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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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