AI Article Synopsis

  • Spinal Muscular Atrophy (SMA) is a genetic disease linked to the SMN1 gene that causes muscle weakness, with a carrier frequency of about 1 in 50 people and a prevalence of 1-3 per 10,000 live births.
  • Turkey initiated the SMA Carrier Screening Program in 2021 to address increasing carrier rates, particularly due to consanguineous marriages, after noticing cases among children of healthcare workers who were unaware of screening options.
  • A survey of 1,322 healthcare professionals revealed that 5.8% were unfamiliar with SMA and 26% lacked knowledge of the screening program, with significant gaps noted in secondary and tertiary professionals and non-physicians, indicating a need

Article Abstract

Spinal Muscular Atrophy (SMA) is an autosomal recessive disease caused by variants in the SMN1 gene, leading to progressive muscle weakness. The carrier frequency of SMN1 gene variants, including variant and copy number variations, is estimated to be around 1 in 50 people, while the global prevalence of SMA is 1-3 per 10,000 live births. In response to the increasing carrier proportion, especially due to consanguineous marriages, Turkey launched the SMA Carrier Screening Program in 2021. Notably, recent SMA cases have been observed in the children of healthcare workers who did not undergo carrier screening, prompting us to evaluate their awareness of this program. After receiving ethics approval, 1,322 healthcare professionals completed a 15-item survey based on the SMA Carrier Screening Guidelines. Of these, 5.8% were unaware of SMA, and 26% lacked information about the national screening program. Awareness of the screening program was significantly lower among secondary and tertiary healthcare professionals compared to primary healthcare professionals (p < 0.0001) and among non-physician healthcare professionals compared to physicians (p < 0.0001). Additionally, a serious lack of knowledge was observed concerning the parts of the screening covering the pregnancy period. Although there is generally high awareness of the SMA Carrier Screening Program among healthcare professionals, significant knowledge gaps exist. These findings highlight the need for increased efforts to more effectively deliver screening programs and continue the education of healthcare professionals. Education and awareness campaigns can enhance program awareness and effectiveness, reach wider audiences, and contribute to preventive measures for the health of future generations.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11645347PMC
http://dx.doi.org/10.1007/s12687-024-00737-4DOI Listing

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