Question: Looking for input on weaning thickener for 3 y/o with down syndrome. Attempted the systematic weaning protocol, but patient did not tolerate past 20% decrease. Patient previously used Vital Stim with minimal results, but I’m unclear on how long it was utilized. Any courses or articles people can point me to help? Thanks in advance. I feel like I keep taking courses and reading things, but there’s just so much to know and keep up with. It’s kind of overwhelming.
Catherine’s Answer: The clinical weaning program for our patients with Down Syndrome will require the use of objective data via VFSS regarding the impact of the change on physiology and airway protection. The literature, much of it recent, is worrisome for silent aspiration due to pathophysiology, likely related to altered sensory and motor components for swallowing. Weaning without the benefit of objective data about the impact could inadvertently adversely affect airway protection for some children. Boston Children’s did a study showing that our clinical impressions about airway invasion risk are often not reliable. That means we must carefully weigh the risk-benefit ratio of clinical weaning for each patient. No intervention is for all patients. It is only as good as the skill of the clinician to problem solve its appropriateness given the big picture.
Follow-up question from therapist: What would you recommend for patients that would like to work on weaning as a VFSS is not typically available in most settings? I’m trying to be explore as many options as possible to provide support to parents. It’s possible they may not be able to be weaned, but I want to make sure I’ve got explored all options to support this family.
Catherine’s follow-up answer: Can you tell me more about history and co-morbidities, and the impressions about swallowing pathophysiology from the most recent VFSS, what level of thickening is he on, by what utensil, is that what was originally objectified on the most recent VFSS (or is there no objective data for that thickening with that utensil?) , respiratory history and interval hospitalizations, what you are seeing clinically? I know that’s a lot of questions but without that info it’s like pulling something out of the air – not useful. Each piece of data has meaning to me as I problem-solve and do a mini-differential (with the caveat that I don’t really know the child and have not laid eyes/hands on the child). The risk is much higher without objective data from a VFSS…knowing more about the bigger picture will help me consider the potential risks to pulmonary health for this child. Even if the VFSS isn’t available in the setting in which you work, such as EI, working through logistics to obtain objective data about the impact on swallowing physiology through a VFSS will always support a more protective plan, especially in the setting of DS. If I were not practicing in acute care, but were in EI, with what I know about the patient so far, and have read in the literature, I’d be wanting objective data from a VFSS. Recent papers have stated that “the treatment should be based on data acquired from an instrumental assessment…”. That causes us to take pause, given that if there are unintended consequences to your plan of care, an expert witness may cite these papers. Careful assessment of the risk-benefit ratio of proceeding without objective data is the treating clinician’s judgement. As a former expert witness, I always to try in my clinical practice to minimize my risks as a professional, knowing that litigation can sometimes come from unintended sources and families in grief. Please don’t take these comments as criticizing your practice — they are not meant to at all, but rather to add another voice of experience that might help someone out there. Your post clearly supports your critical thinking, and your little patient is fortunate to have you supporting the family and safety.
Addendum: Selected References
Velayutham, Priatharisiny, Alexandria L. Irace, Kosuke Kawai, Pamela Dodrill, Jennifer Perez, Monica Londahl, Lauren Mundy, Natasha D. Dombrowski, and Reza Rahbar. “Silent aspiration: who is at risk?” The Laryngoscope 128, no. 8 (2018): 1952-1957.
Weir, K., McMahon, S., Barry, L., Ware, R., Masters, I. B., & Chang, A. B. (2007). Oropharyngeal aspiration and pneumonia in children. Pediatric pulmonology, 42(11), 1024-1031.
Hendrix, J. A., Amon, A., Abbeduto, L., Agiovlasitis, S., Alsaied, T., Anderson, H. A., … & Yi, J. S. (2021). Opportunities, barriers, and recommendations in Down syndrome research. Translational science of rare diseases, 5(3-4), 99-129
Tutor, J. D. (2020). Dysphagia and chronic pulmonary aspiration in children. Pediatrics in Review, 41(5), 236-244.
Bush, D., Galambos, C., & Dunbar Ivy, D. (2021). Pulmonary hypertension in children with Down syndrome. Pediatric pulmonology, 56(3), 621-629.
Brumbaugh, D. E., & Accurso, F. J. (2002). Persistent silent aspiration in a child with Trisomy 21. Current opinion in pediatrics, 14(2), 231-233.
Stanley, M. A., Shepherd, N., Duvall, N., Jenkinson, S. B., Jalou, H. E., Givan, D. C., … & Roper, R. J. (2019). Clinical identification of feeding and swallowing disorders in 0–6 month old infants with Down syndrome. American Journal of Medical Genetics Part A, 179(2), 177-182.
Nordstrøm, M., Retterstøl, K., Hope, S., & Kolset, S. O. (2020). Nutritional challenges in children and adolescents with Down syndrome. The Lancet Child & Adolescent Health, 4(6), 455-464.