A Systematic Literature Review of the Natural History of Respiratory, Swallowing, Feeding, and Speech Functions in Spinal Muscular Atrophy (SMA).

Background: Respiratory and bulbar dysfunctions (including swallowing, feeding, and speech functions) are key symptoms of spinal muscular atrophy (SMA), especially in its most severe forms. Demonstrating the long-term efficacy of disease-modifying therapies (DMTs) necessitates an understanding of SMA natural history.

Objective: This study summarizes published natural history data on respiratory, swallowing, feeding, and speech functions in patients with SMA not receiving DMTs.

Feeding characteristics of healthy infants without reported feeding impairments throughout the first month of life.

Objective: Elucidate characteristics of feeding performance in healthy infants without reported feeding problems throughout the first month of life.

Study Design: Feeding was monitored in 61 healthy infants by caregiver report for 48 h a week from birth to 4 weeks old. Outcomes included feeding modality, how much they consumed, how long the feed lasted, and how many coughing episodes the infant exhibited.

Establishing Normative Values for Healthy Term Infant Feeding Performance: Neonatal Eating Assessment Tool-Mixed, Oral Feeding Scale, and Early Feeding Skills Assessment.

Purpose: Infants with perceived feeding problems are frequently referred for assessment of their feeding abilities. However, little is known regarding how healthy nondysphagic infants perform on commonly used assessments, making determination of impairment difficult. The aim of this investigation was to elucidate the characteristics of healthy term infant feeding performance using three commonly employed clinical assessments: Neonatal Eating Assessment Tool-Mixed (NeoEat-Mixed), Oral Feeding Scale, and Early Feeding Skills (EFS).

Exploring Alternative Methods to Reduce Milk Flow Rate From Infant Bottle Systems: Bottle Angle, Milk Volume, and Bottle Ventilation.

Purpose: Modifying milk flow rate is a common pediatric dysphagia treatment. Though past investigations have demonstrated how this can be achieved using bottle nipples, little is known about the impact of other bottle modifications. The objective of this investigation is to demonstrate how bottle vent, bottle position, and volume of milk alter bottle pressures and milk flow.

Dysphagia Phenotypes in Spinal Muscular Atrophy: The Past, Present, and Promise for the Future.

Purpose The aim of this study was to provide clinicians with an overview of literature relating to dysphagia in spinal muscular atrophy (SMA) to guide assessment and treatment. Method In this clinical focus article, we review literature published in Scopus and PubMed between 1990 and 2020 pertaining to dysphagia in SMA across the life span. Original research articles that were published in English were included.

Effect of a standardized fluoroscopic procedural approach on fluoroscopy time during infant modified barium swallow studies.

Background: Pediatric dysphagia is a term used to describe dysfunctional feeding and swallowing in infants and children. It is estimated that about 1% of children in the United States are affected by these swallowing problems annually. The modified barium swallow study (MBSS) is considered by many as the gold standard in oropharyngeal swallowing assessment.

Capturing infant swallow impairment on videofluoroscopy: timing matters.

Background: Infant videofluoroscopic swallow studies (VFSSs) require clinicians to make determinations about swallowing deficits based on a limited number of fluoroscopically observed swallows. Although airway protection is known to decline throughout a bottle-feed, the paucity of data regarding the timing of this degradation has limited the development of procedural protocols that maximize diagnostic validity.

Objective: We tested the stability of key components of swallow physiology and airway protection at four standardized timepoints throughout the VFSS.

First Steps Towards Development of an Instrument for the Reproducible Quantification of Oropharyngeal Swallow Physiology in Bottle-Fed Children.

The incidence of feeding/swallowing impairments (deglutition disorders) in young children is rising and poses serious acute and long-term health consequences. Accurate detection and prompt intervention can lessen the impact of dysphagia-induced sequelae. Videofluoroscopic Swallow Studies (VFSSs) are used to make critical decisions for medically fragile children despite procedural variability and the lack of agreed upon measures for interpreting and reporting results.

Dysphagia in infants with single ventricle anatomy following stage 1 palliation: Physiologic correlates and response to treatment.

Background: Deficits in swallowing physiology are a leading morbidity for infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliation. Despite the high prevalence of this condition, the underlying deficits that cause this post-operative impairment remain poorly understood.

Objective: Identify the physiologic correlates of dysphagia in infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliative surgery.