Literature DB >> 27277131

Attitudes Toward Discussing Approved and Investigational Treatments for Cystic Fibrosis in Prenatal Genetic Counseling Practice.

Caroline Rung Elsas1, Elinor Langfelder Schwind2, Laura Hercher3, Michael J Smith3, Kara Gardner Young2.   

Abstract

This project aimed to explore the attitudes of prenatal genetic counselors toward discussion of novel approved and experimental CF treatments in the prenatal setting, and to assess how knowledge of genotype-specific, targeted treatments may influence their current practices. Targeted treatments have the potential to impact the health-related quality of life of individuals affected with CF and therefore, knowledge of the availability of such treatments may influence the decision-making process of parents who receive a fetal diagnosis of CF. Using the 2012 FDA approval and introduction of ivacaftor into CF clinical practice as a case study, a survey was designed to explore the opinions and practices of prenatal genetic counselors with regard to counseling for a prenatal diagnosis of CF, and how those practices might be impacted by the availability of a new genotype-specific treatment. Approximately 800 genetic counselors were sent questionnaires in January of 2013. Respondents were provided information about this treatment and were asked to rate its perceived benefits, along with the likelihood that they would discuss potential benefits and limitations with parents receiving a prenatal diagnosis of CF. One-hundred sixty-nine prenatal genetic counselors (21.1 %) responded to the survey. Results indicated that 80 % of respondents 'never heard of the drug', or they were 'not exactly sure' what it was. After reading the materials provided, counselors felt the new treatment would have 'some' or a 'significant' impact on an affected individual's life. Their opinions varied on what information about this treatment they would choose to discuss with their patients; even if the treatment is currently FDA approved and clinically available for affected individuals with the genotype of the fetus. However, they would 'definitely' refer these patients to a specialist to discuss targeted treatments further. Most prenatal genetic counselors indicated there are certain scenarios in the prenatal setting which warrant a discussion of targeted treatments for CF, at least on some level. Counselor's views on sharing information about new treatment options are shaped by their familiarity with the treatment and their perception of its benefits and limitations, their comfort discussing these subjects, and their interpretation of the genetic counselor's role. Most genetic counselors had never heard of ivacaftor or Kalydeco™ prior to taking the survey. Therefore, counselors need to be better educated about the availability of CFTR mutation-based treatments before they will be able to incorporate discussion of new treatment options into their counseling.

Entities:  

Keywords:  Cystic fibrosis; Ivacaftor; Personalized medicine; Prenatal genetic counseling; Targeted treatments

Mesh:

Substances:

Year:  2016        PMID: 27277131     DOI: 10.1007/s10897-016-9978-1

Source DB:  PubMed          Journal:  J Genet Couns        ISSN: 1059-7700            Impact factor:   2.537


  23 in total

1.  A new definition of Genetic Counseling: National Society of Genetic Counselors' Task Force report.

Authors:  Robert Resta; Barbara Bowles Biesecker; Robin L Bennett; Sandra Blum; Susan Estabrooks Hahn; Michelle N Strecker; Janet L Williams
Journal:  J Genet Couns       Date:  2006-04       Impact factor: 2.537

2.  Update in cystic fibrosis 2008.

Authors:  Felix Ratjen
Journal:  Am J Respir Crit Care Med       Date:  2009-03-15       Impact factor: 21.405

Review 3.  ACOG Committee Opinion No. 486: Update on carrier screening for cystic fibrosis.

Authors: 
Journal:  Obstet Gynecol       Date:  2011-04       Impact factor: 7.661

4.  Cystic fibrosis mutations for p.F508del compound heterozygotes predict sweat chloride levels and pancreatic sufficiency.

Authors:  R Sebro; H Levy; K Schneck; D Dimmock; B A Raby; C L Cannon; U Broeckel; N J Risch
Journal:  Clin Genet       Date:  2011-11-29       Impact factor: 4.438

5.  Impact of recent pulmonary exacerbations on quality of life in patients with cystic fibrosis.

Authors:  Maria T Britto; Uma R Kotagal; Richard W Hornung; Harry D Atherton; Joel Tsevat; Robert W Wilmott
Journal:  Chest       Date:  2002-01       Impact factor: 9.410

6.  Quality of life assessment in patients with cystic fibrosis by means of the Cystic Fibrosis Questionnaire.

Authors:  Milena Antonelli Cohen; Maria Ângela Gonçalves de Oliveira Ribeiro; Antonio Fernando Ribeiro; José Dirceu Ribeiro; André Moreno Morcillo
Journal:  J Bras Pneumol       Date:  2011 Mar-Apr       Impact factor: 2.624

7.  Cystic Fibrosis Foundation and European Cystic Fibrosis Society Survey of cystic fibrosis mental health care delivery.

Authors:  J Abbott; J S Elborn; A M Georgiopoulos; L Goldbeck; B C Marshall; K A Sabadosa; B A Smith; A L Quittner
Journal:  J Cyst Fibros       Date:  2015-01-12       Impact factor: 5.482

8.  Quality of life and healthcare utilisation in cystic fibrosis: a multicentre study.

Authors:  Judy M Bradley; Steven W Blume; Maria-Magdalena Balp; David Honeybourne; J Stuart Elborn
Journal:  Eur Respir J       Date:  2012-07-26       Impact factor: 16.671

9.  A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial.

Authors:  Michael P Boyle; Scott C Bell; Michael W Konstan; Susanna A McColley; Steven M Rowe; Ernst Rietschel; Xiaohong Huang; David Waltz; Naimish R Patel; David Rodman
Journal:  Lancet Respir Med       Date:  2014-06-24       Impact factor: 30.700

10.  Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial.

Authors:  Eitan Kerem; Michael W Konstan; Kris De Boeck; Frank J Accurso; Isabelle Sermet-Gaudelus; Michael Wilschanski; J Stuart Elborn; Paola Melotti; Inez Bronsveld; Isabelle Fajac; Anne Malfroot; Daniel B Rosenbluth; Patricia A Walker; Susanna A McColley; Christiane Knoop; Serena Quattrucci; Ernst Rietschel; Pamela L Zeitlin; Jay Barth; Gary L Elfring; Ellen M Welch; Arthur Branstrom; Robert J Spiegel; Stuart W Peltz; Temitayo Ajayi; Steven M Rowe
Journal:  Lancet Respir Med       Date:  2014-05-15       Impact factor: 30.700

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  3 in total

1.  Prenatal genetic testing for cystic fibrosis: a systematic review of clinical effectiveness and an ethics review.

Authors:  Sharon J M Kessels; Drew Carter; Benjamin Ellery; Skye Newton; Tracy L Merlin
Journal:  Genet Med       Date:  2019-08-30       Impact factor: 8.822

Review 2.  Bridging the Gap between Scientific Advancement and Real-World Application: Pediatric Genetic Counseling for Common Syndromes and Single-Gene Disorders.

Authors:  Julie A McGlynn; Elinor Langfelder-Schwind
Journal:  Cold Spring Harb Perspect Med       Date:  2020-10-01       Impact factor: 5.159

3.  Lived experiences of individuals with cystic fibrosis on CFTR-modulators.

Authors:  Annelise Page; Aaron Goldenberg; Anne L Matthews
Journal:  BMC Pulm Med       Date:  2022-01-21       Impact factor: 3.317

  3 in total

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