Abstract

Three new therapies for spinal muscular atrophy (SMA) have been approved by the United States Food and Drug Administration and the European Medicines Agency since 2016. Although these new therapies improve the quality of life of patients who are symptomatic at first treatment, administration before the onset of symptoms is significantly more effective. As a consequence, newborn screening programs have been initiated in several countries. In 2018, we launched a 3-year pilot program to screen newborns for SMA in the Belgian region of Liège. This program was rapidly expanding to all of Southern Belgium, a region of approximately 55,000 births annually. During the pilot program, 136,339 neonates were tested for deletion of exon 7 of SMN1, the most common cause of SMA. Nine SMA cases with homozygous deletion were identified through this screen. Another patient was identified after presenting with symptoms and was shown to be heterozygous for the SMN1 exon 7 deletion and a point mutation on the opposite allele. These ten patients were treated. The pilot program has now successfully transitioned into the official neonatal screening program in Southern Belgium. The lessons learned during implementation of this pilot program are reported.

Highlights

  • Food and Drug Administration and the European Medicines Agency since 2016

  • 95% of patients carry a homozygous deletion of exon 7 in the SMN1 gene, the remaining 5% of cases are due to the deletion of exon 7 on one allele and a deleterious variant on the opposite allele

  • The process that led to implementation of the newborn screening (NBS) program for spinal muscular atrophy (SMA) in Southern Belgium has been previously r­ eported[20]

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Summary

Introduction

Food and Drug Administration and the European Medicines Agency since 2016. these new therapies improve the quality of life of patients who are symptomatic at first treatment, administration before the onset of symptoms is significantly more effective. Nine SMA cases with homozygous deletion were identified through this screen Another patient was identified after presenting with symptoms and was shown to be heterozygous for the SMN1 exon 7 deletion and a point mutation on the opposite allele. Patients with two copies usually present with the most severe and frequent form of spinal muscular atrophy, SMA1. In these patients, symptom onset usually occurs before the age of 6 months, and this type of SMA is associated with high mortality and. SMA is classified into four types, SMA1, SMA2, SMA3, and SMA4, based on maximal motor ability achieved

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