Imagine a world where breathing is a luxury and a cough marks the sound of an inner battle. Interstitial Lung Disease (ILD), a condition characterised by inflammation and fibrosis, is a major cause of severe respiratory illness and can occur in children. While researchers have made progress in slowing the disease in adults, the situation for children remains grim: there are currently no effective treatments, and lung transplantation is their only hope for survival.
To enter the world of ILD is to enter a dark maze. Scientists are struggling to understand the natural history of the disease and its many forms, and the effectiveness of the treatments that have been discovered leaves much to be desired. ILD varies with age.
Affected children often suffer from birth and can die within months if they do not receive a lung transplant. In adults, the progression of the disease is different, with symptoms starting subtly but worsening rapidly, typically resulting in death after just three to five years. Not only when the disease strikes, but who is at risk of developing it varies widely, even within families, adding another twist to the search for a solution.
Researchers know that genetic factors and mutations play an important role, but the rarity of these mutations makes it difficult to accumulate the kind of knowledge that could point the way to more effective strategies. With so many layers of complexity to consider, scientists are forced to look for common factors that promise br.
