People with cystic fibrosis are prone to infections because of the thick, sticky mucus that clogs their airways. The Cystic Fibrosis Foundation is funding research to help fight these infections. 

Why Are Infections a Problem in CF?

Infections are a problem for people with cystic fibrosis because they can cause fever, difficulty breathing, coughing, and excessive inflammation. A cycle of recurring infections and inflammation gradually destroys lung tissue.

People with CF are susceptible to infections from bacteria, viruses, and fungi because abnormally thick, sticky mucus traps these germs in the airways. They also are prone to infections because their mucus and airway liquid does not have the same infection-fighting properties as normal mucus. This abnormal mucus provides an ideal environment for bacteria to form protective layers -- known as biofilms -- that make them more difficult to kill.

Infections take a significant physical and mental toll on people with cystic fibrosis and remain a top concern. To make meaningful progress against this complex challenge, the Cystic Fibrosis Foundation has established the Infection Research Initiative to help improve the detection, diagnosis, prevention, and treatment of infections. 

What Basic Research Is Being Conducted?

Long-term lung infections are a serious, ongoing threat to the lives of people with CF; therefore, the Foundation funds more research programs focused on infections than any other CF complication.

Scientists are researching how germs interact with each other -- including how they compete, how they cooperate, and how they affect the strength or degree of damage that other microorganisms can cause to the lungs. Studying these interactions helps researchers develop better strategies to manage and treat infections.

Jennifer Bomberger, PhD, an associate professor of medicine at the University of Pittsburgh, answers questions from the CF community, such as whether a less serious bacteria can prevent the dominance of a more dangerous bacteria.

A new area of research will explore how microorganisms, such as bacteria, respond to the changing environment in the lungs once people start taking cystic fibrosis transmembrane conductance regulator (CFTR) modulators and eventually other drugs that target the underlying defect of CF.

Difficult-to-treat infections are another area of focus for researchers. Because more people with CF are getting nontuberculous mycobacteria (NTM) infections, the Foundation has funded ongoing research to learn more about them. In the video below, David Nichols, M.D., an associate professor of pediatric pulmonology and medical director of the Cystic Fibrosis Therapeutics Development Network Coordinating Center in Seattle, discusses what NTM is, why it is difficult to treat, and the range of research that is being done to address the problem.

Work done by the Foundation-funded Colorado CF Research Development Program helps with clinical care and lays the groundwork for developing new treatments for infections. Researchers at National Jewish Health in Denver analyze the DNA of NTM samples from patients to help clinicians make treatment decisions. The research program also sends NTM samples to research laboratories and pharmaceutical companies to test potential treatments in the lab.

Two new studies -- PREDICT and PATIENCE -- aim to create standard ways to diagnose and treat NTM. The participating study sites with expertise in NTM have formed the NTM Consortium, which will be able to accelerate future NTM clinical studies. 

The CF Foundation also has funded several programs to identify promising molecules that could be used to treat NTM by capitalizing on advances made by the Bill and Melinda Gates Foundation during its efforts to improve treatment for tuberculosis. (NTM is a bacterial cousin of the bacteria that causes tuberculosis.)

What Treatments Are Being Developed?

The Foundation is funding the development of a wide range of treatments to address infections. In addition, researchers are exploring when to treat, how long to treat, and what medications to use.

One study that will provide insight into the length of antibiotic treatment is the STOP 2 trial. To learn more about this study, watch the following video in which Jennifer L. Taylor-Cousar, M.D., an associate professor in the departments of Medicine and Pediatrics at National Jewish Health, explains why infections are a problem for people in CF, and what is being done to improve treatment.

To address drug-resistant bacteria, the Foundation is supporting clinical research into unique treatments, such as gallium and nitric oxide, that work differently than typical antibiotics.

To grow, bacteria rely on some processes that require iron. If gallium -- an atom nearly identical to iron -- is used instead, it can disrupt these processes. For example, gallium has been shown to kill antibiotic-resistant strains of Pseudomonas in laboratory research. Intravenous (IV) gallium is being tested in clinical trials in people with CF, and an inhaled version is in development.

Inhaled nitric oxide is being tested in clinical trials as a treatment for Pseudomonas and NTM. Nitric oxide is a molecule produced by our bodies that kills bacteria, breaks up biofilms, and improves the movement of cilia -- hair-like structures that help move mucus out of the lungs.

Other potential treatments that are being tested include:

  • New formulations and new uses of existing antibiotics
  • Drugs that would prevent hearing loss caused by aminoglycosides -- commonly used antibiotics such as tobramycin and amikacin

The Foundation is investigating the development of up to three potential therapies that could prevent or reverse hearing loss from aminoglycosides. Two drugs would protect the inner ear from damage. One drug would activate cells in the inner ear to create new hair cells that are responsible for hearing.

The Foundation also is supporting research into fungal infections, especially those caused by Aspergillus fumigatus. This type of fungus can cause complications when it becomes invasive or when it causes an allergic reaction called allergic bronchopulmonary aspergillosis (ABPA). For the past three years, the Foundation has been a major sponsor of Advances Against Aspergillosis, an international conference focusing on new discoveries about this fungus and improving its diagnosis and treatment.

Gina Hong, MD, MPH, assistant professor of medicine at the University of Pennsylvania Medical Center, answers questions from the CF community about research into anti-fungal treatments.

In addition, the Foundation has provided funding to develop an inhaled version of itraconazole to treat ABPA. An inhaled version could have fewer side effects and drug-drug interactions than the high doses needed for oral itraconazole.

To learn more about drugs such as IV gallium and other anti-infective research, watch the Facebook Live event, What's the Bug? New Treatments to Help Fight Infections. The video, filmed at the 2017 North American Cystic Fibrosis Conference, features Drs. Patrick Flume and Christopher Goss.

What Are the Current Treatments?

To learn more about current treatments for infections, visit Antibiotics.

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