Problem-Solving: Aerophagia in infant with cleft lip

QUESTION:

One of my friends just had a baby with a partial cleft lip. She is 6 weeks old and started having trouble with feeding last week. Her pediatrician feels like she is sucking too much air in when feeding. The mother has tried different bottles and nipples. She is on thin formula. She is also having diarrhea and her bottom is raw. Her pediatrician feels like it is caused from the air coming in from her cleft during bottle feeds. She has a cleft lip repair surgery date set in September. What recommendations should if give her? Applying chin support and pressure to cheeks to get a good lip seal? Thickening the formula to slow the flow rate?

Thank you for any advice!

ANSWER:

It is uncommon for a partial/incomplete cleft in isolation to lead to the feeding problems you describe. In my experience, an isolated cleft of the lip does not contribute to feeding problems in infants. It can, however, co-occur with other alterations in oral-motor/structural integrity. These would include: a cleft of the soft palate (even just the uvula) that is not readily apparent to the physician – it would alter the effectiveness of intra-oral suction and lead to ineffective compensatory sucking behaviors that may lead to air swallowing. Other possibilities are a tongue tie (posterior and/or anterior, as these often co-occur with clefts d/t timing of intrauterine aerodigestive structure formation; the tongue tie(s), if present, can lead to ineffective tongue-palate seal and again, air swallowing with each bolus, as well as fatigue and decreased transfer of milk. Another option is that there is co-occurring mandibular hypoplasia (a retruded and/or small jaw, as we used to call it – ENTs typically call it mandibular hypoplasia); this can lead again to poor tongue-palate seal, ineffective tongue patterns for sucking d/t altered intrauterine motor-learning by the tongue, and again compensatory sucking patterns that become maladaptive and not functional. Another possibility is a submucous cleft, which again may not be clear, and lead to ineffective suction, leading to the same maladaptations and adverse functional sequelae.

She may also have altered sucking or coordination patterns unrelated to any structural differences, just “co-occurring”, that has the same undesired results. For example, she may also have a prolonged sucking pattern that can lead to air swallowing. In that case, co-regulated pacing would support a more stable burst-pause pattern and avoid air swallowing,  or the flow are may be too fast, which can lead to swallowing air with each bolus, so a more controllable flow rate would help (i.e., slow flow). I would not thicken liquids or continue changing nipples, as her learning starts over each time, and some of that may be maladaptive. We really need is to understand the “whys” before we can determine what interventions would be indicated.

The loose stools also are concerning for poor formula tolerance or other GI issues that may further adversely affect feeding pleasure and growth. A consult with a pediatric SLP, ENT and GI would be beneficial to complete a thorough differential to find out what is contributing to her poor feeding.

Research Corner: Psychometric Properties of the Early Feeding Skills (EFS) Assessment Tool

Wanted to update you on a new manuscript about the EFS which I co-authored that is now in press with Advances in Neonatal Care.  Abstract below – Dr. Thoyre and I hope to see it published ahead of print in the next few weeks. We are very excited that the psychometrics of the EFS, demonstrating its reliability and validity, will soon be published. 

Psychometric Properties of the Early Feeding Skills (EFS) Assessment Tool Abstract

 Background: Supporting infants as they develop feeding skills is an essential component of neonatal and pediatric care. Selecting appropriate and supportive interventions begins with thorough assessment of the infant’s skills. The Early Feeding Skills (EFS) tool is a clinician-reported instrument developed to assess the emergence of early feeding skills and identify domains in need of intervention.

Purpose: The purpose of this study was to identify the factor structure of the EFS and test its psychometric properties, including internal consistency reliability and construct validity.

Methods: EFS-trained inter-professional clinicians in three settings scored 142 feeding observations of infants aged 33 to 50 weeks postmenstrual age. Redundant and rarely-endorsed items were removed. Factor-analysis methods clustered items into subscales. Construct validity was examined through the association of the EFS with (1) concurrently scored Infant-Driven Feeding Scale-Quality (IDFS-Q), (2) infant birth risk (gestational age), and (3) maturity (postmenstrual age).

Results: Principal components analysis with varimax rotation supported a 5-factor structure. The total EFS demonstrated good internal consistency reliability (Cronbach’s α = .81). The total EFS score had construct validity with the IDFS-Q (r = -.73; p < .01), and with gestational age of a subsample of premature infants (= .22; p < .05).

Implications for Practice: As a valid and reliable tool, the EFS can assist the inter-professional feeding team to organize feeding assessment and plan care.

Implications for Research: The strong psychometric properties of the EFS support its use in future research.

Please plan to join Dr. Suzanne Thoyre and I on August 15thand 16th for a Train-the-Trainer session on the EFS Tool in Atlanta, GA. Learn to use the EFS to effectively plan and provide an infant-guided approach to feeding. Simultaneously learn to train others back home to use the EFS to strengthen your unit’s feeding care. Review current research, the role of experience, dynamic systems theory, and feeding outcomes after NICU. Videotapes with enhanced audio of swallowing and breathing to learn key skill areas of the EFS: respiratory regulation, oral motor and swallowing function, physiologic stability, engagement, and change in coordination patterns of s-s-b as infants develop. Gain confidence scoring early feeding skills as not yet evident, emerging or established. Learn components of an infant-guided, co-regulated approach to feeding and contingent adaptations that make this approach so effective, using the EFS to plan individualized interventions. Receive teaching resources to take back to your unit to train others to use the EFS. As a group, we will network and navigate challenging issues and role-model a collaborative feeding practice. 

Bring yourself, your colleagues, or your whole feeding team! We are aiming for a multi-professional group, putting our heads together to improve feeding experiences for our most vulnerable infants. We hope to see you in Atlanta! 

 

 

 

 

Problem Solving: Infant with Laryngomalacia

QUESTION: I am providing swallowing therapy in a rural EI home.  The PT is a 7 month old male with a history of plagiocephaly, laryngomalacia and glottal surgery.    He demonstrated coughing with feedings and apneic episodes (awake and asleep), prior to surgery with no cyanotic episodes.  When I started treatment, he was 4 months old, post surgery and MBS.

The MBS reported:  Thin liquid-not enough swallows to be diagnostic due to limited latch.   Thickened 1Tbs to 2oz.-consistent, shallow swallows with small volume penetration, which eventually led to silent aspiration.  Suspected aspiration was due to large bolus size as a Level 3 MAM nipple was used.  Thickened 1 to 1 with level 3 nipple-WFL extraction and bolus size, timely swallow initiation, vallecular and pyriform residue observed but PT cleared.

He would not accept the 1-1 thickened feeds at home, so it was decreased 3 to 1 using oatmeal cereal.

The feeding clinic at nearest pediatric hospital recommended introducing plastic juice box and NUK brush to address his oral aversion to the bottle.  In therapy, I instead coached parents to focus on gentle bottle feedings, anticipating readiness to accept bottle and reading the baby’s cues during the feedings and taking breaks when needed, keeping feedings at 15-20 minutes.  This has proved successful.  He takes 3-4 oz at a feeding with 24 plus oz per day.   Mom has been able to return to work and the sitter can also confidently feed him the bottle. He is accepting small amounts of baby food and meltables.  The ENT recently tried to eliminate Zantac but he presented with increase emesis. He is back on the Zantac.  He continues to do best during feedings if he falls asleep with pacifier and then mom presents bottle.  He often reaches for the bottle, smiling and puts in his mouth, but intake is higher and pacing smoother with “sleepy feedings.”  Any thoughts on this????

My other question:  We have decreased thickening to 2.5 Tbs to 4 oz using a new Level 3 nipple.  With the info from the MBS would you decrease thickening further with use of slower flow nipples and how would you structure the decrease?

Thank you for advice in advance!!

 

ANSWER:    I suspect he had a supraglottoplasty which has been correlated in multiple papers in the literature with post-procedure continued impairment in swallowing physiology leading to silent aspiration. Typically, even post-op there continues to be a significant increase in WOB; because we do not know his birth history and early history, it is unclear if there were other co-morbidities whose sequelae may be part of his clinical presentation and therefore need to be a part of the differential.

His respiratory behaviors (both WOB and RR) are missing pieces of the puzzle for me, as these are common areas of challenge with his history. Because infants with LM often have increased WOB and intermittent tachypnea even post-op, I wonder what impact that had on his reluctance during the VFSS to suck (i.e. the need to breathe overrides sucking drive). This can lead to adaptive/compensatory behaviors of not wanting to latch, latching loosely and not transferring milk purposefully. In addition, the flow rate from the MAM, if it was competing with breathing, may have adversely affected the data set.

The information from the instrumental assessment is difficult to understand, because the terminology used in the report is not clear. The usefulness of the data gathered at that VFSS is limited, in that only a MAM nipple was used as they tend to flow faster, and infants S/P supraglottoplasty, if they do PO feed, benefit from optimal flow rate control without the need for thickening (such as a Dr. Browns’ Ultra Preemie or a preemie nipple). In my clinical experience with infants with LM post supraglottoplasty, thickened feedings can themselves create safety issues during a true feeding, related to residue and changes in structural integrity of the airway after surgery. Another consideration is that if we are altering the thickener or level of thickening based only on clinical observations is problematic, especially in the setting of his known silent aspiration. Also, we unfortunately know nothing about the etiology of the airway invasion reported nor if interventions were utilized during the procedure (such as resting and co-regulated pacing) which are crucial interventions for infants with his diagnosis post-op.

I would strongly suggest a repeat instrumental assessment of his physiology by a pediatric trained SLP. The last VFSS appears to have been 3+months ago, and the time from 4 months to 7 months of age, even for the normally- developing infant, is one of great change (structurally and with regards to physiology). It would allow a look at such as a Dr. Browns’ Ultra Preemie or a preemie nipple and use of resting and co-regulated pacing during the procedure. You could then also objectify physiology with the purees and meltables you have started since the last study.

Odd the ENT was planning to d/c the Zantac, as our ENTs typically max-out reflux meds post procedure. This is because it is common for LM and EERD to co-occur, and indeed EERD can cause/exacerbate airway edema and adversely affect laryngeal sensation, which in turn increases the likelihood that events of airway invasion will be silent.

The sleep feeding may indeed be r/t EERD and may also be an attempt to avoid the discomfort or pain associated with eating. Is a pediatric GI doc involved? If his apparent EERD is better managed perhaps the need to sleep fed may be less prominent and he can be guided to be more of an active participant via sensory-motor warm ups prior to feeding. It is likely the plagiocephaly and the torticollis are altering quality of postural/sensory-motor skills (head/neck/shoulder girdle/trunk) , despite perhaps milestones themselves reportedly “approximating” his age level; they may also be altering some of the qualitative aspects of swallowing and feeding, since the head/neck/shoulder girdle/trunk, and even the hips/pelvis, all contribute to form the foundation for feeding and swallowing At his age and with his postural/airway/likely breathing co-morbidities, we need him awake actively learning during feeding , both for better airway protection and for oral-motor learning prerequisite to future skills.

I hope this is helpful. He is quite a complex patient.

Catherine