Most people take breathing for granted — until the ability to breathe is impaired. People with idiopathic pulmonary fibrosis (IPF) know this all too well.
Without the ability to readily take in oxygen, the simplest tasks can become a challenge and the entire body suffers. This chronic, progressive and debilitating disease has a number of medical treatments, but no cure. It’s rare — IPF affects 19.8 per 100,000 persons in the U.S. — and the effects and complications are devastating.
Fortunately, scientists are hard at work studying IPF in hopes of better understanding this disease and developing new treatments and — ultimately — a cure. And some of the hardest-working researchers can be found at the Centre for Inflammation and Tissue Repair (CITR) at the University College of London (UCL) Respiratory. Here’s a quick run-down of their project list, translated into layman’s terms.
- Matrix Regulation in Chronic Lung Disease: No, it’s not the next blockbuster starring Keanu Reeves. Matrix is a fancy way of describing cellular structure and organization. So, this study involves some super smart geeky scientists studying how scar tissue forms at a cellular level so it can be addressed by treatments that target those cells.
- Auto-antibodies and pulmonary fibrosis: OK, that’s a mouthful. Try saying that five times, fast. Our bodies create antibodies to fight infection, but sometimes something goes wrong and those protective agents become the enemy, attacking healthy cells as though they were viruses. (It sounds like science fiction, but it’s science fact.) This study involves examining how these evil auto-antibodies attack the blood vessels in people with interstitial lung diseases, such as IPF.
- Molecular Imaging Studies: Positive Emission Tomography (PET) scans use a special dye with radioactive tracers. The dye is injected into the body via a vein and absorbed by the organs. The scanner then reveals the dye to show blood flow, oxygen absorption, and more. In this study, investigators are using PET scans to see how lung fibrosis has advanced so they can determine how to treat the IPF.
- Leukocyte Activation. Again, very geeky stuff. Leukocytes are white blood cells. (Why not just say that to begin with, right?) White blood cells are produced in the bone marrow and they protect the body against infection. (In other words, they are the good guys.) For some reason — perhaps because of limited oxygen intake — white blood cells are often activated in people with IPF, causing the “good guys” to do damage to the lungs. The presence of certain types of white blood cells have been found to be indicators of IPF progression.
- Airway Epihelial Project. Remember the schoolyard joke, “Hey, your epidermis is showing!”? Epidermis is skin, of course. And epihelial is a type of cell that composes skin and line the inside of your throat, lungs, and other organs. Ephihelial protect your body. This study explores how those cells along the throat (that transfers air to and from the lungs) are affected by white blood cells in people with IPF.
- Platelets and Pulmonary Fibrosis. Blood carries oxygen through the body. And platelets are the cells in blood that are cause clotting. How are they affected by IPF? That’s what these researchers are trying to find out!
- The Lung-COOL Trial: These scientists are developing a new diagnostic tool that can extract cells taken during lung biopsy in a minimally invasive procedure. This means fewer patients would have to undergo surgical lung biopsy, which has greater risk and cost. Very cool.
- The INHALE Study: Can medications for IPF be delivered directly to the lungs through inhalation? This study aims to discover if it’s possible.
And that’s just a sampling of what this busy group of scientists is up to! You can find out more about these and other IPF studies and stay up to date on their progress by visiting the UCL Respiratory Center for Research at Breathing Matters.