Problem-Solving: 3 y/o with Polymicrogyria

Question:

I have a family with 3yr old son dx- polymicrogyria. During current hospitalization, treatment team is recommending NG tube and NPO. He will be sent home with this in place at least 4-8 weeks. We have been working on feeding/swallowing for several months now. My question is, how detrimental to current feeding and swallowing goals is this long term NG tune placement? Should the family receive more counseling regarding PEG prior to returning home (they are in a city, 12 hours from our rural location). I don’t want NG to result in increased risk of aspiration of reflux or hinder oral feeding goals. Is PEG better option at this point? Help please.

Answer:

You are asking excellent questions. Trust your instincts. As you know, this is a complex neurologic diagnosis that has enduring adverse effects on all developmental domains, especially feeding/swallowing. One of the mantras I offer all my medical and nursing colleagues and my students, is “co-morbidities matter”. My experience suggests that this is not the perspective that most medical colleges utilize as the framework for their differentials related to ability to feed orally, both for adequate intake and or airway protection. One set of clinical data/findings about dysphagia for an infant nor child will/should lead us to have very different plan of care when the co-morbidities and history are different. There is no pigeon hole or cookbook we can use but rather it requires a complex critical analysis of the data about that patient in the context of his unique history and co-morbidities that must guide our clinical decision-making, It can then become frustrating for those of us who recognize this close relationship between co-morbidities and prognostication, both through research as well as our clinical experience, when options are offered and decisions are made that don’t assure such a differential. While their intentions are clearly good ones, sometimes there is a tendency in these situations for the medical team to want the parents to be happy (i.e., a wait and see attitude), or to not understand the complexity of the underpinnings lacking, or to not appreciate the complex skills required to feed safely and meet nutritional requirements.

I don’t know if there are other co-morbidities, but if so, those will also act as rate limiters for feeding. This diagnosis, and the nature of its pathophysiology, is the lense through which we as SLPs then look at skill progression and, therefore feeding ability and safety. It is highly unlikely his related feeding/swallowing challenges will resolve in the short term (i.e., 4-8 weeks) as their apparent proposed “short term” NGT plan suggests. The NGT may indeed exacerbate EER/GER and create the setting for onset of feeding aversions due to the negative effects of an indwelling tube that makes the oral facial area unpleasant, especially if they decide to do a bridled NGT which may their plan given that at age three, there is also great risk to dislodge the NGT, which in itself creates risk for airway invasion. At 3 years of age, the likely maladaptive motor maps already developed due to likely struggles to feed will create further stress and roadblocks for this child.

I recommend the team strongly consider a PEG forth above reasons. Look at this recent publication that looks at NG instead of a PEG.

Keep up the good work. The child and his parents are lucky to have you in their corner.

I hope this is helpful.

 

A retrospective review of enteral nutrition support practices at a tertiary pediatric hospital: A comparison of prolonged nasogastric and gastrostomy tube feeding

Ricciuto, Amanda et al.     Clinical Nutrition , Volume 34 , Issue 4 , 652 – 658

They compared prolonged nasogastric and gastrostomy tube use, hypothesizing that earlier gastrostomy improves outcomes. Their results showed: Among 166 patients, the median total tube feeding duration was 24.9 (3.0–75.6) months and varied with underlying disease and swallowing assessment. The median duration of nasogastric tube use was 7.8 (0.7–45.3) months. Food refusal was significantly associated with nasogastric tube exposure >3 months (RR 3.3, p < 0.001, NNT = 3) and anthropometric outcomes were superior in gastrostomy-fed patients. Rates of aspiration pneumonia were similar in both groups. Despite more initial opposition to gastrostomy and a higher complication rate, gastrostomy users appeared more satisfied with their experience, as demonstrated by a much lower discontinuation rate than observed in the nasogastric group. They concluded prolonged nasogastric feeding is associated with increased food refusal and less favorable anthropometric outcomes and earlier gastrostomy placement may be preferable.

Problem-Solving: Struggles transitioning from breast feeding to bottle feeding

Question:

I am seeking out any clinical information (research articles, case studies, hospital/center practices or pathways) for treating infants who are struggling to transition from breast feeding to bottle feeding. My center sees many patients, who are often typically-developing and without a significant medical history,  who are excellent breast feeders but are refusing or having difficulty with bottle feeding. Often,  these mothers are under a lot of pressure,  as they are returning to work and worry that their child will be unable to feed while they are apart. Thanks!

Answer:

In this group of breastfeeding infants who don’t transition to bottle-feeding readily, we may see a wide variety of etiologies even in the subset of otherwise apparently normally developing infants.

I always try to keep my clinical radar open to the less obvious and go from there. Because of the protective controllable flow from the breast (compared to that received from a rubber manmade nipple), the faster (and therefore less controllable) flow rate when offered a bottle may reveal or create true incoordination. Caregivers may select a faster flowing nipple for bottle-feeding without knowing that it often makes bottle feeding harder for  a breastfeeding infant. Perhaps subtle airway differences that may be “manageable” for the infant at breast then make bottle feeding stressful. Perhaps not using the rooting response with the bottle (like mother does at breast) may make organization of the motor maps that underlie the root-to-latch sequence to initiate bottle-feeding difficult. Well-intentioned but ill-advised use of prodding with bottle-feeding  or “spinning the bottle” with bottle-feeding doesn’t happen at the breast; that can override and confuse motor sequencing that creates stop-start patterns and disorganization with the bottle. Caregivers during bottle-feeding may steer the infant back to sucking when he stops to breathe (to accommodate the less manageable flow); this can create physiologic stress when the swallow-breathe interface is then not synchronous.

Taking the infant’s perspective always helps me figure out the “whys” as a starting point. Each infant has his own  “story”, history and parent-infant relationship that makes his clinical presentation unique.  While breastfeeding infants who don’t transition to bottle-feeding readily can be often grouped as a “population”, our thoughtful reflective peeling apart of the layers for each infant is essential. This usually leads me to ask, “what else” (as my friend and mentor Joan Arvedson always says) and keeps my thinking in the “grey zone” where there are more questions than answers and that is a good thing.

I hope this is helpful

Research Corner: Tracheobronchomalacia and CLD in the NICU

Should Neonatologists Rule Out Tracheobronchomalacia in Every Premature Baby With Bronchopulmonary Dysplasia?      Fadous Khalife et al,    J Med Cases. 2019;10(3):72-7   

doi: https://doi.org/10.14740/jmc3259

Commentary from Catherine: This current look at one of the possible adverse effects of prolonged intubation should inform our practice when supporting infants with CLD. Poor coordination and adverse events with PO that may lead to symptomatic or silent airway invasion may have their etiology in TBM. Keep this in your differential as you work with NICU infants who have CLD. Also our kids in PICU who present with persistent airway invasion in the setting of chronic respiratory co-morbidities. A direct laryngoscopy/bronchoscopy by ENT may be something to suggest as you work with your medical team. 

Excerpts:

Bronchopulmonary dysplasia (BPD) is a chronic inflammatory lung disease that affects mainly premature infants; it results from the damage to the immature lungs from mechanical ventilation and prolonged use of oxygen. They suffer from obstructive lung disease.

TBM in children is defined as weakening of the airway wall due to softening of the cartilaginous rings, decreased tone of the airway smooth muscle and collapse. This results in increased airway compliance and reduction of the size of the airway lumen during expiration. The clinical manifestations of malacia vary widely: barking cough, impaired mucous clearance, retractions, dyspnea and prolonged expiratory phase. Children can also have atelectasis and recurrent pneumonia leading sometimes to intubation and difficulty weaning from ventilator support. It may be associated with feeding difficulties.

The acquired type is most commonly associated with prolonged mechanical endotracheal intubation (with more significant effect in premature infants), severe tracheobronchitis and external tracheal compression (double aortic arch, innominate artery compression, vascular rings, left atrial enlargement).

 

 

Problem-Solving: Feeding Aversions and Noonan’s in NICU

Question:

Do you have any suggestions for working with a NICU baby with Moons Rasopathies and food aversion?

Answer:

Do you mean Noonan’s Rasopathies? You don’t mention much about the infant so lots of pieces that are unclearing terms of problem-solving. Not sure if he is in the NICU currently or was in the NICU in the past. But here are some thoughts.

I always look at the system problems that are contributing, i.e. motor, oral-motor, sensory, oral-sensory then focus on those systems and their breakdown. What are the implications of that diagnosis for the underpinnings for feeding safely and well enough to grow. Aversions in an NICU infant and even in an older child are likely a byproduct of the systems that are altered or impaired.

Typically, with Noonan’s, it is hypotonia and altered craniofacial integrity (structural and muscular), plus the adverse effects of the need for NICU care (? Prematurity? Sick newborn? Need for intubation? Respiratory history? Current need for respiratory support? State regulation? Other complex co-existing co-morbidities – such as cardiac, neuro, GI, airway etc.? )

Be sure reflexes are consistent enough to even try pacifier dips. May look aversive due to infant is trying to protect himself (due to respiratory or swallowing safety issues). Lack of engagement maybe an adaptive behavior, a purposeful attempt to avoid feeding. or it may be lack of skill combined with disengagement. Take time to reflect and sort out “why” infant appears  “averse”. Patience and caution will be critical. Normalizing the oral-sensory system, in the context of the reasons for the apparent  “aversions” (or lack of engagement with feeding) will be a needed part of your problem-solving intervention.   If infant has the prequisites and is engaging, and you decide it appears safe to assess small PO trial, I suggest offering slow flow nipple , sidelying, resting , strict co-regulated pacing and use watchful vigilance. An instrumental assessment early on will be most important to objectify physiology due to high risk for airway invasion related to co-morbidities. Part of your role will likely be to protect the infant from being pushed to PO feed by well-intentioned caregivers.

Hope this helps.

 

Problem-Solving: Twelve-Month-old with apparent tongue tie and poor chewing

Question:

I have a 12-month-old that just came in for an evaluation. Lactation consultant at birth reported tie. Mother reported pain while breastfeeding and inadequate suction. She decided not to have the tie cut. Fast forward to 12 months. They just started to introduce chewables about 1 month ago. I see lingual lateralization with emerging munching–it is inconsistent. He does not drink from a straw. He drinks water and whole milk from a spout. He coughs inconsistently when he drinks and it appears, he is not sipping then swallowing but holding liquid in his mouth—-does anyone have experience with tongue tie impacting the peristalsis movement of the tongue for drinking? Mother reported that during his time drinking from a bottle he did not ever cough.

I got a look at his tongue–it appears to be a posterior tie; however, it is hard for me to tell if this is truly what is impacting him. If peristalsis is impacted could that mean decreased laryngeal elevation? Besides recommendation to a dentist and ENT should I recommend a MBS?

Any input would be greatly appreciated!

 

Answer:

Tongue tie is indeed a controversial topic, not only for SLPs but also for our large ENT group, who do not even agree amongst themselves.

I was fortunate as a young pediatric SLP to take an 8-week NDT course (Neuro Developmental Treatment) that was primarily for OTs and Pts and focused on the kinesiology, motor-control and dynamic interrelated components of movement that produce the gross motor, fine motor and oral-motor synergies that underlie functional skills. It also taught us about compensatory motor patterns that can be created by muscular/structural restrictions to movement. It opened a while new world to me about the motor mapping and structural-muscular relationships that are the underpinnings for swallowing and feeding. That perspective has so enhanced my work in dysphagia over many years, and continues to do so, both related to theory, components of movement required, evolution of feeding/swallowing and looking at treatment of feeding/swallowing from a sensory-motor learning perspective.

My clinical experience across the pediatric age groups from neonates through teenagers strongly suggests that truly-tethered oral tissues can change those synergies and create the need for maladaptive/compensatory behaviors with feeding, adversely affecting breastfeeding, bottle-feeding, spoon-feeding, mastication of solids, cup-drinking, and straw drinking.

Amber, I wonder if the 12-month-old you described, who it sounds like was otherwise normally developing, may be having trouble with using the tongue tip and lateral borders of the tongue to move food to the molar ridge and keep it there for chewing and to manage liquid.

Depending on the location of a posterior tongue tie, it may create difficulty accessing the lateral borders of the tongue to assist with bolus control and transport. This can lead to compensations of humping of the back of the tongue or restricted tongue retraction that can adversely affect the swallow. Contraction of the lateral borders of the tongue is required for tongue retraction which is part of the swallow pathway that, yes, as you suggested, could indeed adversely affect pharyngeal peristalsis. There can be pooling of liquid on the surface of the tongue (flattened instead of having a central groove due to restricted “lift of the blade by tethering), creating the need to hold the bolus to avert spilling across the tongue blade into the lateral sulci. If there is a true posterior tongue tie, the muscle coordination with spoon feeding is likely also being affected, though may not be as readily obvious if he can “eat from a spoon.” Compensations during eating can result, including, for example, avoiding/refusing solids, multiple swallows to clear a bolus, ineffective suction with a straw, hard swallows to facilitate bolus transport, overstuffing to ”move food”, swallowing foods not fully masticated, or pocketing due to the inability to transport a bolus properly. Progression to more difficult solids such as meat will be challenging due to lack of disassociation of tongue and jaw movements because a munching pattern is all that is available.

A swallow study, interpreted thoughtfully specific to components of movement along the swallow pathway, can objectify the compensations adversely affecting function. I recently saw an otherwise normally developing 5-year-old referred for a swallow study due to “not chewing”. She had altered articulation (suspicious for tethered oral tissues based on its presentation), refusal of solids, poor cup and straw drinking. Her clinical examination, own history provided through my gentle questions and obvious restrictions in oral motor movements suggested upper lip, anterior tongue and posterior tongue ties. The subsequent instrumental assessment as requested by the MD suggested restrictions in movement that created many of the differences I described above. She was seen by a local pediatric dentist well known of specializing in this area of pediatric practice and had multiple releases. Much relearning was required with her community SLP to support learning of new oral-motor maps and synergies for functional skills. Just one example from many. That does not suggest that all such feeding problems use as examples above are due to ties, but we need to thoughtfully complete a through differential with each infant or child or adolescent, and continue asking questions both during sessions and after, with each other.

My Pediatric PTs colleagues love talking about tethered oral tissues, since they bring such clear understanding of motor synergies and structural-muscular relationships that adversely affect function. Sometimes they say it can affect function quite obviously and at other times, in a more subtle way that alters quality of movement, and affects motor learning going forward, affecting future skills. Might this be applicable to eating and drinking, as it evolves and progresses through motor learning and experience? My thought is yes.

I hope this is helpful.