Global State of the Art and Science of Childhood Dysphagia: Similarities and Disparities in Burden.

Feeding/swallowing and airway protection are complex functions, essential for survival, and continue to evolve throughout the lifetime. Medical and surgical advances across the globe have improved the long-term survival of medically complex children at the cost of increasing comorbidities, including dysfunctional swallowing (dysphagia). Dysphagia is prominent in children with histories of preterm birth, neurologic and neuromuscular diagnoses, developmental delays, and aerodigestive disorders; and is associated with medical, health, and neurodevelopmental problems; and long-term socioeconomic, caregiver, health system, and social burdens. Despite these survival and population trends, data on global prevalence of childhood dysphagia and associated burdens are limited, and practice variations are common. This article reviews current global population and resource-dependent influences on current trends for children with dysphagia, disparities in the availability and access to specialized multidisciplinary care, and potential impacts on burdens. A patient example will illustrate some questions to be considered and decision-making options in relation to age and development, availability and accessibility to resources, as well as diverse cultures and family values. Precise recognition of feeding/swallowing disorders and follow-up intervention are enhanced by awareness and knowledge of global disparities in resources. Initiatives are needed, which address geographic and economic barriers to providing optimal care to children with dysphagia.

Factors That Impact the Timing and Removal of Gastrostomy Placement/Nissen Fundoplication in Children with Bronchopulmonary Dysplasia.

OBJECTIVE: The study aimed to identify factors that impact timing of gastrostomy placement/removal and Nissen fundoplication (NF) in infants with bronchopulmonary dysplasia (BPD). STUDY DESIGN: Clinical data were reviewed retrospectively from patients recruited from the Johns Hopkins Bronchopulmonary Dysplasia Clinic (January 1, 2014-December 31, 2018). RESULTS: Patients with gastrostomy tubes (GTs) placed in the neonatal intensive care unit (NICU) were older at discharge (p < 0.001) and less likely to have abnormal upper gastrointestinal series findings (p = 0.005) than those with GTs placed after NICU discharge. Patients with NF had lower mean gestational ages (p = 0.011), longer NICU stays (p = 0.019), more frequent home ventilation requirements (p = 0.005), and greater likelihood of pulmonary hypertension (p = 0.032) compared with those without. Median age of GT removal was 61.6 months. Patients with GTs were weaned from supplemental oxygen and/or home ventilation before GT removal (p < 0.001). CONCLUSION: Patients with GT/NF were more medically complex than those with GT alone. Patients were more likely to be weaned from home respiratory support before GT removal. KEY POINTS: · Patients with GT/NF were more medically complex than those with GT alone.. · Patients were more likely to be weaned from home respiratory support before GT removal.. · Patients with GTs placed in NICU were older at discharge and less likely to have abnormal upper gastrointestinal series result..

Treatment of Pediatric Patients With High-Flow Nasal Cannula and Considerations for Oral Feeding: A Review of the Literature.

Purpose: High-Flow Nasal Cannula (HFNC) has become an increasingly common means of noninvasive respiratory support in pediatrics and is being used in infants and children with respiratory distress both inside and outside of the intensive care units. Despite the widespread use of HFNC, there remains a paucity of data on optimal flow rates and its impact on morbidity, mortality, and desired outcomes. Given the scarcity of information in these critical areas, it is not surprising that guidelines for initiation of oral feeding do not exist. This review article will review HFNC mechanisms of action, its use in specific populations and settings, and finally what is known about initiation of feeding during this therapy. Conclusions: The practice of withholding oral feeding solely, because of HFNC, is not supported in the literature at the time of this writing, but in the absence of safety data from clinical trials, clinicians should proceed with caution and consider patient-specific factors while making decisions about oral feeding. Well-controlled prospective clinical trials are needed for development of best practice clinical guidelines and attainment of optimal outcomes.

Translation, Cultural Adaptation, Reliability, and Validity Evidence of the Feeding/Swallowing Impact Survey (FS-IS) to Brazilian Portuguese.

The purpose of this study was to translate and adapt the Feeding/Swallowing Impact Survey (FS-IS) into Brazilian Portuguese and provide a validated instrument for caregivers of children with feeding/swallowing disorders. This cross-cultural study involved initial translation, synthesis of translations, back-translation, Committee of Experts, and pre-test. The sample consisted of 95 primary caregivers of children with feeding/swallowing disorders classified by Pediatric Dysphagia Evaluation Protocol (PDEP) in mild (n = 9), moderate-severe (n = 40), or profound (n = 46) dysphagia. Reliability and evidence of validity based on test content, response processes, internal structure and the relations to other variables were investigated. Internal consistency, test-retest, exploratory and confirmatory factor analysis were performed, in addition to the correlation with PedsQL™ Family Impact Module (PedsQLTMFIM). The pre-test participants did not report any difficulties in understanding the translated version. The Brazilian Portuguese version of FS-IS (Pt-Br-FS-IS) presented Cronbach's Alpha of 0.83, Exploratory Factor Analysis verified that the instrument would not be unifactorial (KMO = 0.74 and Bartlett's sphericity test p < 0.001) and Confirmatory Factor Analysis confirmed the original model in three subscales with χ2/df = 1.23, CFI = 0.92, TLI = 0.90, RMSEA (90% CI) 0.049 (0.011-0.073) adjustment indexes and the ICC was excellent in all subscales and total score. The correlation with PedsQL™FIM was significant in the total score and subscales. This study successfully translated and cross-culturally adapted the FS-IS instrument to the Brazilian Portuguese language and the investigation of its reliability and validity evidence suggests that the Pt-Br-FS-IS is a reliable and valid tool to measure the impact of feeding/swallowing disorders on the quality of life of caregivers of affected children.

Multidisciplinary Management of Ataxia Telangiectasia: Current Perspectives.

Ataxia telangiectasia (A-T) is a rare autosomal recessive disease caused by mutations in the ataxia telangiectasia mutated (ATM) gene. In the absence of a family history, the diagnosis of A-T is usually not made until the child is older and symptomatic. Classic A-T is characterized by a constellation of clinical symptoms including progressive ataxia, oculocutaneous telangiectasias and sinopulmonary disease and is usually associated with absence of ATM protein. Other laboratory features associated with A-T include elevated serum levels of alpha-fetoprotein (AFP) and increased chromosomal breakage with in vitro exposure to ionizing radiation. Sinopulmonary symptoms can occur to varying degrees across the lifespan. Some children will also have hypogammaglobulinemia and impaired antibody responses requiring supplemental gamma globulin. People with hypomorphic ATM mutations are often considered to have mild A-T with onset of ataxia and neurological progression occurring later in life with less impairment of the immune system. The risk of malignancy, however, is significantly increased in people with either classic or mild A-T. While hematological malignancies are most common in the first two decades of life, solid organ malignancies become increasingly common during young adulthood. Deterioration of neurologic function with age is associated with dysphagia with aspiration, growth faltering, loss of ambulation and decline in pulmonary function, morbidities that contribute to shortened life expectancy and decreased quality of life. Premature death is often due to malignancies or chronic respiratory insufficiency. A-T is currently managed with supportive care and symptomatic treatment. Current clinical trials, however, represent progress and hope towards disease-modifying therapies for A-T.

Growth in ataxia telangiectasia.

BACKGROUND: Ataxia telangiectasia (A-T) is a DNA repair disorder that affects multiple body systems. Neurological problems and immunodeficiency are two important features of this disease. At this time, two main severity groups are defined in A-T: classic (the more severe form) and mild. Poor growth is a common problem in classic A-T. An objective of this study was to develop growth references for classic A-T. Another objective was to compare growth patterns in classic A-T and mild A-T with each other and with the general population, using the CDC growth references. A final objective was to examine the effects of chronic infection on height. RESULTS: We found that classic A-T patients were smaller overall, and suffered from height and weight faltering that continued throughout childhood and adolescence. When compared to the CDC growth references, the median heights and weights for both male and female patients eventually fell to or below the 3rd centile on the CDC charts. Height faltering was more pronounced in females. Birthweight was lower in the classic A-T group compared to mild A-T and the general population, whereas birth length was not. Finally, we investigated height and BMI faltering in relation to number of infections and found no association. CONCLUSIONS: Classic A-T appears to affect growth in utero. Although children appear to grow well in very early life, faltering begins early, and is unrelenting.

Inflammatory Effects of Thickened Water on the Lungs in a Murine Model of Recurrent Aspiration.

OBJECTIVE: Liquid thickeners are commonly recommended in individuals with dysphagia and recurrent aspiration as a strategy for pneumonia prevention. The goal of this study was to examine the effects of small amounts of aspirated liquid thickener on the lungs. STUDY DESIGN: Animal model. Prospective small animal clinical trial. METHODS: Adult Sprague Dawley rats (n = 19) were divided into two groups and underwent three intratracheal instillations of either xanthan gum-based nectar-thick water (0.1-0.25 mL/kg) or water-only control over the course of 8 days. Blood was collected from a peripheral vein on days 1 and 8 and submitted for complete blood count (CBC) analysis. Rats were euthanized 10 days after the last instillation, and the lungs were harvested. Histopathology was conducted on lung specimens by a blinded licensed veterinary pathologist and scored for evidence of lung injury and pneumonia. RESULTS: Fifteen animals (8 nectar-thickener group, 7 control group) survived until the endpoint of the study (day 18). Serum CBC did not show abnormalities at any timepoint in either group. Histological evidence of lung inflammation and edema were significantly greater in the nectar-thick group compared to controls (P < .05). Signs of inflammation included aggregates of foamy macrophages, expansion of bronchiolar lymphoid tissue, and large numbers of eosinophilic intraalveolar crystals. Histiocytic and neutrophilic pneumonia was noted in one animal that received thickened liquids. CONCLUSION: Recurrent aspiration of small amounts of thickened water resulted in significant pulmonary inflammation in a murine model of aspiration. Results of this study support the need for further investigation of liquid thickener safety and its efficacy in reducing the pulmonary complications of swallowing disorders. LEVEL OF EVIDENCE: NA Laryngoscope, 131:1223-1228, 2021.

A Multidisciplinary Approach to Pediatric Feeding Disorders: Roles of the Speech-Language Pathologist and Behavioral Psychologist.

Purpose Pediatric feeding disorders (PFDs) present as a complex clinical challenge because of the heterogeneous underlying etiologies and their impact on health, safety, growth, and psychosocial development. A multidisciplinary team approach is essential for accurate diagnosis and prompt interventions to lessen the burdens associated with PFDs. The role of the speech-language pathologist (SLP) as a member of the multidisciplinary team will be highlighted. Method This clinical focus article reviews the definition of PFDs and pertinent literature on factors that contribute to the development of PFDs, the accurate diagnosis, and current interventions for infants and children. As part of the multidisciplinary team, the SLP has an integral role in determining whether a child cannot or will not eat and working with the team to identify and carryout appropriate interventions. Collaboration between SLPs and psychologists/behavioral specialists in conjunction with the parents/caregivers as part of the multidisciplinary team is essential to the advancement of therapeutic goals. Conclusions Due to their complex nature, the successful management of PFDs is only possible with the care and expertise of a multidisciplinary team, which includes parents/caregivers. SLPs are important members of these multidisciplinary teams and provide valuable input for the accurate identification and effective remediation of PFDs.

Best Practices in Modified Barium Swallow Studies.

Purpose The modified barium swallow study (MBSS) is a widely used videofluoroscopic evaluation of the functional anatomy and physiology of swallowing that permits visualization of bolus flow throughout the upper aerodigestive tract in real time. The information gained from the examination is critical for identifying and distinguishing the type and severity of swallowing impairment, determining the safety of oral intake, testing the effect of evidence-based frontline interventions, and formulating oral intake recommendations and treatment planning. The goal of this review article is to provide the state of the science and best practices related to MBSS. Method State of the science and best practices for MBSS are reviewed from the perspectives of speech-language pathologists (SLPs) and radiologists who clinically practice and conduct research in this area. Current quandaries and emerging clinical and research trends are also considered. Results This document provides an overview of the MBSS and standards for conducting, interpreting, and reporting the exam; the SLPs' and radiologist's perspectives on standardization of the exam; radiation exposure; technical parameters for recording and reviewing the exam; the importance of an interdisciplinary approach with engaged radiologists and SLPs; and special considerations for examinations in children. Conclusions The MBSS is the primary swallowing examination that permits visualization of bolus flow and swallowing movement throughout the upper aerodigestive tract in real time. The clinical validity of the study has been established when conducted using reproducible and validated protocols and metrics applied according to best practices to provide accurate and reliable information necessary to direct treatment planning and limit radiation exposure. Standards and quandaries discussed in this review article, as well as references, provide a basis for understanding the current best practices for MBSS.

BaByVFSSImP© A Novel Measurement Tool for Videofluoroscopic Assessment of Swallowing Impairment in Bottle-Fed Babies: Establishing a Standard.

AIM: This investigation tested the construct validity of the first standardized assessment tool, the BaByVFSS Impairment Profile, (BaByVFSSImP©), developed for the quantification of swallowing observations made from videofluoroscopic swallow studies (VFSS) in bottle-fed babies. METHOD: Construct validity of the measures was tested using descriptive methods and confirmatory factor analysis (CFA) of swallowing scores obtained from a cohort of bottle-fed babies (median age 3 months 1 day, interquartile range 1 month 4 days-7 months 4 days) sequentially referred for VFSS based on clinical signs, symptoms, or risk factors associated with dysphagia and/or aspiration. Main outcome measures were emergence of functional domains derived from swallowing component impairment scores. RESULTS: Confirmatory factor analysis resulted in 21 significant components (factor loadings ≥ 0.5) grouping into five functional domains labeled for common contribution to overall swallowing function. The tool was organized into the BaByVFSSImP. Clinical relevance was explored using correlational analyses between domain scores, maximum penetration/aspiration scores, feeding status, and caregiver burden. INTERPRETATION: Quantification of physiologic swallowing impairment captured by BaByVFSSImP holds promise for identification of physiologically based targets for intervention, clinical decisions regarding enteral feeding, and tracking the trajectory of swallowing impairment throughout development in young children.

Quantification of the source, amount and duration of aspiration in the lungs of infants using gamma scintigraphy.

BACKGROUND: Aspiration can cause acute symptoms and chronic lung disease in the developing lung. However, the source of aspiration in infants is often unclear, making the choice of intervention difficult. OBJECTIVE: To quantify the source, amount and duration of lung aspiration in infants using gamma scintigraphy. METHODS: Two infants with clinical evidence of gastroesophageal reflux and oropharyngeal dysphagia swallowed formula radiolabeled with 99mtechnetium on Visit 1. Radiolabeled-formula was instilled by nasogastric tube on Visit 2. Lung aspiration was quantified over four hours and expressed as percent of total radioactivity administered. RESULTS: Aspiration was greatest with swallowing, compared to instillation, peaking between 2.0% and 2.4% within 30 min and between 0.40% and 0.65% within 20 min, respectively. Radioactivity remained above zero four hours after either administration. CONCLUSIONS: Quantification of the source, amount and duration of lung aspiration in infants is feasible using gamma scintigraphy. The impact of aspiration accrual on clinical care deserves further investigation.

Feeding methods for infants with single ventricle physiology are associated with length of stay during stage 2 surgery hospitalization.

BACKGROUND: Tube feedings are often needed to achieve the growth and nutrition goals associated with decreased morbidity and mortality in patients with single ventricle anatomy. Variability in feeding method through the interstage period has been previously described, however, comparable information following stage 2 palliation is lacking. OBJECTIVES: To identify types of feeding methods following stage 2 palliation and their influence on length of stay. DESIGN: Secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative registry was performed on 932 patients. Demographic data, medical characteristics, postoperative complications, type of feeding method, and length of stay for stage 2 palliation were analyzed. RESULTS: Type of feeding method remained relatively unchanged during hospitalization for stage 2 palliation. Gastrostomy tube fed only patients were the oldest at time of surgery (182.7 ± 57.7 days, P < .001) and had the lowest weight-for-age z scores at admission (-1.6 ± 1.4, P < .001). Oral + gastrostomy tube groups had the longest median bypass times (172.5 minutes, P = .001) and longest length of stay (median 12 days, P < .001). Multivariable modeling revealed that feeding by tube only (P < .001), oral + tube feeding (P ≤ .001), reintubation (P < .001), and prolonged intubation (P < .001) were associated with increased length of stay. Neither age (P = .156) nor weight-for-age z score at admission (P = .066) was predictive of length of stay. CONCLUSIONS: Feeding methods established at admission for stage 2 palliation are not likely to change by discharge. Length of stay is more likely to be impacted by tube feeding and intubation history than age or weight-for-age z score at admission. Better understanding for selection of feeding methods and their impact on patient outcomes is needed to develop evidence-based guidelines to decrease variability in clinical practice patterns and provide appropriate counseling to caregivers.

Structure and Functions of Pediatric Aerodigestive Programs: A Consensus Statement.

Aerodigestive programs provide coordinated interdisciplinary care to pediatric patients with complex congenital or acquired conditions affecting breathing, swallowing, and growth. Although there has been a proliferation of programs, as well as national meetings, interest groups and early research activity, there is, as of yet, no consensus definition of an aerodigestive patient, standardized structure, and functions of an aerodigestive program or a blueprint for research prioritization. The Delphi method was used by a multidisciplinary and multi-institutional panel of aerodigestive providers to obtain consensus on 4 broad content areas related to aerodigestive care: (1) definition of an aerodigestive patient, (2) essential construct and functions of an aerodigestive program, (3) identification of aerodigestive research priorities, and (4) evaluation and recognition of aerodigestive programs and future directions. After 3 iterations of survey, consensus was obtained by either a supermajority of 75% or stability in median ranking on 33 of 36 items. This included a standard definition of an aerodigestive patient, level of participation of specific pediatric disciplines in a program, essential components of the care cycle and functions of the program, feeding and swallowing assessment and therapy, procedural scope and volume, research priorities and outcome measures, certification, coding, and funding. We propose the first consensus definition of the aerodigestive care model with specific recommendations regarding associated personnel, infrastructure, research, and outcome measures. We hope that this may provide an initial framework to further standardize care, develop clinical guidelines, and improve outcomes for aerodigestive patients.

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. This investigation represents the first steps in the development of a novel tool for the quantification of oropharyngeal swallow physiology from full-length VFSS examinations in bottle-fed children. The Modified Barium Swallow Impairment Profile MBSImP™© served as the conceptual assessment model for development of components and operational score variants to characterize distinguishable VFSS observations. Twenty-four components of swallowing physiology were validated via expert consensus. Training materials included a library of 94 digitized video images comprised of distinct score variants for each component. Materials were disseminated to seven speech-language pathologists (SLPs) who participated in didactic and self-training sessions, and rated components. All SLPs achieved ≥80% reliability criterion after completing two or three training sessions. Agreement for 17 (71%) components was achieved after two sessions. Nutritive sucking/oral and airway-related components were most difficult to distinguish. Three sessions were required for 2 (33%) of the sucking/oral components and 4 (57%) of the airway-related components. These findings support the feasibility to standardize training and reliably score swallowing physiology using precise definitions and unambiguous visual images, and represent preliminary steps towards content validity and reliability of a standardized VFSS tool for bottle-fed children.

Instrumental Assessment of Pediatric Dysphagia.

Speech-language pathologists (SLPs) have fulfilled primary roles in the evaluation and management of children with feeding/swallowing disorders for more than five decades. The increased incidence and prevalence of newborns, infants, and children with feeding and swallowing disorders has resulted in increased use of instrumental swallowing evaluations. The videofluoroscopic swallow study and fiberoptic endoscopic evaluation of swallowing are the two most commonly used swallowing assessments by SLPs, with ultrasound used less frequently. This article focuses on updates over the past decade in the procedures and utility of instrumental assessments of swallowing function, and identifies future directions that may enable us to meet the needs of the children who are in our care to attain functional outcomes.

Ataxia telangiectasia: a review.

DEFINITION OF THE DISEASE: Ataxia telangiectasia (A-T) is an autosomal recessive disorder primarily characterized by cerebellar degeneration, telangiectasia, immunodeficiency, cancer susceptibility and radiation sensitivity. A-T is often referred to as a genome instability or DNA damage response syndrome. EPIDEMIOLOGY: The world-wide prevalence of A-T is estimated to be between 1 in 40,000 and 1 in 100,000 live births. CLINICAL DESCRIPTION: A-T is a complex disorder with substantial variability in the severity of features between affected individuals, and at different ages. Neurological symptoms most often first appear in early childhood when children begin to sit or walk. They have immunological abnormalities including immunoglobulin and antibody deficiencies and lymphopenia. People with A-T have an increased predisposition for cancers, particularly of lymphoid origin. Pulmonary disease and problems with feeding, swallowing and nutrition are common, and there also may be dermatological and endocrine manifestations. ETIOLOGY: A-T is caused by mutations in the ATM (Ataxia Telangiectasia, Mutated) gene which encodes a protein of the same name. The primary role of the ATM protein is coordination of cellular signaling pathways in response to DNA double strand breaks, oxidative stress and other genotoxic stress. DIAGNOSIS: The diagnosis of A-T is usually suspected by the combination of neurologic clinical features (ataxia, abnormal control of eye movement, and postural instability) with one or more of the following which may vary in their appearance: telangiectasia, frequent sinopulmonary infections and specific laboratory abnormalities (e.g. IgA deficiency, lymphopenia especially affecting T lymphocytes and increased alpha-fetoprotein levels). Because certain neurological features may arise later, a diagnosis of A-T should be carefully considered for any ataxic child with an otherwise elusive diagnosis. A diagnosis of A-T can be confirmed by the finding of an absence or deficiency of the ATM protein or its kinase activity in cultured cell lines, and/or identification of the pathological mutations in the ATM gene. DIFFERENTIAL DIAGNOSIS: There are several other neurologic and rare disorders that physicians must consider when diagnosing A-T and that can be confused with A-T. Differentiation of these various disorders is often possible with clinical features and selected laboratory tests, including gene sequencing. ANTENATAL DIAGNOSIS: Antenatal diagnosis can be performed if the pathological ATM mutations in that family have been identified in an affected child. In the absence of identifying mutations, antenatal diagnosis can be made by haplotype analysis if an unambiguous diagnosis of the affected child has been made through clinical and laboratory findings and/or ATM protein analysis. GENETIC COUNSELING: Genetic counseling can help family members of a patient with A-T understand when genetic testing for A-T is feasible, and how the test results should be interpreted. MANAGEMENT AND PROGNOSIS: Treatment of the neurologic problems associated with A-T is symptomatic and supportive, as there are no treatments known to slow or stop the neurodegeneration. However, other manifestations of A-T, e.g. immunodeficiency, pulmonary disease, failure to thrive and diabetes can be treated effectively.

Pediatric Feeding/Swallowing: Yesterday, Today, and Tomorrow.

Speech-language pathologists (SLPs) have played primary roles in the evaluation and management of children with feeding/swallowing disorders for more than five decades. Medical, surgical, and technological advances have improved the survival of young fragile infants and children, many of whom will present with feeding/swallowing problems. Regardless of their underlying etiologies, many of these children are at risk for aspiration-induced lung disease, undernutrition or malnutrition, developmental deficits, and stressful interactions with their caregivers. Unfortunately, our understanding of the physiology/pathophysiology of swallowing and its maturation, the development of standardized and efficacious evaluation and therapy tools, and identification of functional outcomes have not kept pace with our ability to identify children who are at increased risk for dysphagia and the associated sequelae. Given this paucity of evidence to guide practice in pediatrics, clinicians rely upon a combination of data extrapolated from adults with dysphagia, anecdotal reports, and institution-specific guidelines. This article focuses on updates in population demographics and advances in evaluation and treatment over the past decade and identifies future directions that may enable us to meet the needs of the children who are in our care to attain functional outcomes.

Assessment of impaired coordination between respiration and deglutition in children and young adults with ataxia telangiectasia.

AIM: This cross-sectional investigation aimed to assess the value of non-invasive measures of temporal respiratory-swallow coupling in individuals with ataxic swallowing. METHOD: Twenty participants (11 males, 9 females; range 9-21y) with ataxia telangiectasia were presented with water and pudding boluses. Their 193 swallows were compared with 2200 swallows from 82 age-matched healthy controls. The two components of airway protection during swallowing that were analyzed were: direction of peri-deglutitive airflow and duration of deglutitive inhibition of respiratory airflow (DIORA). RESULTS: Safe expiratory patterns of peri-deglutitive airflow occurred significantly less often in participants with ataxia telangiectasia than in age-matched control participants (younger p<0.015 and older p<0.001). The frequency of an expiratory pattern of peri-deglutitive airflow increased with age in participants in the comparison group (p=0.006), but not in those with ataxia telangiectasia (p=0.234). With age, mean duration of DIORA decreased in controls (p<0.001) but was unchanged in participants with ataxia telangiectasia (p=0.164). INTERPRETATION: Non-invasive quantitative measures of respiratory-swallow coupling capture temporal relationships that plausibly contribute to airway compromise from dysphagia. Changes in respiratory-swallow coupling observed with advancing age in control participants were not seen in participants with ataxia telangiectasia. Measures of perturbations may herald swallowing problems prior to development of pulmonary and nutritional sequelae.