Problem-Solving with Catherine: 5-year-old with Athetoid Cerebral Palsy

QUESTION: I work in an outpatient pediatric rehab setting and recently saw a 5-year-old boy with athetoid cerebral palsy (CP) and frequent tonic reflex. His suck-swallow-breathing coordination is poor (multiple suckles followed by multiple swallows, then gasp for air), wheezes heavily, and often he has difficulty clearing his phlegm, has poor lingual range/control, uses phasic bite when foods presented. Parents have not had a VFSS done before.
 His parents “force” feed him with bread, which often stays in his tongue/sides of roof of his mouth. With thin liquids fed with a bottle, anterior spillage occurred (he can’t seal with his lips). Except for liquids (bottle-fed) he is often in distress during mealtimes. My other colleagues reported that he was also fed with rice. I tried educating parents and demonstrated to them how he handles puree and thickened milk much better, but they were against the recommendation, and stated that what I interpreted as distress (e.g., tensing/tightening his lips, head turn, increased tonic reflex, protesting (tensed vocalization) during mealtimes, and crying), is just him trying to eat. They claimed that whenever he attempts to do anything, that’s him tensing up, so they continued to ignore my advice and called my observation a ‘cookie cutter’ statement from textbook, which doesn’t apply to their boy. They said the child has never choked with bread all these years, why stopped him now?
I’ve observed him smiling and relaxed when he’s in comfort, and in my experience, if a diet is too difficult for a non-verbal child to manage, these are signs of distress.  He is quite intentional with his communication (smiles and looks at you if he wants an action/object), it’s hard for me to understand where his parents’ perspective.
I wanted to run by you to see if I might have misinterpreted this child’s body cues. What other strategies can you use to convince parents?
I referred them for a VFSS, so they can at least ‘view’ what’s happening when their child’s eating.

Our children with athetoid cerebral palsy typically present with complex swallowing/feeding challenges similar to those you describe and are very often fragile feeders. Their lack of postural stability and excessive postural mobility provide a poor base of support for the head/neck and oral motor/laryngeal/pharyngeal control along the entire swallow pathway. What you are seeing is indeed worrisome related to the swallow-breathe interface, likely caused by “problems down the line” as part of an overall unstable hyolaryngeal and esophageal complex. This affects not only chewing but the foundations for even swallowing saliva with integrity, and, as such, can often create the perfect storm for airway invasion, both silent and symptomatic. Safety of his liquid swallows is very likely adversely affected as well, especially given that the tongue plays a key role in liquid control and transport. The anterior spillage you describe has many aberrant components beyond lip control that likely do impact swallowing integrity and safety, as the entire system must work together in synergy. He likely lacks the stability and control for any solids, both purees (that may lead to stasis after the swallow) and the “pieces” that likely disperse and can be “underperceived” by a sensory system that is also likely adversely affected, given the overall neuromotor picture. The overt distress behaviors you have witnessed are a typical constellation with this diagnosis and its functional implications. His behaviors to some extent reflect what we call adaptive behavior, i.e., refusals and struggle that likely reflect his fear, learned discomfort and indeed possible airway invasion, that then lead to maladaptive feeding/swallowing behaviors. For families, this long-observed feeding behavior has perhaps become what they see as “just him” when he eats or drinks… much like an NICU nurse who at times may view the excessive WOB and tachypnea we witness at feedings with an infant with CLD as “just who the baby is”. But we know that, who that baby or child “is” is indeed worrisome. It reflects risk and is not then “ok”, when it comes to airway protection and indeed neuroprotection, for both children with complex co-morbidities, and for infants.

I suspect this little guy has learned to “struggle through” his parents’ well–intentioned feeding approach, sadly. They don’t understand his feeding behavior and its actual significance, which is his communication to them. And they are likely still grieving the loss of the normal child their hearts expected, so your thoughtful and likely quite accurate assessment of risk is something they may not want to hear, which is understandable in many ways. Our hearts go out to them.

Trust your instincts. The VFSS will be important to help them see the “why” behind his struggles. They are fortunate to have you in his corner.

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