Coalition For Pulmonary Fibrosis

Leading the Fight Against PF - 10 Years of Progress

2011 marks the 10th Anniversary of the Coalition for Pulmonary Fibrosis (CPF) - founded in 2001 to provide information, support, advocacy and hope to the tens of thousands of patients with PF. Until the CPF was established, there were no resources for patients or families facing PF, and little to no government or public interest in or awareness of the seriousness of a disease taking as many lives as breast cancer. The CPF immediately set out to fill that gap in services and shortly after its founding also began directly supporting research into the disease. An impressive series of partnerships around the country, from powerful institutions such as the American Thoracic Society to all major Pulmonary Fibrosis (PF) research and treatment centers, has dramatically increased the CPF's reach and impact.

With the help of the thousands of individuals who have become members of the organization, the CPF's national effort has given voice to the tens of thousands of patients who have been lost to PF, and those who cared for them. The CPF has been there to guide the patients and families trying to navigate the very difficult challenges PF, supporting those in need every step of the way. The organization has worked to raise restricted funds to directly support research that is seen as cutting edge and innovative in the field.

In recent years, the CPF has brought national exposure to the disease via press coverage, has helped put in place over 50 support groups across the country, and has partnered with Congress to generate the first-ever funding allocation request for PF. The national press coverage recently generated is helping to break the secrecy around PF. Understanding that awareness breeds attention and support, the CPF anticipates that increased emphasis on making PF a widely known disease will lead to increased attention and funding for the search for treatments and a cure.

The CPF's objective is to provide services to the PF community as long as they are needed. This anniversary year will be marked by expanded awareness efforts and the anticipated reintroduction of a research allocation bill in Congress. The work to accrue new funds to support research is always a primary goal.*

With over $2 million in research grants funded, the organization's grants have been encouraging new scientists to look at the pathways that may lead to treatments. From a small number of research projects focused on PF in 2001, the increase has been dramatic and now under investigation are many new potential therapeutic approaches.

• More than $2million awarded in grant funding for PF research
   
• More than 50 support groups nationwide
   
• Comprehensive Newsletter and Website to provide information on drug trials, events, research news, advocacy issues, support resources and fundamental questions about PF.
   
• Free support, information and referral service free of charge to any and all those seeking help
   
• Partnership with Congress to introduce the first legislation directly impacting PF
   
• Action to protect PF patients' rights has helped win important battles such as access to Medicare Disability regardless of age
   
• Active participation with the American Thoracic Society, the nation's premier pulmonary organization
   
• Awareness efforts that have resulted in national television and radio coverage
   
• Maintaining the national scope and impact of our work while remaining personal in service to every patient or caregiver in need
   
• Assisting researchers in building cohorts of patients to undergo drug trials in order to help save lives in the future
   
• Actively partnering with many national groups working to increase attention to rare disease research
   
• Supporting and managing multiple annual events nationwide to raise funds and awareness for the cause

* Some Key Research Pathways for Pulmonary Fibrosis as of March 2011

Growth Factors:

• Monoclonal antibodies to inhibit "bad" cytokines (protein growth factors, such as TGF-beta, TNF-Alpha, or CTGF, that activate inflammation)
   
• Regulation of epithelium growth factors such as Keratinocyte growth Factor (KGF)
   
• Protein kinase inhibitors
   
• Suppression of immune responses to Collagen V (a fibrillar collagen)
   
• Blocking the levels of Lysophosphatidic acid and encouraging the action of sphingosine 1-phosphate (S1P) , which seems to prevent inflammatory cells from entering the lung
   
• Blocking Interleukin 13, a possible cause of scarring
   
• Antifibrotic or antifibrogenic agents (such as Pirfenidone, PRM-151, interferon and certain blood-pressure-lowering medications) to suppress the scarring process

Drug Combinations:

• Combined approaches such as NAC, Prednisone & Azathioprine vs. NAC alone. NAC is an antioxidant, Azathioprine a cytotoxic agent and Prednisone is ant-inflammatory.
   
• Use of Prostaglandin E2 and DNA methylation inhibitors to stop dysregulated gene expression

Genetics:

• Ongoing study of blood and genetic biomarkers
   
• Use of Prostaglandin E2 and DNA methylation inhibitors to stop dysregulated gene expression
   
• Targeting regulatory molecules absent in PF that can prevent expression of PF associated genes (Piper)
   
• Targeting the process of epithelial cells changing into myofibroblasts, possibly a mechanism that contributes to abnormal accumulation of scar tissue in the lung.

Others:

• Anti-pulmonary hypertension medication
   
• Use of thalidomide for cough
   
• Antioxidants (such as N-acetylcysteine) to prevent damage to lung tissue
   
• Endothelin receptor antagonists (such as Bosentan or Letairis) - terminated study