Researchers Target a Cure for Cystic Fibrosis, IPF, and Other Chronic Lung Diseases

Researchers who study rare diseases often look at what’s going wrong in the body in order to find a treatment. But the team studying rare lung diseases at the Perelman School of Medicine at the University of Pennsylvania are shifting their focus to examine what goes right. The hope is that identifying the specific cells and pathways that promote repair and regeneration (rather than scar tissue formation) in the lung will lead them to develop more precise and effective therapies. Sounds like a plan!

For people with idiopathic pulmonary fibrosis (IPF), cystic fibrosis and the more common COPD, this approach could be a game changer. With all the advances in medicine present today, the lung presents unique challenges when it comes to diagnosis, let alone treatment, of chronic conditions.

“The complicated structure of lungs is why it is difficult to quickly diagnose the exact type of lung disease a person may have with any certainty,” said Edward E. Morrisey, PhD, a professor of Cell and Developmental Biology, and director of the Penn Center for Pulmonary Biology. “Also, as there is considerable reserve capacity in our lungs most people are not diagnosed with lung diseases such as IPF until the disease has progressed significantly.”

That’s a fancy way of saying that a person can lose half of their lung function before feeling ANY symptoms! In the US alone, approximately 100,000 people have IPF, 30,000 have CF, and 30 million suffer from COPD, and the damage from the scarring and depletion of lung function is currently irreversible. So having a way to target regenerative cells to help these people breath easier would be a major medical break through.

The Penn researchers have successfully identified types of cells in our lungs that promote self-renewal and regeneration of lung alveoli, the tiny air sacks in the lungs that allow the exchange of oxygen and carbon dioxide. These sacks are damaged by the scarring formed by IPF and CF. And the more damage is sustained the harder it is for cells to regenerate, leading to a vicious, potentially deadly, cycle.

Morrisey says the Penn team wants to target these regenerative cells while inhibiting lung cells called myofibroblasts that form scar tissue after injury, and likely contribute to diseases such as IPF. Knowing the detailed molecular differences between these two cell types should help in the next generation of targeted therapies such as nanomedicine.  Let’s hope their aim is true!

To learn more about CF check out our partners at CysticLife and Strawfie Challenge.

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