Download Complex Decision Making in Pediatric Dysphagia

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
Children’s Hospital Boston
ASHA November 17, 2006
Miami, Florida
Contact Information:






Kara Fletcher Larson, MS, CCC-SLP
[email protected]
Alana Lowry, MS, CCC-SLP
[email protected]
Jennifer Miller, MS, CCC-SLP
[email protected]
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
How did we get here?




Major pediatric medical center
Children’s Hospital Boston, MA
2005 performed 864 pediatric videofluoroscopic
swallow studies
Range in ages from 38 weeks PMA- young
adults with developmental disabilities (early 20’s)
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
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.
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
Chemotherapy Agent:
Vincristine




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
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
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”
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
Case Study:
Vincristine Toxicity

Insert VFSS # 1 of silent
aspiration with thin
liquids (2/3/05)
Lowry, Fletcher, Miller ASHA 2006
Case Study
Vincristine 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
Lowry, Fletcher, Miller ASHA 2006
Case Study:
Vincristine Toxicity

Insert VFSS of normal
swallow function with no
aspiration (4/21/05)
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
Outcome: Case Study
Vincristine 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
Lowry, Fletcher, Miller ASHA 2006
Complex Decision Making in
Pediatric Dysphagia Part 2
Type 1 Laryngeal Cleft
What is a Laryngeal Cleft (LC)?


Communication between the posterior larynx
and esophagus
Failure of tracheo-esophageal septum to develop
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
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.
Lowry, Fletcher, Miller ASHA 2006
Classification of Laryngeal Clefts





According to length
Type 1: interarynenoid only
Type 2: partial cricoid
Type 3: complete cricoid
Type 4: extending into trachea
Lowry, Fletcher, Miller ASHA 2006
Classification of Laryngeal Clefts
Benjamin and Inglis, 1989
Lowry, Fletcher, Miller ASHA 2006
Lowry, Fletcher, Miller ASHA 2006
Clinical Signs & Symptoms of
Type 1 Laryngeal Cleft







Noisy breathing
Inspiratory stridor
Coughing & choking with feedings
Chronic pulmonary infections
Aspiration
A’s and B’s with feedings
Cyanosis
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
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.
Lowry, Fletcher, Miller ASHA 2006
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)
Lowry, Fletcher, Miller ASHA 2006
Type 1 LC at Children’s Hospital
Boston



30 patients diagnosed with type 1 laryngeal cleft
from 2000-2005.
21 patients repaired.
>90% patients with improved swallow function
after repair.
Lowry, Fletcher, Miller ASHA 2006
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?
Lowry, Fletcher, Miller ASHA 2006
Associated Congenital Anomalies
with laryngeal cleft




Pallister-Hall Syndrome
G Syndrome
TEF
Esophaeal Atresia and Stenosis
Lowry, Fletcher, Miller ASHA 2006
Team Approach to Differential
Diagnosis






SLP (pediatric feeding & swallowing specialist)
Otolaryngologist (ENT)
Pulmonologist
Gastroenterologist
Radiologist
Developmental Pediatrician
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
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
Lowry, Fletcher, Miller ASHA 2006
Alternate treatments
for Type 1 LC




Identification and management of GERD
Thickened liquids
NG-tube or G-tube
These treatments may be implemented prior to
surgical repair
Lowry, Fletcher, Miller ASHA 2006
Surgical treatment of Type 1 LC



Historically, an invasive surgical procedure
Endoscopic procedure
Robotic Procedure at Children’s Hospital
Boston
Lowry, Fletcher, Miller ASHA 2006
Laryngeal Cleft
Endoscopic repair
Lowry, Fletcher, Miller ASHA 2006
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 210 months post surgery
Lowry, Fletcher, Miller ASHA 2006
Case Study
Laryngeal Cleft
16-month-old
development
Admitted
boy with normal growth and
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)
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
Lowry, Fletcher, Miller ASHA 2006
Case Study:
Laryngeal Cleft

INSERT VFSS HERE
of pt. aspirating with thin
and nectar-thick liquids
Lowry, Fletcher, Miller ASHA 2006
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 preoperative diet)

Repeat VFSS 4 ½ months s/p repair revealed no aspiration with
thin and nectar-thick liquids

Patient cleared for unrestricted oral diet
Lowry, Fletcher, Miller ASHA 2006
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.
Lowry, Fletcher, Miller ASHA 2006
Complex Decision Making in Pediatric Dysphagia
Lowry, Fletcher Larson & Miller, 11-17-06
References










Benjamin B, Inglis A. Minor congenital laryngeal clefts: diagnosis and classification. Ann Otol Rhinol Laryngol
1989;98:417-420.
Bermudez, M., Fuster, JL, Llinares, E., Galera, A, Gonzalez, C. Intraconazole-related increased vincristine
neurtoxicity: case report and review of literature, Journal of Pediatric Hematology & Oncology, 2005, July
27(7): 389-92.
Boseley, Mark et al., The utility of fiberoptic endoscopic evaluation of swallowing (FEES) in diagnosing and
treating children with Type 1 laryngeal clefts. International Journal of Pediatric Otorhinolaryngology (2006) 70,
339-343.
Chien, Wade et al., Type 1 laryngeal cleft: Establishing a functional diagnostic and management algorithm,
International Journal of Pediatric Otorhinolaryngology (2006). Article in press.
Cotton, R.T. & Prescott, C.A.J. 1998. Congenital anomalies of the larynx. In Cotton, R.T. & Myer, C.M. (eds).
Prescribed paediatric otolaryngology: 497-513. Philadelphia: Lippincott-Raven.
Jeng, MR, Feusner, J. Itraconazole-enhanced vincristine neurotoxicity in a child with acute lymphoblastic
leukemia. Pediatric Hematology & Oncology. 2001, March: 18 (2): 137-42.
Langmore, Susan. Evaluation of oropharyngeal dysphagia: which diagnostic tool is superior, Curr. Opin.
Otolaryngol. Head Neck Surg. 11 (2003) 485-489.
Parsons, D, Stivers, F, Giovaeto, D, Phillips, S. Type1 posterior laryngeal clefts, Laryngoscope 108, March
1998. 403-410.
Schulmeister, Lisa, RN, MN, CS, OCN. Preventing Vincristine Sulfate Medication Errors. Oncology Nursing
Forum, Volume 3, No. 5, E90-E98.
Watters, K, Russell, J. Diagnosis and management of type 1 laryngeal cleft, Int. J. Pediatric
Otorhinolaryngology. 67, June 2003. 591-596.
Lowry, Fletcher, Miller ASHA 2006