Catherine Shaker Fall Seminars to Inform Your Practice

Join your colleagues in Austin or Houston for advanced clinical learning opportunities! Choose your focus: advanced pediatric swallowing/feeding or pediatric/neonatal swallow studies or state-of-the-art NICU assessment.

October 22-23 (Austin) or November 10-11 (Houston): The Early Feeding Skills Assessment Tool: A Guide to Cue-Based Feeding in the NICU

October 24 (Austin) or November 12 (Houston): Neonatal/Pediatric Videofluoroscopic Swallow Studies.

October 25-26 (Austin) or November 13-14 (Houston): Advanced Infant/Pediatric Dysphagia: Problem-Solving Complex Patients and Practice Challenges 

Each state-of-the-art seminar infused with the latest research and problem-solving, enhanced by my 45 years of progressive clinical experience. I’ll be teaching the NICU-focused seminar with Dr. Suzanne Thoyre, PhD RN, a gifted, acclaimed NICU researcher and neonatal nurse, who beings insights that support evidence-based feeding practice, partnership with neonatal nurses, and strategies to guide your NICU team to better feeding outcomes.

A welcoming environment that fosters interaction and learning along with each other. Will change your practice.

Click here to download Shaker 2022 On-Site Seminars Brochure

Click here to Register 

Click here for On-Site Information for Austin

Click here for On-Site Information for Houston

Research Corner: Congenital Heart Disease and Vocal Fold Immotility

Congratulations to our pediatric colleague, Christine Rappazzo, for this wonderful addition to our evidence base related to the potential impact of the need for heart surgery on airway protection in our infant population. This, combined with the documented increased risk for R vocal fold motion impairment post ECMO in this population, helps us to advocate for our involvement in safe progression to PO for these vulnerable infants.

Citation: Narawane, A., Rappazzo, C., Hawney, J., Clason, H., Roddy, D. J., & Ongkasuwan, J. (2021). Vocal Fold Movement and Silent Aspiration After Congenital Heart Surgery. The Laryngoscope.

Abstract

Infants who undergo congenital heart surgery are at risk of developing vocal fold motion impairment (VFMI) and swallowing difficulties. This study aims to describe the dysphagia in this population and explore the associations between surgical complexity and vocal fold mobility with dysphagia and airway protection.

Methods

This is a retrospective chart review of infants (age <12 months) who underwent congenital heart surgery between 7/2008 and 1/2018 and received a subsequent videofluoroscopic swallow study (VFSS). Demographic information, Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) category of each surgery, vocal fold mobility status, and VFSS findings were collected and analyzed.

Results

Three hundred and seventy-four patients were included in the study. Fifty-four percent of patients were male, 24% were premature, and the average age at the time of VFSS was 59 days. Sixty percent of patients had oral dysphagia and 64% of patients had pharyngeal dysphagia. Fifty-one percent of patients had laryngeal penetration and 45% had tracheal aspiration. Seventy-three percent of these aspirations were silent. There was no association between surgical complexity, as defined by the STAT category, and dysphagia or airway protection findings. Patients with VFMI after surgery were more likely to have silent aspiration (odds ratio = 1.94, P < .01), even when adjusting for other risk factors.

Conclusion

Infants who undergo congenital heart surgery are at high risk for VFMI and aspiration across all five STAT categories. This study demonstrates the high prevalence of silent aspiration in this population and the need for thorough postoperative swallow evaluation.

This will inform your pediatric practice whether in the inpatient or community pediatric setting.

 

 

 

 

Learning about Feeding/Swallowing: An International Pediatric Perspective

I was thrilled to welcome new SLP friends from Europe who traveled to Chicago to learn along with me! Florentine (left) from The Netherlands, and Heida (right) from Iceland.  Between them, they traveled over 7000 miles to be with me for 5 days. Despite our geographic distance, our infant and pediatric patents are so similar, as are the challenges we face each day. Their approaches and physician/institutional influences really are not all that different but their wisdom in thriving with different and less abundant resources were an eye opener for all of us. International perspectives on feeding and swallowing always round out conversations at my courses. It has been my pleasure over the years to welcome an ENT from Saudi Arabia, a pediatric dentist from Japan and in-the-trenches therapists from other far way places including Spain, Greece, the UK, South Africa, Australia, India, The United Arab Emirates, Ireland and Israel. Needless to say, my brain is on fire after the chance to share our passion for helping infants and children with feeding and swallowing problems. And it is great to have contacts for future problem-solving that literally span the globe! Safe travels to my new friends, Florentine and Heida!

Pediatric Swallowing/Feeding: “We are all in this together!”

I just returned from teaching in Chicago for 5 days at Advocate Aurora Healthcare.  What an amazing pediatric team they have…engaged, thoughtful and embracing both the evidence base and the value of problem-solving. We were joined by therapists from across the US and Europe for conversations about our shared challenges …complex infants and children who land on our doorsteps, navigating swallow studies with physician colleagues, implementing IDDSI, fostering cue-based feeding in a volume-driven culture, GI co-morbidities from FTT through  ARFID in kids, the latest information on aspiration in the pediatric population, risks for feeding on CPAP/HFNC, enduring impacts of neurologic sequalae, and integrating motor learning as the foundation for rehabilitating and habilitating the swallow from infants through adolescents, and so much more. We all came away with new ideas, new data, new perspectives and a resounding sense that we are all in this together as pediatric swallowing and feeding specialists! We can do this!

Problem-Solving: 30-month-old with Random Gagging and Altered Swallowing Physiology

QUESTION:

Recently, I completed a MBSs on a 30-month-old child secondary to complaints of vomiting, gagging/choking at random. At age 24 months, child had tonsillectomy/adenoidectomy completed secondary to vomiting/gagging with the hopes this would correct child’s difficulties. MOC reports that this did help some however reports difficulties have not completely resolved. Per MOC, child had pacifier until age of 20 months. At time of MBSs child did consume pureed and thin liquids, they refuse all other bolus consistencies. Child noted to have a high palate arch and slightly narrowing in front of mouth. Delayed swallow to the level of the pyriform sinus was observed, suspect secondary to poor retraction and elevation of tongue to soft palate due to high arch. No penetration or aspiration noted. Appropriate ROM with tongue was observed during attempts at oral facial/motor examination. Per MOC, child is a picky eater, consumes 30oz of milk daily, will pocket food and spit it out and gag on water intermittently.

Based off of how this child presents, I would focus on lingual strengthening, age-appropriate mastication patterns and acceptance of age-appropriate foods. For a child of this age, what would your recommendations be for treatment/tasks to obtain these goals/exercises etc.? I am running into a roadblock per say. From my research and reading, I have found great ideas for older children or adults who follow verbal directives, however due to this child’s age, I am stuck!

ANSWER:

Is the child otherwise normally developing? Is postural /sensory-motor control age-appropriate?  I am asking because sometimes this type of clinical presentation is part of a bigger sensory processing issue or part of a constellation of craniofacial alterations or alterations across developmental domains. That creates a different “bigger picture” from which to problem-solve.

Craniofacial malformations often co-occur with changes in the muscular network that supports those structures. The high arched (and sometimes “tented”) palate can co-occur as part of a genetic syndrome and can co-occur with mandibular hypoplasia. Mandibular hypoplasia alters lingual and supra/infrahyoid muscular ROM and their functional coordination.

Interestingly, I have seen across the age span that, with this clinical presentation you described, it is not uncommon to have co-occurring tethering of oral tissues. I suspect that, if there are TOTs, this may be because the formation of these structures and muscular attachments occurs around the same time in utero. Then their motor sequences are initially mapped in utero through swallowing of amniotic fluid. So, the underpinnings for a well-integrated oral-motor system are underway quite early. Maladaptive networks also start in utero and the foundations for function can then start off in infancy already altered and impact future function that “feeds forward”, as our PT colleagues call it, in motor learning. So, implications unfold overtime.

If there are tethered oral tissues, or related alterations, they can at times be more subtle. These alterations can create challenges for the emergence of motor plans along the swallow pathway, and for bolus control and manipulation. That may also provoke the air swallowing that can lead to the vomiting/emesis you report. We must of course recognize that tethered oral tissues are not always the explanation/etiology, but should be a part of your thoughtful differential, as it could explain the functional limitations you describe. As could altered oral-facial tone, an altered overriding postural network, and/or sensory integration problems, and other possibilities, depending on the unique “bigger picture” for this child. Thinking through that bigger picture will best guide targeted interventions.

A wonderful resource on the neurodevelopmental underpinnings for feeding development is included in Robyn Walsh and Lori Overland’s book on “Functional Assessment and remediation of Tethered Oral Tissues”. Even if TOTs are not part of this child’s etiology(ies), their tutorial included in their book is not just about TOTs but is a foundational must read on functional oral motor development. By two of our wonderful SLP colleagues.

The swallow study would likely reveal any alterations in base of tongue retraction and pressure generation that may be created if tethering were impacting this child’s swallow pathway. For some children, the oral phase appears most altered, but that of course can cause problems down the line such as gagging and “sudden” loss of control which mother describes, which may reflect challenges with coordination during the dynamic swallow (when the need for exquisite motor mapping is required).

The attached article by my colleague Laura Brooks that adds to our understanding of the potential implications of some of the potential alterations. Even though with your patient there was no witnessed airway invasion, there was an alteration in physiology that likely is connected somehow to the functional differences you are seeing. This may take some peeling apart layers of data through a second and third look. Or more. And it may take a while to sort out and trial the interventions that best meet your differential. And that’s ok. It’s complex but you have a good start.

Click here for Laura Brooks article

Practicing at the Top of Your Profession in Feeding/Swallowing

An SLP asked recently, “What resources, articles, courses, etc. did you find helpful when you were first learning infant feeding? I’m looking for something to give me what I really need.”

“My seminars” I told her, “are designed to do just that. When I created them, and as I update them, I always think ‘What do I wish I had known, both research and clinical information, to practice in peds dysphagia when I started out? What is essential to work toward practicing at the top of our professions?’  My NICU and Pediatric as well as Advanced seminars, my Peds/ Neonatal Video Swallow Studies and Cue Based seminars are filled with everything I want to pass along. As I learn from colleagues and attendees at my courses, I weave that in as well. Sometimes I almost run out of time!

I will always offer you a welcoming environment that fosters interaction and learning along with each other.

Click here to download Shaker 2022 On-Site Seminars Brochure

Click here to Register

 

Problem-Solving with Catherine: Navigating Acute Care/PICU Practice Challenges

QUESTION: I am working with a team of non-dysphagia specialists because of shortage of SLP (not in the USA) in pediatric ICU (PT, nurses). When I arrived, I realized that they do everything wrong in SLP standards : start dysphagia exam too early on, speed dysphagia bedside exam with a bit of water, rely only on coughing to assess if dysphagia is present or not, do not do a single swallow FEES/VFSS, do not even know/care about silent aspiration, progress up very rapidly through textures when mastication is not yet possible in my opinion, give rapid directions to families and do not overwatch. Of course, they refer to SLPs for complicated cases, they are scared with or when they are unsure. We are left with the slowly recovering kiddos. they simply judge by overall recovery. If overall is fine, dysphagia is fine for them. But I feel they would be more complications in the adult population however with this type of approach. I am starting to question my stance on dysphagia eval among children in ICU. Is it way simpler than we may think? Do you have an opinion?

ANSWER: I would continue to use the evidence base as your guide, combined with your clinical expertise. Our PICU patients are often fragile but the adverse response to decisions or approaches that are not consistent with best practice may be silent, may increase need for respiratory support without adverse overt events and the child may “run under the radar” for a while. That doesn’t mean you abandon your critical thinking. Not sure if this is a travel assignment or they are the newly added team members, but you will need to decide how best to advocate for your peds patients and to reconcile this risk. That means perhaps having a conversation with them that is respectful, offering practice guidelines by ASHA or research evidence, thinking about how to gain administrative support to foster collaboration and best practice in dysphagia, and what your next steps will be to assure there is appropriate care and colleagues “do no harm” which you likely have an obligation to address via risk management. Should something adverse occur and there is litigation, your documentation and whether it and your actions were consistent with best practice would be scrutinized and determined by an expert pediatric acute care SLP. Always manage patients and document as if litigation is possible, because it is. Trust your instincts. It’s a really challenging situation but best to take control of it in one way or another versus being a bystander when it may affect your professional practice and patient long-term outcomes. That may indeed be leaving if the risk to you is not acceptable, which is clearly possible based on what we know. I hope this is helpful.

 

Problem-Solvers Join Catherine Shaker for Advanced Learning Opportunities

Such a wonderful group joined me in Atlanta last week! Here are some of the amazing problem-solvers from Childrens Heathcare of Atlanta and from across the US who came together for my Cue-based Feeding and Advanced Infant/Pediatric Dysphagia seminars. It was a great opportunity to apply critical thinking to neonates and children through school-age/adolescence with feeding and swallowing challenges. Everyone’s brains were “on fire” as I like to say, with conversations about complex co-morbidities, navigating swallow studies and case discussions.

I hope to see you at one of my next offerings in Chicago, Austin or Houston!

Click here to download Shaker 2022 On-Site Seminars Brochure

Click here to Register

Celebrating the Promise of Cue Based Feeding for our Infants

Dr. Suzanne Thoyre,  a gifted, acclaimed NICU researcher and neonatal nurse, joined me this weekend at Childrens Healthcare of Atlanta for our Cue-based Feeding seminar. We shared our insights and evidence-based feeding practice from a combined nearly-100-years of practice in the NICU. Therapists from across the US and Canada learned along with us to explore infant-guided assessment and intervention and develop a common language in the NICU to support neuroprotection and better feeding outcomes for infants who require intensive care. One of the attendees noted: “As out NICU team continues to transition to cue-based feeding, we will be better able to establish treatment plans for our little ones and have the team on the same page.”

We hope to see you in Austin (October) or Houston (November) for this exceptional learning opportunity.

Click here to download Shaker 2022 On-Site Seminars Brochure

Click here to Register

Problem-Solving: RR and PO Feeding in the NICU

QUESTION: I have a 33wker with a VSD that nursing is PO feeding every feeding. Are there studies regarding respiratory rate while feeding? The infant looked comfortable at 65 and below but I told nursing to stop feeding with rate above 80 (or allow major rest break for her to slow it down because she’s often cueing).  I’d love hard data to support those numbers

ANSWER:

I don’t know of any randomized controlled trials that yielded this data. Clinically we know that spontaneous RR is typically different than monitored RR, due to an artifact created by the monitor not consistently sensing rapid shallow breaths. We gather he best data by counting the spontaneous breaths, as it’s more reliable. Anytime the RR is above 60 we know there is greater risk, as it takes a second to complete the pharyngeal swallow. RR over 60 increases risk that the swallow and breathing will co-occur or “collide” as I like to say to parents. Work of breathing often goes hand in hand with the RR but can often be a more worrisome indicator of risk for airway invasion. As the effort goes up, breaths are more shallow, there is then less tidal volume, and the infant is more likely to need an urgent breath. In those moments, the need to breathe will override the swallow. Often this means opening the airway too early or not fully closing it at the moment of the swallow. Because NICU infants are at higher risk to silently aspirate, the infant described may show no outward signs. We know he is a 33 weeker – which means to me that he was born at 33 weeks. What is his PMA? Or are you meaning he is now 33 weeks PMA; if so, what was his GA? Helps me make sense of his risk. The VSD will drive up WOB and RR at baseline, and with the aerobic demands of feeding. Does he have an otherwise unremarkable history except for VSD? Currently requiring respiratory support? What are saturations like with PO? What physiologic and behavioral stress signs do you see even with interventions? What interventions are the nurses using to protect him? Perhaps having you offer guided participation while a nurse feeds and you think along with her, might build a conversation about airway protection and neuroprotection, both of which are likely at risk given what we know.

Problem-Solving: Thickening Post VFSS

QUESTION:

I work in a pediatric hospital, and we’ve been running into difficulty when it comes to the recommendation to thicken liquids. Our speech dept procedure is not to recommend or endorse a specific product and place that responsibility on the physicians after we relay the MBS results. Often GI doctors are more comfortable with thickening choices, contra-indications, etc. but our pulmonologists and cardiologist feel that they do not have the training to make those choices and don’t feel comfortable. As a dept we want to support our doctors and I was hoping another facility has run into this issue or addressed it and could share their processes. Thank you!

ANSWER:

It is important I think to discuss options with families and providers, and consider home environment, access, cultural preference, caregiver capability and potentially the providers don’t have that insight. This goes a long way towards recommendations and strategies that are more likely to be carried out by families. Along the way we help providers who are not GI specialists benefit from our problem-solving and our ability to bring the current evidence-base to the conversation.

I rarely thicken liquids these days. When it is indicated as a last-resort intervention, based on objective data regarding pathophysiology and potential airway invasion observed during the VFSS in the setting of that child’s co-morbidities, I find a few things quite helpful.

During the VFSS, we have likely utilized particular thickeners that give us objective data about their impact, have flow tested the liquids with those thickeners, and have some impression as to what may be optimal (risk-benefit ratio from swallowing perspective). We might then offer that data as a part of the problem-solving discussion with the physician/team, recognizing that the prescriptive nature of what is ultimately determined by the physician’s order will be a biproduct of further discussion. This is an opportunity to collaborate about patient care and builds relationships with the medical team. This bringing together of each professional’s data and perspective has been referred to as “cross-fertilization”. I love that term and its implications for how we practice or might optimally practice as lifelong learners. With cross-fertilization, everyone’s engagement exposes all professionals to different bodies of scientific literature knowledge, as that supports collaborative practice. We all benefit from these exchanges, especially our patients and families.

Another resource to share with physicians that is, to my knowledge, part of the latest evidence-base we have available, is this wonderful paper by the team at Boston Childrens, including Kara Larson SLP and two amazing GI docs. As it states, the purpose of this review is to discuss current knowledge and recent findings regarding clinical aspects of thickeners for pediatric gastroesophageal reflux and oropharyngeal dysphagia. We review evidence for thickener efficacy, discuss types of thickeners, practical considerations when using various thickeners, and risks and benefits of thickener use in pediatrics.  

Duncan, D. R., Larson, K., & Rosen, R. L. (2019). Clinical aspects of thickeners for pediatric gastroesophageal reflux and oropharyngeal dysphagia. Current Gastroenterology Reports21(7), 1-9.

Problem-Solving: Young Infant with Mandibular Hypoplasia and Glossoptosis

Question:

I have an infant (2-week-old) coming in for a feeding eval next week (mother reported difficulty latching on breast and bottle, feeds last 30-40 min, infant not gaining back birth weight), referred by ENT, script says micrognathia and glossoptosis. I did a quick research scan and didn’t find a lot regarding micrognathia besides osteogenesis. Open to suggestions or any research articles you can point me to regarding this topic… who to refer to? any other treatment you’d recommend?

Answer:

This clinical presentation in infants can be quite complex. Sucking, swallowing and the swallow-breathe interface can all be affected, as can overall postural control and sensory-motor learning. Once you see the infant, can begin your differential about what relevant areas are impacting swallow function, and what other consults or diagnostics might be helpful.

Good that you already have an ENT involved who has evaluated the infant and provided impressions. Do you know if there was a flexible scope done as part of the ENT consult? You may want to check, as that will also be helpful to better understand the functional dynamics of the base of tongue on this infant’s swallow. Due to the glossoptosis, you will likely hear inspiratory stridor created by intermittent obstruction of the base of the tongue against the posterior pharyngeal wall. This can interfere with swallow-breathe interface and alter swallowing physiology. An elevated swaddled sidelying position will help to utilize the effects of gravity to help bring the mandible forward. An instrumental assessment of swallowing physiology will likely be a part of your differential, as there is often altered oral and pharyngeal phase physiology that creates risk for both silent and symptomatic airway invasion with this clinical presentation.

It is also likely that there may be associated tethered oral tissues, as the timing of developmental emergence of structures in utero predisposes these correlations. As mentioned already, the tethering, if it is present, can actually help the infant to compensate for the glossoptotic forces and therefore help keep the tongue from falling farther into the hypopharynx.  This reminds us of how important it is to not “signal react” to one aberration (e.g., tongue tie) , without considering its dynamic influence on the big picture, i.e., releasing the tethering would cause more problems, given this infant’s co-morbidities. So, compensating for the adverse impact of possible tethering on the suck may be a focus. The infant may have a “compression-only” sucking pattern r/t the glossoptosis in and of itself, as it often leads to a thickened and bunched lingual body (intrinsic and extrinsic tongue muscles often don’t develop properly in the setting of this malformation). The altered lingual control can affect not only establishment of suction but also oral bolus control, and cause “problems down the line” (i.e., with pharyngeal function). The Dr. Brown’s nipple with specialty valve may work but sometimes the tongue may be too far back in the mouth to even permit an effective latch with that system. An option would be the Haberman, but without any squeezing of the teat; increasing flow rate would be contraindicated with this clinical presentation, as it would increase bolus size and adversely affect bolus control and airway protection

Co-regulated pacing and resting will be essential, as the infant’s WOB (work of breathing) will be increased. Breathing is typically adversely affected by challenges with airway maintenance created by the need for further retraction of the tongue base that is part of the swallow process. For this infant, that will make it difficult for the infant to maintain the airway as it reconfigures from a respiratory tract to an alimentary retract dynamically during feeding.

Mother will likely need your support to focus on infant-guided feeding (versus volume), learning about what the infant’s behaviors during feeding mean (what infant is trying to communicate), waiting for the infant to root (that suggests he is then, at that moment, better managing his airway and is ready to suck), and thoughtfully and contingently offering interventions to optimize safety.

This wonderful paper by my friend and colleague Laura Brooks at Children’s Healthcare of Atlanta provides valued additional insights for my rationale to consider a swallow study to objectify physiology, given this infant’s co-morbidities:

Brooks, L., Evans, S., Alfonso, K. et al. The Role of Dysphagia Assessment in the Identification of Upper Airway Obstruction in Infants. Dysphagia (2022). https://doi.org/10.1007/s00455-022-10433-5

This pathophysiology Laura describes is not uncommon when an infant presents with these challenges. The alterations along the swallow pathway created by glossoptosis may be part of the reason for poor intake. Perhaps the infant is trying to protect his airway when he purposefully disengages during feeding. I appreciate Laura’s adding to our evidence base and reinforcing that “co-morbidities matter,” as I always like to say.

Problem-Solving: NICU infant aspiration versus LPR/EER?

Question:

I have a question about an NICU patient who is currently 39-3, formerly 25-2. BPD, chronic respiratory, very prolonged intubation. I did pre-feeding milk drops, etc but she never even began cueing until 35 weeks when on 2Liters. I used a Dr. Brown’s Ultra Preemie and progressed PO very slowly due to increased WOB. She has now had steroids and was just weaned to room air this past week. She takes full volume and is doing much better pacing herself and I have upgraded her to a Dr. Brown’s Preemie.  She has always had spells and apnea but for the past 3 weeks every time you go to burp her, she bears down like she is trying to maybe protect her airway from reflux, she then holds her breath and desats. I have tried smaller volumes and burping more frequently but no luck getting her burp until further into the feeding. The desats are not as low as they once were but I just feel like I see so many babies that are suffering from large volumes and experience reflux. She does have occasional choking spells and I offered to do MBSS but was shot down. The neo that day wanted to thicken with rice. We haven’t been thickening very much since I came to the unit and when the neos want to thicken they choose oatmeal. There is also a SIM spit up formula for term babies that is thicker. What should I do in this situation? I fear I am missing aspiration but most of the babies I take for fluoro don’t aspirate and I just see the retrograde flow (reflux). I have read laying them on their right side may help. What are our research proven reflux tools – I know a lot of the answer is -time to mature but is there anything else I should be doing?

 

Answer:

Sounds like you have offered her thoughtful preparation for infant-guided, neuroprotective feeding. I’d be extra careful about letting her set her own pace, given her respiratory hx. Even healthy term NBs don’t “pace themselves” until closer to 2 months of age per the research. Though she may take breaths on her own with PO, given her hx it is likely the breaths may be even a bit latent or a bit insufficient, or she may have some element of gulping that results in air ingestion. Have you tried burping in elongated sidelying? I find that better takes the pressure off the abdomen than semi-upright burping in caregiver’s lap (since increased abdominal pressure which can provoke EER/LPR). The choking could be co-occurring EER/LPR or events of airway invasion/t mistiming of swallow-breathe interface, which is what we typically see if we were to do a VFSS on an infant with her hx and diagnoses. We rarely thicken too. Seems with Dr Brown’s nipples and contingent co-regulated pacing with resting and elevated elongated sidelying we can minimize EER/LPR events as much as possible and avert need to thicken for reflux. At the same time, we are optimizing the swallow-breathe interface with the same interventions that also minimize/inhibit gulping (and air swallowing). Sim Spit up has a slightly increased viscosity which may help – but I’d try the more strict/frequent pacing. Yes, our neos are strict about VFSS understandably but this hx is worrisome and is the classic presentation that is a silent aspirator when objectified.  Studies show that L side down the for first hour post feeding then R side down the second hour post feeding is optimal based on gut anatomy –but RNs must balance head shaping and “changing position with cares” as well, so it cannot be a perfect world. This is a fabulous paper on reflux! Does not include newer meds due to publication date but is otherwise so wonderful!— Schurr, P., & Findlater, C. K. (2012). Neonatal mythbusters: evaluating the evidence for and against pharmacologic and nonpharmacologic management of gastroesophageal reflux. Neonatal Network31(4), 229-241.

 

Problem-Solving: 2 -year-old with Pierre Robin Syndrome

Question: I have a complex case I have been following since her time at our NICU. She is 2 years old, and I just completed a repeat VFSS (outpatient, her seventh to date) which continues to show significant dysphagia with high aspiration and airway obstruction risk.

History is significant for Pierre Robin sequence (chromosome deletions at 10q11 and 20p12) and cleft palate. She was born full term but required intubation after birth for critical airway. She required mandibular distraction hardware placement for about 2 months, has required supplemental nutrition by means of a feeding tube since birth (has had a PEG for over a year), and had a bilateral palatoplasty around 18 months of age. She’s been followed by feeding therapy, but despite recommendations for NPO because of her significant risks, she really wants to eat and drink. She pulls food off of the table from her siblings’ plates and has been eating/drinking regular/thin without specific correlation to any respiratory compromise.

I wish I could insert a fluoro clip here as I think it will be hard to spell this out… her swallow is significantly uncoordinated. With all consistencies the bolus reaches the pyriforms and at the onset of her “swallow” she presents with immediate regurgitation to the oral cavity and nasopharynx. No barium enters the esophagus with the initial swallow, it almost appears that the timing of UES relaxation follows tongue base retraction after the majority of contents have been redirected from the hypopharynx. This sequence is repeated 18 times prior to what appears to be a complete swallow, during which the contents that weren’t incidentally passed through the UES are cleared through the pharynx. Laryngeal penetration and aspiration at the height of the swallow occur during most of the uncoordinated “swallows” (which is silent). The pattern is the same with all consistencies and is paired with an open mouth posture. When provided with tactile cueing to keep her lips closed, she appears to have the coordination/pressures needed to clear small boluses with single swallows, but her adaptations seem to drive the motor planning train at this point. I ethically could not proceed with solid foods even though mom called two months ago to ask if we could ‘try solids no matter what’ during the study… they had hot dogs for dinner last night and brought pieces of hot dog to the study today. I have no explanation for how a choking event did not happen.

My heart really goes out to this family; they need a lot of guidance, and it has been a journey for them. Our oral maxillofacial surgeon and gastroenterologist have agreed to have a sit-down care conference with the family which I am extremely grateful for… my question is in regard to where to go from here. I think she needs intensive feeding therapy at this point and the clinics that I am most familiar with in our area are behaviorally driven to help kids wean from tube feedings… but she is a special case in which she really wants to eat and drink but is not safe to do so… does anyone have any specific cleft palate clinics that they have worked with? Mom is very open to this and was very open to me posting the patient’s case here to get some ideas for them.

Superior based pharyngeal flap surgery is in the discussion, but until her adaptive “swallows” are not the driving force of her function, I don’t see how she will safely manage as I think her ability to redirect boluses into her nasopharynx are her saving grace (as strange as that sounds).

Any and all help/insight is appreciated.

 

Answer: The infants and children we see never cease to amaze me with their adaptive behavior to accomplish a task in the face of such challenges. Bonnie Martin Harris always reinforces the dynamic nature of the swallow pathway, and how alterations in one point along that pathway can create compensatory behaviors along the pathway that may not always be beneficial but may indeed be maladaptive behaviors. What is the source of the alteration versus the compensatory behavior always needs to be sorted out, and you have done that so very well!

I have had three children with similar clinical and videofluoroscopic presentations. They each had mandibular hypoplasia, only one had the benefit of mandibular distraction. The impact of mandibular hypoplasia on motor learning in utero is not always fully appreciated. Early on, it creates muscular and structural restrictions to base of tongue integrity (decreased ROM, decreased posterior prolusion, decreased BOT retraction for the dynamic swallow). The fetus swallows amniotic fluid from 17 weeks on, and so by birth at term, has had 23 weeks of motor learning to help build the motor maps that underpin the swallow. Mandibular hypoplasia leads to reduced oral cavity space, this displaces the tongue body, leads to an altered tongue position, typically retracted back into the oral cavity and often with some form of glossoptosis; it also can create muscle shortening that will impede full ROM for the necessary base of tongue retraction. Even post MDO (Mandibular Distraction Osteogenesis), many infants continue to show reduced BOT retraction on VFSS for this reason. That’s why MDO is not a “fix all” for Pierre Robin sequence or mandibular hypoplasia, with its attendant alterations in muscular function, though MDO is unfortunately viewed as such by some medical professionals (i.e., “the infant was distracted, why doesn’t he eat?”). The co-occurring open mouth posture likely adversely affects an anterior seal on the bolus, which, along with reduced BOT retraction, acts as another impediment to generating the pressure required to relax and open the UES. The resistance to bolus flow causes the bolus to take the path of least resistance. The need to establish the more typical motor plan in order to habilitate (or maybe even rehabilitate at this point despite her young age) feeding skills is important.

Working on an anterior seal (lots of fun ways to do that at her age) and pressure generation with smaller boluses would be helpful. I wonder if a short repeat study to objectify optimal viscosity/viscosities for pressure generation might be informative, and like a short biofeedback for her, if she is cognitively appropriate, and perhaps to objectify the impact of optimal cueing strategies, utensils and neuromotor facilitation provided. Pharyngeal manometry to better objectify the role of pharyngeal pressure generation in bolus flow.

I so appreciate your critical thinking and living in the gray zone as I like to call it; where you are ok with pausing to think of possibilities, weighing them and asking more questions, instead of rushing to an answer. I’d be happy to work alongside each of you anytime.

Research: Down Syndrome and Swallowing Pathophysiology

I wanted to share a summation of recent research on prevalence of pathophysiology in infants with Down Syndrome that adds to our evidence-base. It suggests that a high index of suspicion is warranted, and watchful vigilance is required.

Narawane et al (2020) found high prevalence of both oral and pharyngeal dysphagia (89.8% and 72.4% respectively) in infants with Down Syndrome during VFSS. Laryngeal penetration was present in 52% and aspiration in 31.5%, often with thin liquids. When aspiration occurred, it was “silent” in 67.5%.

Jackson et al. (2019) looked at presence of deep laryngeal penetration and/or aspiration (on VFSS or FEES) in infants with Down Syndrome younger than 6 months versus those 6-12 months old. 31.9% of the younger group showed abnormal findings, compared to 51.3% in the older group. CHD and laryngomalacia were identified as risk factors for aspiration.

Stanley et al (2019) looked at 100 infants with Down Syndrome younger than six months via VFSS. 96% showed abnormal results. CHD was not a risk factor in this study, but co-occurring risk factors were desaturation during feeding, airway/respiratory anomalies, being underweight and prematurity.

Jackson et al. (2016) looked at older children with Down Syndrome (mean age of 2.1 years), and found oral motor difficulties in 63.8%, pharyngeal dysphagia in 56.3% and aspiration in 44.2%. Aspiration events were mostly “silent”.

 

References

Narawane, A., Eng, J., Rappazzo, C., Sfeir, J., King, K., Musso, M. F., & Ongkasuwan, J. (2020). Airway protection & patterns of dysphagia in infants with down syndrome: Videofluoroscopic swallow study findings & correlations. International journal of pediatric otorhinolaryngology132, 109908.

Jackson, A., Maybee, J., Wolter‐Warmerdam, K., DeBoer, E., & Hickey, F. (2019). Associations between age, respiratory comorbidities, and dysphagia in infants with down syndrome. Pediatric pulmonology54(11), 1853-1859.

Jackson, A., Maybee, J., Moran, M. K., Wolter-Warmerdam, K., & Hickey, F. (2016). Clinical characteristics of dysphagia in children with Down syndrome. Dysphagia31(5), 663-671.

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 A179(2), 177-182.