Experience of carrier couples identified through a population-based carrier screening pilot program for four founder autosomal recessive diseases in Saguenay-Lac-Saint-Jean

Prenat Diagn. 2018 Jan;38(1):67-74. doi: 10.1002/pd.5055. Epub 2017 May 12.

Abstract

A pilot population-based carrier screening program started in 2010 in the Saguenay-Lac-Saint-Jean region of Quebec, Canada, for four recessive diseases with local founder effects (tyrosinemia type I, autosomal recessive spastic ataxia of Charlevoix-Saguenay, congenital lactic acidosis, and Andermann syndrome).

Objectives: The objective of this study was to describe the experience of carrier couples identified through this program.

Methods: Semi-structured interviews were performed with carrier couples. Thematic analysis of interview transcripts was performed to identify emerging themes.

Results: Interviews were performed with 15 carrier couples (56% response rate). Carrier couples had little knowledge about the target diseases before being identified as carriers, despite pre-test education sessions. The main motivation for screening was a recommendation by a peer who had been screened, even for those with a positive family history of one of the target conditions. Couples perceived themselves at low risk of being a carrier couple, whatever their family history. Being found to be a carrier couple was initially a shock, illustrating how ill prepared they were for such a result, but carrier couples appreciated knowing their status.

Conclusion: Our results emphasize the informational needs of couples to make informed decisions and the importance of post-test counseling for those with positive results. Our findings can inform counseling procedures in expanded carrier screening. © 2017 John Wiley & Sons, Ltd.

MeSH terms

  • Adult
  • Agenesis of Corpus Callosum / diagnosis*
  • Cytochrome-c Oxidase Deficiency / diagnosis*
  • Female
  • Founder Effect
  • Genetic Carrier Screening*
  • Heterozygote
  • Humans
  • Leigh Disease / diagnosis*
  • Male
  • Muscle Spasticity / diagnosis*
  • Peripheral Nervous System Diseases / diagnosis*
  • Pilot Projects
  • Spinocerebellar Ataxias / congenital*
  • Spinocerebellar Ataxias / diagnosis
  • Tyrosinemias / diagnosis*
  • Young Adult

Supplementary concepts

  • Corpus callosum agenesis neuronopathy
  • Leigh syndrome , French Canadian type
  • Spastic ataxia Charlevoix-Saguenay type