Understanding How Bacterial Enzymes Opens Door to Cystic Fibrosis Advancements
For those living with cystic fibrosis, every day is a battle to breathe and be well. The lungs are the most heavily impacted organ system affected by cystic fibrosis, in part due to the chronic accumulation of thick, sticky mucus that builds up outside the surface of the lungs. Over time, and given the repeated risks of infection and damage, the lungs suffer so much that most cases of cystic fibrosis end in respiratory failure.
Scientists have struggled to understand the disease in the past, although discoveries in the last twenty years have greatly improved treatment options and therapies for those born with the genetically inherited condition. Perhaps the most important discovery regarding the condition was the discovery of the gene responsible for cystic fibrosis, the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which must be inherited from both parents.
Since individuals diagnosed with cystic fibrosis are now living longer lives, more and more research is being performed in search of new, life-extending treatments that could offer these individuals even longer and higher-quality lives. Part of this continued research is in understanding the mechanics and inner-workings of the complicated disease.
Recently, scientists examined the role of bacterial enzymes in developing complications associated with the disease, and their discoveries may open the door to new and effective treatment options.
Examining the root of infection
Cystic fibrosis is characterized by the thick buildup of mucus in a patient’s lungs, but the universal threat to a person’s wellbeing is the ever-looming risk of lung infection. Once a person with cystic fibrosis develops a lung infection, recovery could be extremely difficult, and the damage in both the short and long term could be devastating. Those in advanced stages of the disease suffer a high rate of chronic lung infection, and the damage is usually extensive enough to culminate in respiratory failure.
Researchers have been working to better understand the relationship between the symptoms of cystic fibrosis and the universally high risk of lung infection in CF patients. They made a connection recently upon studying bacteria present in the body and lungs of these individuals. Certain bacteria were found to be routinely present in individuals with advanced stages of cystic fibrosis, and scientists studied these bacteria to see if they were a byproduct of complications, or the cause.
It was determined that the bacteria, Pseudomonas aeruginosa, is at least partially responsible for chronic inflammation in the cystic fibrosis lung. When too much of the bacteria is present, it secretes an enzyme called CFTR inhibitory factor (Cif), which blocks the body’s ability to naturally resolve inflammation. As a result, the lungs of individuals with cystic fibrosis are subjected to chronic inflammation, heightening the risk of infection and further complication.
In most cases of infection in late-stage cystic fibrosis, Pseudomonas aeruginosa is present in large amounts. Scientists found it remarkable that the bacteria continued to thrive in the hyper-inflammatory lungs of cystic fibrosis patients and were drawn to studying it in part for that reason. When inflammation in the lungs is not allowed to resolve over a long period of time, it can cause immense damage to the organs. After a period of prolonged stress, the result is typically respiratory failure.
Read on to learn more about this discovery and what it means for cystic fibrosis.