Published on September 13, 2007
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:: Contact Information: Kara Fletcher Larson, MS, CCC-SLP [email protected] Alana Lowry, MS, CCC-SLP [email protected] Jennifer Miller, MS, CCC-SLP [email protected] Incidence of Pediatric Dysphagia: 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 andamp; Perman 2000) Summarized in Oct. 2006 Brackett, Arvedson andamp; Manno in SID #13 newsletter How did we get here?: 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) Patient Demographics: 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 Trends in Referral Concerns: 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 Complex Decision Making: 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. Pediatric Oncology: 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 Chemotherapy Agent:Vincristine: 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 Side Effects of Vincristine: Neurotoxicity: 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 Results of VFSS in Children Receiving Vincristine: 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 Results of VFSS in Children Receiving Vincristine: 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 Management of Pharyngeal Dysphagia in Children with Vincristine Toxicity: 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 andamp; Dysphagia Team Aspiration with Thin Liquid Only: 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 Medical Concerns: 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 Medical-Ethical Considerations: 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 Resolution of Swallow Function: 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 Case Study Vincristine Toxicity: 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' Case Study Vincristine Toxicity: 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 Case Study:Vincristine Toxicity: Case Study: Vincristine Toxicity Insert VFSS # 1 of silent aspiration with thin liquids (2/3/05) Case StudyVincristine Toxicity: 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 Case Study:Vincristine Toxicity: Case Study: Vincristine Toxicity Insert VFSS of normal swallow function with no aspiration (4/21/05) Case Study Vincristine Toxicity: 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 . Outcome: Case StudyVincristine Toxicity: 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 Complex Decision Making in Pediatric Dysphagia Part 2: Complex Decision Making in Pediatric Dysphagia Part 2 Type 1 Laryngeal Cleft What is a Laryngeal Cleft (LC)?: What is a Laryngeal Cleft (LC)? Communication between the posterior larynx and esophagus Failure of tracheo-esophageal septum to develop Laryngeal Embryology: 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 Types of Laryngeal Clefts: 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. Classification of Laryngeal Clefts: Classification of Laryngeal Clefts According to length Type 1: interarynenoid only Type 2: partial cricoid Type 3: complete cricoid Type 4: extending into trachea Slide30: Classification of Laryngeal Clefts Benjamin and Inglis, 1989 Slide31: Clinical Signs & Symptoms of Type 1 Laryngeal Cleft: Clinical Signs andamp; Symptoms of Type 1 Laryngeal Cleft Noisy breathing Inspiratory stridor Coughing andamp; choking with feedings Chronic pulmonary infections Aspiration A’s and B’s with feedings Cyanosis Differential Diagnosis of Type 1 LC: 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 Suspicion of Type 1 LC: 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. Incidence of Laryngeal Clefts (all types): 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 andamp; Prescott, 1998) Type 1 LC at Children’s Hospital Boston: Type 1 LC at Children’s Hospital Boston 30 patients diagnosed with type 1 laryngeal cleft from 2000-2005. 21 patients repaired. andgt;90% patients with improved swallow function after repair. Incidence on the rise: 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 andamp; 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? Associated Congenital Anomalies with laryngeal cleft: Associated Congenital Anomalies with laryngeal cleft Pallister-Hall Syndrome G Syndrome TEF Esophaeal Atresia and Stenosis Team Approach to Differential Diagnosis : Team Approach to Differential Diagnosis SLP (pediatric feeding andamp; swallowing specialist) Otolaryngologist (ENT) Pulmonologist Gastroenterologist Radiologist Developmental Pediatrician Center for Aerodigestive Disorders (CADD): 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 Typical course of patient : 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 Alternate treatmentsfor Type 1 LC: 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 Surgical treatment of Type 1 LC: Surgical treatment of Type 1 LC Historically, an invasive surgical procedure Endoscopic procedure Robotic Procedure at Children’s Hospital Boston Laryngeal Cleft Endoscopic repair: Laryngeal Cleft Endoscopic repair Timeline from diagnosis to recovery: 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 Case Study Laryngeal Cleft: 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) Case Study Laryngeal Cleft: 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 Case Study:Laryngeal Cleft: Case Study: Laryngeal Cleft INSERT VFSS HERE of pt. aspirating with thin and nectar-thick liquids Case Study Laryngeal Cleft: 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 Summary: Vincristine Toxicity in Pediatric Pharyngeal Dysphagia: 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. Complex Decision Making in Pediatric DysphagiaLowry, Fletcher Larson & Miller, 11-17-06References: Complex Decision Making in Pediatric Dysphagia Lowry, Fletcher Larson andamp; 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 andamp; 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. andamp; Prescott, C.A.J. 1998. Congenital anomalies of the larynx. In Cotton, R.T. andamp; 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 andamp; 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.