Question:

I am presently working with an 8 month old who is s/p cleft palate repair, x 1 month. She is an ex 35 weeker with a trach and PEG tube. Her list of problems/diagnoses: Pierre Robin Sequence, Peter’s anomaly, ASD, Stankiewitcz-Isidor Syndrome, retrognathia, and micrognathia. Currently, she eagerly accepts the pacifier and bottle. We are using Dr. Brown’s level 1 with the valve but this flow seems a little fast and are trying the Newborn level. However, her tongue pulls back and therefore is not wrapping around the nipple for traction. She gets approximately 10-15ml in 30 min only due to compression. Any time that I attempt to put a gloved finger on top of her tongue to work on drawing it forward she gags. At best she reluctantly allows my gloved finger with formula just to the tip of her tongue. Any recs? Also, what resources/books do you recommend for infants/children with cleft palate?

Answer:

I think right now the cleft is the last important factor and is likely not contributing to what you are seeing with PO attempts. Her complex co-morbidities make a her a fragile feeder,  I would think, with high risk of silent airway invasion. The muscular and structural restrictions to the mandible, and therefore, to the tongue (glossoptosis) associated with the Pierre Robin and Peter’s Anomaly, likely lead to adaptive behaviors in an attempt to protect the airway. That then leads to the maladaptive behaviors you are seeing. My guess is her trach was placed  to establish an airway (not for long-term ventilation), so without a PMV (which she cannot wear due her airway obstruction), she also has no taste, no smell and has no subglottic pressure for airway protection. I would not PO with what I know, but rather, work on readiness non-nutritively right now. ENT would be very helpful  to guide your plan of care by assessing growth and readiness for decannulation in the future.