[Cystic fibrosis: facing patient transition from the pediatrician to the internist]
Luis E Vega-Briceño1, Ernesto Guiraldes C, Ignacio Sánchez D
1Secciones de Respiratorio Pediátrico, Respiratorio Pediátrico, Departamento de Pediatría, Pontificia Universidad Católica de Chile. levega@puc.cl
Insights
Increased survival in cystic fibrosis (CF) patients leads to new complications. Early diagnosis and management, including antimicrobials and physiotherapy, are key for better outcomes in CF patients.
Area of Science:
- Pulmonology
- Infectious Diseases
- Genetics
Context:
- Increased survival in cystic fibrosis (CF) patients presents new management challenges.
- CF patients exhibit early inflammatory pulmonary changes and are susceptible to bacterial infections (Staphylococcus aureus, Haemophilus influenzae, Pseudomonas aeruginosa).
- Disease presentation varies with age, with severe forms leading to early respiratory insufficiency.
Purpose:
- To highlight the evolving landscape of cystic fibrosis (CF) complications.
- To emphasize the importance of early diagnosis and comprehensive management strategies.
- To underscore the need for coordinated, multidisciplinary care transition from pediatric to adult services.
Summary:
- Advances in diagnostics and therapeutics improve CF patient survival and enable earlier detection.
- Effective CF management requires timely antimicrobial selection, pancreatic enzyme supplementation, and early respiratory physiotherapy.
- Recognizing CF progression as a continuous process necessitates flexible care transitions and integrated specialist communication.
Impact:
- Improved patient outcomes and quality of life for individuals with cystic fibrosis.
- Reduced burden of pulmonary and non-pulmonary complications in the growing CF population.
- Enhanced understanding of CF management, guiding clinical practice and future research.
Abstract:
The increased survival of patients with cystic fibrosis (CF) resulted in the appearance of new pulmonary and non-pulmonary complications. Even though subjects with CF present with inflammatory pulmonary changes at birth, several pathogens such as Staphyloccocus aureus, Haemophilus influenzae and Pseudomona aeruginosa contribute to the progression of pulmonary injury. Clinical presentations vary according to patient age; even though mild forms exist, patients with severe forms, develop respiratory insufficiency and end-stage disease at an early stage. Today, new diagnostic and therapeutic tools, increase the possibility of an early diagnosis and of greater survival. Successful management will depend on the timely selection of adequate antimicrobials, the use of pancreatic enzyme supplementation and early institution to respiratory physiotherapy. The transition from pediatric care to adult care must occur according to development of each patient. This timing must be flexible and there should be a constant communication and coordination within the different specialists in internal medicine. Thus, it is crucial to recognize the disease progression as a continuous process, giving the appropriate physiologic support and evaluating the needs of the patient and close relatives.
Related Concept Videos
Cystic Fibrosis: Management
Sinus disease and chronic sinusitis...
Cystic Fibrosis: Pathogenesis
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Pharmacokinetics in Pediatric Patients: Drug Excretion
Chronic Pancreatitis II: Collaborative Care
Assessment:
Chronic Obstructive Pulmonary Disease-V: Management
Smoking Cessation
Chronic Kidney Disease III: Interprofessional Care


