Pseudomonas aeruginosa and Staphylococcus aureus are bacterial pathogens frequently associated with pulmonary complications and disease progression in cystic fibrosis (CF). However, these bacteria increasingly show resistance to antibiotics, necessitating novel management strategies. One possibility is bacteriophage (phages; bacteria‐specific viruses) therapy, where…
February 2021
Remaining Barriers to Normalcy in Cystic Fibrosis VI
Drug development for cystic fibrosis
The first regulatory approval for a drug developed specifically for cystic fibrosis (CF) occurred in 1993, and since then, several other drugs have been approved. Median predicted survival in people with CF in the United States has increased…
Sleep‐disordered breathing in cystic fibrosis
Sleep‐disordered breathing (SBD) is an under recognized comorbidity in the cystic fibrosis (CF) population across the lifespan. Nocturnal hypoxemia, obstructive sleep apnea, and nocturnal hypoventilation are respiratory abnormalities that occur commonly during sleep in patients with lung disease,…
Emerging technologies for cystic fibrosis transmembrane conductance regulator restoration in all people with CF
Although effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy has the potential to change the lives of many patients with cystic fibrosis (CF), it is unlikely that these drugs will be a game changing therapy for all.…
Novel imaging techniques for cystic fibrosis lung disease
With an increasing number of patients with cystic fibrosis (CF) receiving highly effective CFTR (cystic fibrosis transmembrane regulator protein) modulator therapy, particularly at a young age, there is an increasing need to identify imaging tools that can detect…
Novel therapies for treatment of resistant and refractory nontuberculous mycobacterial infections in patients with cystic fibrosis
Respiratory infections caused by non‐tuberculous mycobacteria (NTM) are a major cause of morbidity for patients living with cystic fibrosis (CF), as NTM pulmonary disease (NTM‐PD) is challenging to both diagnose and eradicate. Despite the lengthy courses of the…
Enhancing care for individuals with advanced cystic fibrosis lung disease
While remarkable advances in cystic fibrosis (CF) care have led to improvements in survival and quality of life, many individuals with CF are living with advanced cystic fibrosis lung disease (ACFLD) and others will face continued disease progression…
Entering the era of highly effective modulator therapies
Since the discovery of the gene responsible for cystic fibrosis (CF) in 1989, hopes have been pinned on a future with novel therapies tackling the basis of the disease rather than its symptoms. These have become a reality…
The remaining barriers to normalcy in CF: Advances in assessment of CF lung disease
Despite early diagnosis of cystic fibrosis (CF) through newborn screening, a substantial proportion of infants and young children with CF still demonstrate physiologic and structural evidence of lung disease progression, such as obstructive airway disease and bronchiectasis. The…
Promoting emotional wellness in children with cystic fibrosis, Part I: Child and family resilience
Attention should be given to individual and family well‐being from a child’s first interaction with the medical team and continuing throughout development, especially for families who experience chronic illnesses, such as cystic fibrosis (CF). While much attention has…
Promoting emotional wellness in children with CF, part II: Mental health assessment and intervention
This is the second of two companion papers that examine the emotional wellness of children with cystic fibrosis (CF) during the early years of life, defined here as the period between birth and age 12. Both papers promote…