Related Experiment Video

Updated: Feb 13, 2026

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
05:56

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

Published on: August 29, 2025

589

Difficult conversations: Discussing prognosis with children with cystic fibrosis

Julia Gray Farber1, Mary G Prieur2, Christine Roach3

  • 1University of North Carolina School of Medicine, Chapel Hill, North Carolina.

Pediatric Pulmonology
|March 13, 2018
PubMed

Insights

Communicating cystic fibrosis (CF) prognosis to children is challenging. Patients often learn about their life expectancy and disease progression earlier than healthcare providers anticipate, highlighting the need for better communication strategies.

Area of Science:

  • Pediatric Pulmonology
  • Medical Communication
  • Patient-Centered Care

Background:

  • Cystic Fibrosis (CF) is a chronic, progressive, and life-threatening condition.
  • Current guidelines lack specific recommendations for discussing prognosis with pediatric CF patients.
  • Effective communication of prognosis is crucial for managing CF care.

Purpose of the Study:

  • To assess recall and current practices regarding prognosis communication in cystic fibrosis.
  • To identify patient, parent, and provider perspectives on discussing prognosis.
  • To solicit recommendations for improving prognosis communication in CF care.

Main Methods:

  • Conducted semi-structured interviews with young adults with CF, their parents, and CF healthcare providers.
  • Assessed participants' recall of learning about CF prognosis and current communication practices.
  • Gathered recommendations for enhancing prognosis communication.

Main Results:

  • Young adults with CF learned about limited life expectancy (ages 8-16) and disease progression (ages 7-19), often from physicians or online resources.
  • Patients and parents reported earlier awareness of prognosis than providers assumed.
  • While some experienced distress, most patients reported minimal impact on adherence; patients and parents desired physician involvement, but providers cited barriers like reluctance and time constraints.

Conclusions:

  • Prognosis communication in CF is vital yet challenging for all involved.
  • Developing tools and strategies for timely, accurate prognosis discussions is essential.
  • Improved communication can ensure patients receive necessary information effectively.

Keywords:
communicationcystic fibrosisprognosis

More Related Videos

Growing a Cystic Fibrosis-Relevant Polymicrobial Biofilm to Probe Community Phenotypes03:53

Growing a Cystic Fibrosis-Relevant Polymicrobial Biofilm to Probe Community Phenotypes

Published on: April 19, 2024

1.2K
Visualization of Pseudomonas aeruginosa within the Sputum of Cystic Fibrosis Patients07:25

Visualization of Pseudomonas aeruginosa within the Sputum of Cystic Fibrosis Patients

Published on: July 16, 2020

3.6K

Related Concept Videos

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
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,...
896
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
549
Gene Conversion02:08

Gene Conversion

Other than maintaining genome stability via DNA repair, homologous recombination plays an important role in diversifying the genome. In fact, the recombination of sequences forms the molecular basis of genomic evolution. Random and non-random permutations of genomic sequences create a library of new amalgamated sequences. These newly formed genomes can determine the fitness and survival of cells. In bacteria, homologous and non-homologous types of recombination lead to the evolution of new...
10.7K
Gene Conversion02:08

Gene Conversion

3.1K
Conversion of Units01:36

Conversion of Units

Sometimes, there is a need to convert from one unit to another one. For instance, reading a cookbook in which quantities are expressed in units of liters or ounces may require conversion of quantities to cups. Or, when looking up directions on how to get to a location, we may be interested to know how many miles we are going to walk. In this case, we would have to convert units of feet or meters to miles.
The first step in the unit conversion is to list the given units and the units required...
37.1K
Conversion of Alcohols to Alkyl Halides02:48

Conversion of Alcohols to Alkyl Halides

This lesson delves into the conversion of alcohols to corresponding alkyl halides and the mechanism of action for different reagents. Typically, the hydroxyl group is first protonated to convert it to a stable leaving group. Consequently, based on the starting alcohol, the mechanism undergoes either of the nucleophilic substitution routes, SN1 or SN2. Tertiary alkyl halides are made using the two-step SN1 mechanism that occurs via a carbocation intermediate, which is stabilized by...
8.5K