibrosis. Telomerase activity is dependent on the presence of a related protein named TERT. The study found that mice with reduced TERT

levels also had reduced telomerase activity in the lungs, and less severe lung fibrosis. This effect was reversed if the TERT-deficient mice were transplanted with TERT- sufficient bone marrow cells prior to lung injury.”

Coprexa™ is an oral, anti-copper agent that is high- ly specific for the reduction of free copper in blood serum. Pipex Pharmaceuticals is developing Coprexa™ for fibrotic disorders based upon the rationale that the fibrotic disease process is dependent upon the availability of free copper in the body. Coprexa™ has demonstrated the ability to inhibit fibrosis in a number of animal trials. The company is planning to initiate a phase III clinical trials.

Better Breather Speaker Topics: Keep It Lively!

Are you starting a new group or scheduling speakers for your Better Breather Club meetings for 2008? Janie Severns of Coarsegold, CA, has suggestions of people to keep your sessions interesting and lively!

Ask a Pulmonologist or Respiratory Therapist to speak on How the Heart and Lungs Work Together or Asth- ma and COPD Medicine and Management Programs. A Physical Therapist will teach you Better Breathing through Exercise, Chair Exercises for COPD or ask a Yoga Instructor to visit. A Home Health Nurse, Registered Di- etician, Oxygen Supplier, Psychologist, Occupational Ther- apist and Emergency Medical Personnel are all full of valu- able information. Your own members may share travel experiences and other tips. With just a little planning, you will be on top of your COPD!