Complex Decision Making in Pediatric Dysphagia

1
Complex Decision Making in Pediatric Dysphagia

• Alana Lowry, MS, CCC-SLP
• Fletcher Allen Health Care
• Kara Fletcher Larson, MS, CCC-SLP
• Jennifer Miller, MS, CCC-SLP
• Childrens Hospital Boston
• ASHA November 17, 2006
• Miami, Florida

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Contact Information

• Kara Fletcher Larson, MS, CCC-SLP
• Kara.fletcher_at_childrens.harvard.edu
• Alana Lowry, MS, CCC-SLP
• Alana.lowry_at_vtmednet.org
• Jennifer Miller, MS, CCC-SLP
• Jennifer.miller2_at_childrens.harvard.edu

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Incidence of Pediatric Dysphagia

• 25 in all children
• 80 in children with developmental disabilities
• 3-10 of children exhibit severe feeding problems
• Occur with greater prevalence in children with
  physical disabilities, medical illness and
  prematurity
• (Manikam Perman 2000)
• Summarized in Oct. 2006 Brackett, Arvedson
  Manno in SID 13 newsletter

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How did we get here?

• Major pediatric medical center
• Childrens Hospital Boston, MA
• 2005 performed 864 pediatric videofluoroscopic
  swallow studies
• Range in ages from 38 weeks PMA- young adults
  with developmental disabilities (early 20s)

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Patient Demographics

• 50 of our patients fall in the age range of 6
  months- 3 years of age
• 6 of patients referred from Level 3 NICU
• 4 of patients referred by partnership with Dana
  Farber Cancer Institute/ Pediatric Oncology
  Division
• 13 of patients referred by the Otolaryngology
  Division

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Trends in Referral Concerns

• Given high volume of VFSS performed we began to
  observe trends in subset of patient populations
• Pediatric Oncology
• Increased incidence in identification and
  diagnosis of the Type 1 laryngeal cleft

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Complex Decision Making

• Low incidence problems in pediatric dysphagia
• High stakes for safe and effective management of
  oropharyngeal dysphagia
• Medical, surgical, ethical and clinical questions
  we face when treating these children
• Highlight the role of the SLP as the preferred
  provider of dysphagia services.

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Pediatric Oncology

• Patients referred for VFSS with chief complaint
  of coughing and choking with thin liquids
• All patients referred were undergoing
  chemotherapy consisting of the drug Vincristine
  (enrolled in specific treatment protocol for type
  of cancer)
• Onset of symptoms occurred 3-14 days during the
  treatment of a 6 week cycle

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Chemotherapy AgentVincristine

• Chemotherapy treats the type of cancer with
  medication that is toxic to tumor cells or kills
  them through interaction with receptors that
  indicate programmed cell death or prevent cell
  division.
• Typically given in cycles
• Cycle typically lasts 4-6 weeks
• Period drug administration- resting period

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Side Effects of Vincristine Neurotoxicity

• Involves peripheral, autonomic, and central
  neuropathy
• Primary and dose limiting toxicity of Vincristine
• Most side effects are dose related and reversible
• Neurotoxicity can persist for months after
  discontinuation of therapy
• Rare cases can be permanently disabling

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Results of VFSS in Children Receiving Vincristine

• All patients referred were full oral feeders at
  the time of referral
• All patients undergoing intravenous
  administration of Vincristine
• Parents report onset (often sudden) of
  sputtering, coughing and choking mainly with
  liquids
• Attending oncologist referred patient for VFSS

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Results of VFSS in Children Receiving Vincristine

• Silent aspiration with thin liquids
• Silent aspiration with thin and nectar thick
  liquids
• Silent aspiration with thin, nectar and honey
  thick liquids
• No evidence of aspiration with purees or solids

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Management of Pharyngeal Dysphagia in Children
with Vincristine Toxicity

• Results reported back to Oncology Team
• Based on the extent of aspiration modifications
  to the oral feeding regimen were initiated
• In cases of aspiration with all liquid
  consistencies discussion regarding non-oral
  supplementation took place with the MD
  Dysphagia Team

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Aspiration with Thin Liquid Only

• Diet of nectar thick liquids
• Recommend referral to nutrition to ensure
  adequate hydration and child acceptance
• Report results to Oncology Clinic
• Medical team to discuss changes to dose/strength
  of Vincristine
• Develop plan for repeat VFSS once team feels
  neurotoxicity is resolving
• Parents also report improved clinical status
  which helps guide timeline for reassessment of
  swallow function

15
Medical Concerns

• Larger medical concern whether to discontinue
  cycle of Vincristine to avoid further
  exacerbation of the toxicity vs. decreasing the
  dose/strength of the Vincristine.
• Child may be made NPO with continuation of
  chemotherapy with dose changes.
• Child put on rest from a swallowing standpoint
  with period of going off the drug
• Above decision made by attending oncologist with
  input from the Oncology-Dysphagia team

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Medical-Ethical Considerations

• Decision to withhold chemotherapy treatment to
  allow neurotoxicity to improve
• Parental stressors regarding decision
• Patients taken off Vincristine for weeks while
  swallow function improves
• Child continues to orally feed with modifications
  in place

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Resolution of Swallow Function

• Swallow function resolved (returned to
  pre-Vincristine status) in 100 of patients.
• Range of time it took for swallow function to
  return to normal
• Normal defined as back to full oral diet of thin
  liquids, purees and solids
• of VFSS patients underwent until swallow
  function resolved. (at what time intervals).
• Recurrence once patient resumed Vincristine
  treatment
• Yes in some patients
• Even at reduced strength of drug (50 strength).
• Oncology team was very conservative with
  re-starting chemotherapy/ altered doses and child
  monitored closely

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Case Study Vincristine Toxicity

• 5/10/04 3 ½ year old girl is diagnosed with
  
• acute lymphoblastic leukemia (ALL)
• Immediately begins chemotherapy (including
  vincristine)
• Throughout 7 months of chemotherapy, pt.
  is seen frequently in clinic for chronic upper
  respiratory tract congestion and persistent
  coughing

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Case Study Vincristine Toxicity

• 12/27/04 Diagnosed with pneumonia on
  chest x-ray
• 2/3/05 Pt. referred for initial VFSS by
  oncology team 9 months into chemotherapy
  treatments
• VFSS revealed silent aspiration with thin
  liquids
• Patient safe to continue to receive
  nectar-thick liquids, purees, and chewable
  solids

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Case StudyVincristine Toxicity

• Insert VFSS 1 of silent aspiration with thin
  liquids (2/3/05)

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Case StudyVincristine Toxicity

• 2/4/05 Vincristine component of chemotherapy
  is withheld
• Pt. remained on nectar-thick liquids, purees,
  solids
• 2/28/05 Repeat VFSS continued to reveal silent
  aspiration
• with thin liquids
• Recommendation remain on altered oral diet
• 4/21/05 Repeat VFSS revealed normal swallow
  function with no documentation of aspiration
  with thin liquids
• Respiratory status stable

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Case StudyVincristine Toxicity

• Insert VFSS of normal swallow function with no
  aspiration (4/21/05)

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Case Study Vincristine Toxicity

• 4/28/05 Vincristine resumed (50 strength)
• (Pt. maintained nectar-thick liquid diet)
• 6/20/05 2 mo. follow-up VFSS revealed silent
  aspiration with thin liquids
• Recommendation Cont. nectar-thick liquids
• Pt. continues receiving vincristine
• Pt. was asymptomatic from respiratory
  standpoint during this time
• .

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Outcome Case StudyVincristine Toxicity

• 10/1/05 Patient completed course of chemotherapy
  
• (No longer receiving vincristine)
• 11/3/05 Repeat VFSS was normal with no further
  evidence of aspiration with thin liquids
• Pt. cleared for full oral diet
• Follow-up Patient tolerated re-introduction of
  thin liquids and maintained stable respiratory
  status

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Complex Decision Making in Pediatric Dysphagia
Part 2

• Type 1 Laryngeal Cleft

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What is a Laryngeal Cleft (LC)?

• Communication between the posterior larynx and
  esophagus
• Failure of tracheo-esophageal septum to develop

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Laryngeal Embryology

• Trachea and esophagus share common lumen during
  embryogenesis
• 35th day of gestation
• Laryngeal cleft is the failure of the
  interarytenoid tissue or cricoid tissue to fuse
  in the posterior midline

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Types of Laryngeal Clefts

• Four classifications of laryngeal clefts
• Type 3 and 4 diagnosed on first day of life due
  to severity
• Type 1 and 2 diagnosis may take months to years.
• Type 1 is the focus of our talk today.

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Classification of Laryngeal Clefts

• According to length
• Type 1 interarynenoid only
• Type 2 partial cricoid
• Type 3 complete cricoid
• Type 4 extending into trachea

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Classification of Laryngeal Clefts
Benjamin and Inglis, 1989
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(No Transcript)
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Clinical Signs Symptoms of Type 1 Laryngeal
Cleft

• Noisy breathing
• Inspiratory stridor
• Coughing choking with feedings
• Chronic pulmonary infections
• Aspiration
• As and Bs with feedings
• Cyanosis

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Differential Diagnosis of Type 1 LC

• VFSS (MBS)
• FEES
• Chest x-ray
• Referral to pediatric Otolaryngologist and
  Pulmonologist
• High degree of suspicion of type 1 laryngeal
  cleft (LC)

• Direct laryngoscopy is needed for definitive
  diagnosis and is the gold standard for diagnosis

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Suspicion of Type 1 LC

• Child presents with normal development with
  exception of isolated swallowing dysfunction
• No evidence of neurogenic, medical, and genetic
  etiology for swallow dysfunction.

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Incidence of Laryngeal Clefts (all types)

• Rare, less than 0.1
• Incidence increases to 0.6 in patients with the
  co-existence of TEF and laryngeal cleft
• Strong association with other anomalies, but in
  our population has often existed in isolation
• (Cotton Prescott, 1998)

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Type 1 LC at Childrens Hospital Boston

• 30 patients diagnosed with type 1 laryngeal cleft
  from 2000-2005.
• 21 patients repaired.
• gt90 patients with improved swallow function
  after repair.

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Incidence on the rise

• Literature review documents incidence of type 1
  laryngeal cleft higher than in the past.
• 7.6 (Chien et al, 2006)
• 6.2 (Watters Russell, 2003)
• 7.1 (Parsons et al, 1998)
• Are there now more patients with type 1 laryngeal
  cleft or are we getting better at the diagnosis?

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Associated Congenital Anomalies with laryngeal
cleft

• Pallister-Hall Syndrome
• G Syndrome
• TEF
• Esophaeal Atresia and Stenosis

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Team Approach to Differential Diagnosis

• SLP (pediatric feeding swallowing specialist)
• Otolaryngologist (ENT)
• Pulmonologist
• Gastroenterologist
• Radiologist
• Developmental Pediatrician

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Center for Aerodigestive Disorders (CADD)

• Monthly meeting to review complex cases and
  collaborate on differential diagnosis
• Multidisciplinary team approach to diagnosis and
  treatment for aerodigestive cases
• CADD clinic meets 1x per month
• Patients see GI, ORL, Pulmonary and VFSS on same
  day

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Typical course of patient

• VFSS documentation of aspiration of thin liquids
• Unable to visualize laryngeal cleft on
  fluoroscopy
• Patient placed on treatment of thickened liquids
• PCP referral to Otolaryngologist for further
  assessment

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Alternate treatmentsfor Type 1 LC

• Identification and management of GERD
• Thickened liquids
• NG-tube or G-tube
• These treatments may be implemented prior to
  surgical repair

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Surgical treatment of Type 1 LC

• Historically, an invasive surgical procedure
• Endoscopic procedure
• Robotic Procedure at Childrens Hospital Boston

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Laryngeal Cleft Endoscopic repair
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Timeline from diagnosis to recovery

• VFSS
• ORL consult
• Direct laryngoscopy
• Maintenance diet
• Repair
• Repeat VFSS 6-8 weeks after repair
• Full recovery not documented on VFSS until 2-10
  months post surgery

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Case Study Laryngeal Cleft

• 16-month-old boy with normal growth and
  development
• Admitted to CHB for -respiratory distress
• -fever of 102
• -perioral cyanosis
• -mother reports history of 6 episodes of
  pneumonia in the past 5 months (all LLL)

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Case Study Laryngeal Cleft

• Videofluoroscopic swallow study performed during
  admission
• Revealed
• silent aspiration with thin liquids
• silent aspiration with nectar-thick liquids
• Safe to consume honey-thick liquids, purees and
  chewable solids orally
• Recommended nutrition consult to assess hydration
  needs on honey-thick liquids

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Case StudyLaryngeal Cleft

• INSERT VFSS HERE of pt. aspirating with thin and
  nectar-thick liquids

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Case Study Laryngeal Cleft

• PCP referral to Otolaryngology (ORL)
• Direct laryngoscopy and bronchoscopy performed
• Type I laryngeal cleft diagnosed.
• 1 month later endoscopic repair of Type I
  laryngeal cleft by ORL
• Sent home after surgery on honey-thick liquids
  (same pre-operative diet)
• Repeat VFSS 4 ½ months s/p repair revealed no
  aspiration with thin and nectar-thick liquids
• Patient cleared for unrestricted oral diet

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Summary Vincristine Toxicity in Pediatric
Pharyngeal Dysphagia

• Low incidence problem but with significant
  consequences for pulmonary health, swallow
  function and treatment decisions.
• Increased awareness to respiratory symptoms in
  pediatric patients undergoing chemotherapy
  treatment.
• Decreased referral time.
• Highlights the importance of the role of the SLP
  on the dysphagia-oncology team.

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Complex Decision Making in Pediatric
DysphagiaLowry, Fletcher Larson Miller,
11-17-06References

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