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Behavioral Medicine by gjjur4356

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									Paradoxical Vocal Fold Motion
      and Habit Cough
         Christopher J. Hartnick MD MS EPI
     Associate Professor, Dept. of Otology and
                     Laryngology
    Director, Pediatric Airway, Voice, Swallowing
                        Center
        Massachusetts Eye and Ear Infirmary
               Harvard Medical School
                      Boston, MA
      Christopher_hartnick@meei.harvard.edu
                         A case
• 16 year old female
    basketball player
•   Persistent difficulty
    breathing during
    competition
•   Treated for asthma with
    no change in sx’s
•   Negative w/u for organic
    causes?
•   Affecting her ability to
    compete
      A case

   • What is this?
     • Is it real?
• Is it all in her head?
                 What is it?
• Historically referred to by many names:
  • Munchausen’s stridor
  • Vocal cord dysfunction
  • Irritable larynx syndrome
  • Laryngeal hypersensitivity
  • Malingering…
                     What is it?
 • A spectrum of disorders with similar manifestations

    VFs   adduct rather than abduct with inspiration

• Why? Multiple theories/
  explanations…
  • Laryngeal irritants
    producing a form of
    laryngospasm
  • Psychological conditions
    (anxiety)
  • Laryngeal dystonia
         General Characteristics
• Most commonly seen in ages 10-40
   • Children as young as 4 months have been diagnosed
   • More commonly seen in females
• Episodic stridor with inhalation
   • Typical noisy breathing
   • Typical characteristic “stressor” (performance based, theater,
     exercise, etc): MOSTLY A HISTORICAL DIAGNOSIS
   • PVFM stops when no more “stressor” and then starts again
     when “stressor” returns
• Description of throat tightness
            Stepping back a bit..
          • Influence of the larynx on breathing


“The Gateway to the Lungs” and “Protector of the airway”



                         Swallow
                          Cough
                       Throat clear
                         Sneeze
                      Laryngospasm
Before we get too far…




• The larynx provides the balance
    Laryngeal protective response
• Laryngeal protective response (laryngeal
  adductive reflex)
  • firm adduction of the vocal folds
  • regardless of the underlying etiology
• Create large subglottic pressure gradient
  • expel any real, perceived, or potential irritant from
    compromising the airway below the level of the glottis
 Laryngeal protective reflex




• Complex interaction between the brain and larynx
      • Compromise can occur at any point
• Central - neurologic(dystonia) psychiatric (anxiety)
        • Laryngeal hyper/hyposensitivity
               • Abnormal signaling
    Factors associated with abnormal
           laryngeal response

• Physical Illness (URI,   • Hypersensitivity to
    Flu, etc.)               fumes/irritants
•   Laryngopharyngeal      • General Fatigue
    Reflux                   Emotional Stress
•   Relative dehydration   • Psychological Stress
•   Asthma                 • Anxiety
•   Allergies
   When does it become a problem?
• Downward spiral initiated by overexuberant
 adductive protective responses
  • Vocal fold edema and erythema
  • Hoarseness
  • Anxiety associated with fear of not being able to breathe
    • Can be task specific
  • Cough - we’ll come back to that
• Symptoms progress and become self-reinforcing
• A spectrum of symptoms and manifestations that
 can be very individualized
                    Back to PVFM
PVFM involves episodic, inappropriate adduction
 of the vocal folds during the inspiratory phase of
 the respiratory cycle resulting in intermittent
 (usually partial) glottic obstruction.

(Divi, V.; Hawkshaw, M.J; Sataloff, R.T.)
                   Types of PVFM

• Classification
 scheme devised by
 Maschka et al
  • Attempt to relate sx’s
    to various organic &
    inorganic causes



                             Maschka D et al. Laryngoscope. 1997;107:1429-1435.
    Diagnoses associated with PVFM
• Neurologic
   • Respiratory dystonias (similar to spasmodic dysphonia)
   • Generally chronic and consistent inspiratory adduction
• Psychogenic
• Increased laryngeal irritability
   • Allergy, reflux, chemotriggers
• Supraglottic collapse and vocal fold hypomobility
   • Bernoulli effect secondary to elevated inspiratory pressures

   • THINK GLOBALLY AS PVFM IS A DIAGNOSIS OF EXCLUSION
                      Evaluation
• History
  • “refractory asthma”
  • repeated ER visits for dyspnea
     • Intubation? trach?
  • Symptoms during exertion
     • Competitive sports? Scholarship?
     • Over time symptoms may be present at rest
  • Reflux/allergy sx’s
  • Psychiatric history
     • Type A, high achieving personality
                         Evaluation
• Exam
  • Focused general exam
  • Dynamic laryngeal exam
    +/- strobe
     • “Seeing is believing”
     • Inciting factors as
       necessary
     • Supraglottic tissues
                       Evaluation
• Laryngeal EMG?
  • May show adductory firing
    during inspiration
• Spirometry?
  • Flattening of inspiratory loop
  • Preserved FEV1
• Imaging as dictated by
  history to r/o organic
  causes
So now we know what PVFM is…
          • But what is this?




            • Is it related?
   • Is it part of the PVFM spectrum?
          14 year old with cough
• Cough of several months duration that began
  after a respiratory infection.
• Cough interfered with school
  • His cough became so disruptive he stayed home
    from school for the past 2 months.
  • Could not make himself understood at school
  • Social frustration
         14 year old with cough

• Treatment with antibiotics and then albuterol
  provided no relief.
• PMH
  • Adopted, no known birth history
  • Hx of mild asthma.
• Soc-only child, excellent student
• ROS-no fevers, weight loss or night sweats
• Medications: Robitussin DM
           14 year old with cough
• Exam: RR 18, comfortable but unable to stop
    coughing
•   Oxygen saturation in RA: 99%
•   Chest: clear
•   Abd: benign
•   Ext: no clubbing
      Differential for Chronic Cough
• LARGE!!!
• Post-nasal drip (sinusitis/ adenoid hypertrophy)
• Cough variant asthma
• GER/LPR
• Infections
• Foreign body
• Habit Cough
• Functional Cough/ Functional Voice Disorder


          FIRST YOU MUST RULE OUT THE BAD
                      Habit cough
• Persistent cough with no organic cause
  • Variety of presentations (positional, temporal
    associations
• Often triggered by a physical stimulus such as
 illness
  • becomes habitual in nature after the original etiology
    resolves
  • Triggers include:
     • Increased physical or emotional stress, anxiety, etc.,
     • Secondary gain (missing school, avoiding a bully, etc.)
• Can become debilitating
        PVFM & Habit Cough
• Are they along the same spectrum of conditions?
• Maybe so, maybe not…
• Similar associations and symptoms of PVFM
  • Often underlying anxiety provoking issue
• Similar therapies often provide relief
                  Treatments
• Each child is individualized
• Although the therapies for PVFM and habit
  cough are similar, each case has specific
  nuances
• No room for “cookbook” medicine
Treatment options?
• Speech/Voice therapy
• Psychiatric techniques
  •   Biofeedback
  •   Hypnosis?
  •   Psychotherapy
  •   Medication
• Botox
• Nebulized lidocaine
         Role of Voice Therapy
• The vast majority of children respond to
  targeted voice therapy
• Empower the child with knowledge and skills to
  reduce the anxiety component associated with
  not being able to breathe and calm the laryngeal
  response
• “Teach them that they are in control”
      Voice Therapy Evaluation

• In depth history
• Differentiate specific laryngeal symptoms
  from similar conditions (asthma variants)
• Identify specific triggers. Laryngeal breathing
  pathologies generally have specific, often
  predictable triggers.
    Voice Therapy for PVFM spectrum
• Laryngeal Control Exercises
   • Active control of laryngeal muscles
   • prevent, reduce, and stop muscular adduction at the level of the glottis.


• Laryngeal Desensitization
   • Behavioral modification
   • elimination of excessive, non-productive phonotrauma


• Breathing techniques
   • Specific breathing techniques known to optimize abduction of the vocal folds
   • restore comfortable, relaxed breathing and calm the larynx
   • reducing the overall anxiety component of not being able to breathe.
      When to refer to psychiatry?
• When the patient has failed a trial of speech therapy
• When the patient has significant functional impairment
  at home or at school
• When the patient has clear symptoms of a psychiatric
  illness
• Consideration of screening surveys
  • Revised Children’s Manifest Anxiety Scale (RCMAS)
     • 37 question anxiety survey

• When your gut tells you to!
       Psychological Assessment
• 3 Components:
   • Functional behavioral analysis
   • Evaluation of concurrent stressors
   • Psychiatric diagnostic evaluation
    Functional Behavioral Analysis
• What role do the symptoms play in the patient’s
  environment?
• Do the symptoms allow the patient to avoid something
  difficult?
  • School
  • Family events
  • Peer interactions
• Although not conscious, this avoidance is a powerful
  reinforcer and may have triggered the symptom
 Evaluation of Concurrent Stressors
• Voice symptoms can emerge as the result of a
  stressful event with which the patient has
  difficulty coping
• Examples from clinical practice include:
  • Divorce
  • Parent’s illness
  • Adoption of a sibling
  Psychiatric Diagnostic Evaluation
• Clinical interview which assesses for the
  presence of psychiatric disease
• Assess symptoms in major domains of disease
  • Mood disorders, Anxiety disorders, Psychosis
• Evaluate, in particular, for the presence of co-
  morbid disorders:
  • Anxiety Disorders, Conversion disorder
  • Rule out Tourette’s, Tic disorders
          Treatment Algorithm
• Biofeedback
• Hypnosis
• Psychotherapy
• Medication
  Treatment Algorithm- Biofeedback
• Learned control of a
  physiological process that is
  normally involuntary or no
  longer under voluntary
  control
• Requires an external device
  to measure the physiological
  response
   •   Surface EMG
   •   Heart Rate Variability
   •   Respiration Rate & Amplitude
   •   Skin Temperature
   •   Skin Conductance
   •   Neurofeedback
 Biofeedback: Mechanism of Action
• Operant conditioning -
  adaptive, directional
  physiological change is
  intrinsically reinforcing
• Form of skill training -
  response is present in
  rudimentary form
• Nonspecific relaxation
  response - learned through
  knowledge of results and
  therapist controlled
  reinforcement
   Treatment Algorithm- Hypnosis
• For patients with inadequate or no response
 after 3-6 sessions of biofeedback, consider
 hypnosis
                Hypnosis
…the early study of [hypnotism] constitutes
 practically the beginning of the
 experimental psychology of motivation.

 EDWIN G. BORING (1950)
       How Does Hypnosis Work?
• Dissociation
  • Hypnotic responses occur in an altered state of
    consciousness
• Social psychological
  • Hypnotic responses are intentional acts with deep
    imaginative involvement and absorption
           Treatment Algorithm-
              Psychotherapy
• For patients with significant concurrent
 stressors:
  • Psychotherapy to increase coping skills
  • School or family interventions as needed
     • increased academic support for patients with learning
       disabilities
     • parent guidance and reassurance in symptom
       management
  Treatment Algorithm- Medication
• For patients with co-morbid psychiatric illness:
  • Psychiatric medication if indicated
• Anxiety
  • Benzodiazepines (short-term management)
  • SSRIs (longer-term management)
  • Atypical antipsychotics (low dose)
• Depression
  • SSRIs
  • Atypical antidepressants
       Returning to our first case
• Initial attempts at voice therapy failed…
• Underwent biofeedback by endoscopically
 observing her laryngeal exam
 Another option when therapy fails
 (or you need immediate results…)
• Returning to the child with the cough
• No improvement with initial voice therapy
• Parents and child are desperate
  • Need results now
                 Other options?
• Botox injection to thyroarytenoid
  • Break the cycle to allow voice therapy to work
  • May provide short or long term relief
  • INFORMED CONSENT is mandatory
  • Variable dosing: 5 - 7.5 units to each TA
  • ? Improved outcomes with EMG confirmed
    diagnosis
      Botox in pediatric laryngology
• Informed consent is paramount
  • February 2008 FDA warning
  • http://www.fda.gov/cder/drug/early_comm/botulinium_toxins.htm
             Patient’s clinical course
• Botox Injections
   • 5 u to bilat TA
• f/u 1 week time interval
• Referred for psychiatric eval
• Uncovered deep anxiety
  regarding mother’s kidney txp
• Recurrence at 10 weeks
  managed with biofeedback
  and complete resolution
                       Other options
• Nebulized lidocaine
  • In the setting of habit cough that has become
    intolerably disruptive of sleep
  • Dosing based on pediatric bronchoscopy literature
     • 4mg/kg of 2% lidocaine solution
     • Full cardiac monitoring?
  • Short term solution that may allow therapy to be
    successful

     Gjonaj ST et al. Chest. 1997 Dec;112(6):1665-9
                  In summary
• PVFM represents the physical manifestation of a
  paradoxical overexuberant laryngeal adduction
• PVFM presents as a spectrum of disorders
  arising between the CNS and the larynx
• Habit cough may represent a different
  manifestation of the spectrum
• Therapy is a multidisciplinary quest to uncover
  the underlying problem and provide relief
                 One last case…
• 12 year old female brought to clinic on a stretcher
• Past 6 months uncontrollable cough
   • Multiple admissions with negative w/u
   • Has missed a tremendous amount of school
• Only means to control is to lie flat
• No recent illnesses or sick contacts
• No previous medical history
               One last case…




     • Mother and father divorced for several years
• Symptoms tracked to new boyfriend moving into house
               One last case…
• Same day referral for voice therapy
  • Unable to break
• Discussed options
• Given acuity and inability to sit upright
  • Botox 7.5 units to each TA
  • Cough well-controlled postoperatively
        One last case…




            • Immediate control
   • Referred for psychiatric evaluation
      • Long term control with psychotherapy
• Now back in school and resuming her life
Thank you!

								
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