Cystic fibrosis is an inherited, fatal disorder that affects the lungs and pancreas. Some 70,000 people worldwide, including nearly 30,000 in the United States, suffer from the disease, which is marked by the production of abnormally viscous mucus. In the lungs, this mucus blocks the airways, causing lung damage andmakes it hard to breathe resulting in chronic respiratory infection. CF is a life-threatening condition ultimately resulting in premature death from respiratory failure. Bacterial and fungal airway chronic lung infections have been related to greater morbidity with a more rapid deterioration in lung function. Median predicted survival estimates are fewer than 40 years in the US and EU.
For many years, both researchers and clinicians have focused on the problems in CF caused by chronic bacterial airway infection. Understanding the microbial flora of cystic fibrosis patients’ respiratory tracts is of considerable importance, as patient morbidity and death are primarily caused by chronic drug-resistant respiratory infections whose diverse ecosystems vary greatly in different age groups. It is known that cystic fibrosis patients with chronic Pseudomonas aeruginosa, resistant Staphylococcus aureus (MRSA) or mixed bacterial-fungal infection have a higher death rate and higher rate of respiratory decline.
Pseudomonas aeruginosa is the most prevalent organism in the airway colonization of CF patients.
S.aureus is the second most common pathogen in CF and is known to spread easily from one person to another. Among the bacteria that cause lung infection in patients with CF, MRSA is becoming more common and is now found among 25 percent of this particular population. MRSA is resistant to multiple antibiotics, and lung infections caused by this microorganism significantly reduce life expectancy.
The consequences of infection by individual bacteria have been well studied andrevealed just how important of a role that pathogens play in CF. Both fungi and bacteria co-exist in CF lungs and the interactions among them influence their pathological potential, worsening the pulmonary function, and leading to lung function decline.
The biggest challenge for today’s medicine is the rise of multiresistant microorganisms. Numerous researchers are warning of an imminent post-antibiotic era or ‘antibiotic apocalypse’ and CF is not an exception. Physicians treating patients with CF are increasingly faced with infections caused by multidrug resistant microorganisms. Antibiotic resistance in CF respiratory infections results from bacteria changing and rendering antibacterial drugs no longer effective. The challenge of treating these increasingly antibiotic-resistant infections with a limited spectrum of options has spurred interest in novel antimicrobial drug development. There are only a few drugs available to treat lung infection is CF patients and the antibiotic resistance is on the rise.
TGV-inhalonix was recently granted Ophan Drug Designation from the U.S. Food and Drug Administration to aid in its development of an inhaled antimicrobial, Mul-1867, that in early tests has been shown to be safe and effective against antibiotic-resistant microorganisms and bacterium within biofilms.