Question: We are currently seeing a 4 year 11-month-old female born at 31 weeks gestational age with an undiagnosed neuromuscular disorder. She presents with global developmental delay, chronic oxygen requirement (<0.5L at 21%), trach dependent with suctioning requirement 1-2x per hour orally and within the trach. This patient can tolerate wearing a PMV for greater than 60 minutes but no distinguishable difference in swallow safety or timing during MBS. She has global gross motor delays, can sit independently but unable to crawl or transition from lying to sitting. She has severe cognitive delays but does communicate with use of head nodding/shaking and signs within routine based activities.
Patient’s parent’s primary goal is for the patient to eat orally. She has been receiving speech therapy services targeting dysphagia including use of NMES, thermal tactile stimulation, jaw stability (kinesiotape), and oral trials of tastes (flavor spray and hard munchables with strong flavor with clearing and oral care by SLP) only. We have previously trialed: Large bolus size to increase sensation, strong flavors, use of assisted jaw closure and focus on labial closure. Patient’s spontaneous swallows range from 1-8 swallows within a 60-minute session. She silently aspirates consistently on MBS on all consistencies. Per result of MBS patient lacks velar engagement or elevation and has bolus movement into the nasal cavity during attempted initiation of swallows. She is also unable to maintain a labial seal.
Please provide any treatment ideas we have not considered. We appreciate your time.
Answer: What a difficult patient. I am happy to provide informal guidance that may assist you in your ultimate management plan.
At almost 5, her multiple co-morbidities reflect the severity of her dysphagia. So many pieces. You are trying, I can tell, to pull together any possible way to help meet the goal set by her family, that she be an oral feeder. The problem is that the goal is most likely not attainable for right now, and perhaps may never be, due to the severe level of impairment both overall and specific to her dysphagia. Let me try to explain why this is my impression.
Her postural control, based on what we know, is a fundamental component for her poor swallowing integrity. The poor stability and strength we see in her body is likely mirrored in her oral-pharyngeal mechanism.
I don’t think her palate problem (“lack of velar engagement or elevation”) is a “palate problem” per se but, given her clinical presentation, and the bigger picture, what was inadvertently perceived as “lack of velar engagement or elevation” reflects the nature of her swallowing impairment. I suspect that the “bolus movement into the nasal cavity during attempted initiation of swallows” most likely reflects passive retrograde flow into the nasopharynx, due to a severe delay in swallow initiation. The bolus is moving passively into the nasopharynx, just like it moves passively into the airway and is aspirated. This may be due to a combination of poor sensation, poor driving force of the tongue and poor driving force on the bolus anteriorly (poor lip seal described). Likely that her pharyngeal constrictors are also lax and contribute to bolus mis-direction to both the nasal and laryngeal airways – all associated with hypotonia. Given her described MA (severe cognitive delays), she likely lacks not only the postural but also the cortical neural underpinnings to be able to chew the hard munchables described. Her need for suctioning suggests she does not swallow her saliva, which is often associated with such a severe delay in swallow initiation and sensory-motor deficits as described. She requires a trach but it’s not clear how long it has been in place. It is clearly providing a current means of pulmonary toileting (suctioning) but may have been placed secondary to past need for ventilator support given her undiagnosed neuromuscular disease; she wears a PMV but has significant suctioning needs; this suggests she is not perceiving her secretions despite restoration of oral airflow by the PMV and is not swallowing her saliva.
Given this, I would suggest not offering her hard munchables, based on the impairments in her swallowing physiology, as she appears to lack the many prerequisites for chewing, so the hard munchables may be silently aspirated and/or obstruct her airway. While use of a larger bolus for increased sensation may be somewhat helpful under a different clinical situation, it may be problematic for her, given her hypotonia; she is likely to not sense that large bolus, too, and there is risk for it to move with gravity silently. Her poor jaw stability and lack of lip closure are both likely secondary to poor oral stability and oral hypotonia, so passive attempts at closure/approximation to swallow would be just that and won’t impact the underlying neuromotor components needed to swallow.
PT/OT are most critical interventions currently to build the postural base/framework to support swallowing of saliva as a starting point. You might work in co-therapy with them, while they are working on postural stability and support; you could provide deep pressure tactile input to the base of the tongue, onto into the biting surfaces of the teeth, offer a biter/teether to provide deep pressure input into the mandible (via the biting surfaces), deep pressure into the blade of the tongue to promote active tongue movement and driving force. An NDT trained SLP could guide you through this approach. I would also focus on tolerance of tooth brushing to promote optimal oral hygiene. Use of good oral care before any oral intervention is essential to reduce potential for bacteria laden oral secretions move into her lungs during therapy activities. Flavor spray on the biter/teether might be permitted but given her ongoing needs for flow (21% Fi02), the spray can mix with saliva and then be aspirated as well; check with her pulmonologist for guidance with this, as well as any tastes being offered. Because she is a known silent aspirator with multiple co-morbidities, with likely immunocompromise, even therapeutic tastes may not be appropriate. I would be having a conversation with her primary physician (and/or a key specialist like pulmonology) to ask for support in discussing with the family that PO feeding in the short term is very likely to place her at risk for re-hospitalization. Helping them understand the neural (cortical), postural, and sensory prerequisites will be helpful. A goal for them would be for her to swallow her saliva, as that suggests that the prerequisites for PO feeding trials are in closer reach.
These are such hard conversations to have, as families of children with such all-encompassing co-morbidities want their children to eat and we understand that deep longing. We never want to take their hope away and must give them guarded optimism, but at the same time be realistic and specific about what is within our clinical expertise to change at given points in the child’s developmental trajectory. Never hesitate to tell the family that it is hard for you to tell them what you must, and that you are not giving up on their child but also must assure that everyone is on the same page about what will help now and focus on the “now”.
You are thoughtful and kind, and the family is fortunate to have you in their corner.
I hope this is helpful.