Cystic Fibrosis

Overview

Cystic fibrosis (CF) is a life-threatening genetic disorder that results in the accumulation of thick, sticky mucus in the lungs, clogging airways and leading to infection and chronic inflammation. Moreover, because of the inability to clear the airways, bacteria colonize and form biofilms that are difficult for antibiotics to penetrate. The accumulation of mucus and biofilms are key drivers of pulmonary exacerbations and recurrent, intractable infections in cystic fibrosis patients that lead to a decline in pulmonary function and, ultimately, cause premature death.

Current treatment options for complications of pulmonary exacerbations are limited. Antibiotics used to treat exacerbations have side effects, reduced efficacy against biofilms, and can lead to antibiotic resistance with excessive use. Mucolytics are ineffective in clearing the mucus that builds up during exacerbations, and complicate effective treatment. Thus, there is increased urgency for new therapeutic approaches that address the biofilms and mucus accumulation during exacerbations, providing both clearance and potentiating antibiotic activity.

A new treatment to alter the progression of CF

Synspira is developing proprietary modified polysaccharide molecules that facilitate mucus expectoration and biofilm disruption, thereby rendering otherwise protected bacteria susceptible to antibiotics. Synspira’s lead molecule, SNSP113, seeks to treat pulmonary exacerbations in cystic fibrosis by controlling infection and inflammation. SNSP113 reduces mucus adhesion and viscosity, breaking up bacterial biofilms, and potentiating antibiotic activity, improving lung function.

How SNSP113 works at the lung surface

On the lung’s surface, mucus is moved up and out of the airways by structures known as cilia to keep inhaled bacteria and harmful substances from remaining in the respiratory system. In cystic fibrosis (CF), the mucus is thick and viscous, preventing free movement of the cilia and allowing bacterial colonies to form biofilms. Inhaled SNSP113 interacts with native glycoproteins in mucus and polysaccharides in bacterial biofilms to loosen the mucus and disrupt the biofilms. In doing so, SNSP113 allows normal clearance to occur and exposes bacteria previously protected by biofilms and mucus, making them more susceptible to antibiotics.

Patient Resources

CF Living

A place for community, support, and information for those living with cystic fibrosis (CF).

Cystic Fibrosis Community Support

An extensive list of support groups from Cystic Fibrosis Research Inc.

Cystic Fibrosis News Today

Recent news on new therapies, drugs, clinical trials and resources for cystic fibrosis.

Cystic Fibrosis Research Institute

As we work to find a cure for cystic fibrosis, CFRI seeks to inform, engage and empower the CF community to reach the highest possible quality of life.

The Boomer Esiason Foundation

A dynamic partnership of leaders in the medical and business communities joining with a committed core of volunteers to heighten awareness, education and quality of life for those affected by cystic fibrosis, while providing financial support to research aimed at finding a cure.

Cystic Fibrosis Foundation

A vast resource for researchers, doctors, caregivers, patients and supporters of patients with cystic fibrosis.

Roadmap to CF – The Bonnell Foundation

The Bonnell Foundation helps CF patients with availability and access to CF medications and assistance around insurance.

Cystic Fibrosis Services

Walgreens Pharmacy’s program to assist patients with CF to get the medications they need.