Kwasnik.Diagnosis & Management Of Pulmonary
3/4/2021Diagnosis and Management ofPulmonary Manifestations inNeuromuscular Disease Aleksandra Kwasnik, MD Billings Clinic Department of Pulmonary, Critical Care and Sleep Disorders Big Sky Pulmonary Conference March 5th 2021Objectives Review types of neuromuscular injury/ illness and associated disability Discuss diagnostic tests and considerations Overview of management Example of specific disease entity and society recommendations for its managementAnatomy ofNeuromuscularDysfunction1
3/4/2021Patterns of respiratory abnormality in neuromuscular disease Alterations in respiratory drive Compensatory to chronic hypoventilation Bulbar/ oropharyngeal weakness Aspiration/ choking risk Vocal cord spasticity Inspiratory weakness Hypoventilation Expiratory weakness Ineffective cough/ bronchial clearanceDiagnosisHistoryPhysicalEarly signs: fatigue, orthopnea, morningheadache, dyspnea on exertionSpeaking in phrases/wordsLate signs: dyspnea at restInclude questions targeting OSA riskThoracoabdominal dyssynchrony, accessorymuscle useCoexistent anatomical abnormalities (e.g.kyphoscoliosis)Pulmonary functiontesting Spirometry (including uprightand supine FVC measurement) NIF/MIP/SNIP Cough peak flow Arterial blood gas analysis Pulse oximetry Nocturnal oximetry2
3/4/2021Polysomnography Utility Patients with intact PFTs butsigns/symptoms ofhypoventilation Suspected comorbid OSA/CSA Troubleshooting noninvasiveventilation Drawbacks Cost Wait times/ delays Patients may need assistancewith ADLs/mobility difficult forsleep lab staffDefinitions Required for diagnosis of restrictive thoracic disorder and/or chronicrespiratory failure related to neuromuscular disease Symptoms of hypoventilation Required testing (one or more of the below): Awake ABG with pCO2 45 Nocturnal oximetry with 5 min SpO2 88% NIF/MIP/SNIP 60 cmH20 Upright (or supine) FVC 50% predicted Not required: Sleep study!!!3
3/4/2021Managementoverview Referral to specialty multidisciplinary clinic if available/desired Nasopharyngeal care Oropharyngeal manifestations Bronchial hygiene/ cough support Noninvasive ventilation Invasive ventilation Supportive nutrition/ hydration Palliative care Aspiration precautions Management of siallorhea Nocturnal Sip ventilation Emphasis on protein/ caloric adequacy Timing of feeding tube laints Signs/ symptoms: Congestion, inability to breathethrough nose/ blow nose Causes: Rhinitis of disuse Mouth breathing Rhinitis medicamentosum Medications Nasal steroids (caution: thrush) Nasal ipratropium Nasal azelastine4
3/4/2021Oropharyngeal issues Aspiration precautions Head of bed elevation Speech/ swallow evaluation PEG/G‐tube discussion (timing important inrelation to respiratory function) Management of sialorrhea AtropineGlycopyrrolate (nebulized vs. subcutaneous)Scopolamine patchAmitriptylineBotulinum toxinIrradiationSurgeryPortable suction machineCaution: thrushBronchial hygiene/cough support CPF 270: Manual breath stacking Breath Stacking Demonstration ‐ YouTube CPF 160: Mechanical in/ex‐sufflation Aka cough assist device ‐40/ 40 cm H20 Assessment of sputum consistency Hydration Consider nebulized saline vs.albuterol Chest PT (manual vs. vest)Noninvasive ventilationfor restrictive thoracicdisorders Types: home ventilator vs. respiratoryassist devices **do not use CPAP, auto‐BPAP or BPAPASV for this indication**5
3/4/2021Initiation of non‐invasive ventilation Education Setting of expectations, timing Insurance coverage Required diagnosis: Restrictive thoracicdisorder Required documentation: Symptoms ofhypoventilation Required testing: Awake ABG with pCO2 45 Nocturnal oximetry with 5 minSpO2 88% NIF/MIP/SNIP 60 cmH20 Upright (or supine) FVC 50%predicted Talk to your local DMEsBernardo J. Selim, MD, FCCP; Lisa Wolfe, MD, FCCP; John M. Coleman III, MD, FCCP; andNaresh A. Dewan, MD, FCCP Initiation of Noninvasive Ventilation for Sleep RelatedHypoventilation Disorders: Advanced Modes and Devices CHEST 2018; 153(1):251‐265VT: start 8‐10cc/kg IBWRate 10‐12 unless needhigher based on ABGPS min: at least 5EPAP generally low unlessOSA suspectedPS max: as tolerated**specify high triggersensitivity and default orslow rise time when able6
3/4/2021Does themask matter?Improving comfort/ adherence Chin strap Offload pressure/weight ofthe tubing Address nasal congestion Early recognition andtreatment of skinbreakdown7
3/4/2021Monitoring of non‐invasive ventilation Download Symptoms Trigger sensitivity Air hunger Nocturnal oximetryAshima S Sahni and Lisa Wolfe Respiratory Care in Neuromuscular Diseases.Respiratory Care May 2018, 63 (5) 601‐608; ive ventilation types Mouthpiece “sip” ventilation Biphasic cuirass ventilationInvasive ventilation Rates of tracheostomy placement forventilation vary by disease state andprognosis E.g. Duchenne MD vs. ALS Special consideration to bulbar weakness Discuss home care vs. placement Caregiver discussions Increased rates of institutionalization Limited chronic vent facilities in thenorthern Rocky Mountain region8
3/4/2021Supportive care Nutrition/hydration Always ask about hydration (manypatients avoid due to mobility issues) Discuss timing of feeding tube Palliative care Advance directives/ 5 wishesOpioidsBenzodiazepinesOxygen generally not recommendedExample: AmyotrophicLateral Sclerosis Why the emphasis? Predictable mortalitypredominantly related torespiratory failure Quality of life and survivalbenefit in early intervention Relatively clear andaccepted guidelines(American Academy ofNeurology)Journal of Neurology Research, ISSN 1923-2845 print, 1923-2853 online, Open Access9
3/4/2021Journal of Neurology Research, ISSN 19232845 print, 1923-2853 online, Open AccessReferences Ashima S Sahni and Lisa Wolfe Respiratory Care in Neuromuscular Diseases. RespiratoryCare May 2018, 63 (5) 601‐608; DOI: https://doi.org/10.4187/respcare.06210 Bernardo J. Selim, MD, FCCP; Lisa Wolfe, MD, FCCP; John M. Coleman III, MD, FCCP; andNaresh A. Dewan, MD, FCCP Initiation of Noninvasive Ventilation for Sleep RelatedHypoventilation Disorders: Advanced Modes and Devices CHEST 2018; 153(1):251‐265 Ikeda, Ken, Osamu Kano, Kiyokazu Kawabe, & Yasuo Iwasaki. "Patient Care and Treatmentin Amyotrophic Lateral Sclerosis." Journal of Neurology Research [Online], 3.1 (2013): 1‐11. Web. 28 Feb. 2021 Mda.orgAny questions?10
Alternative ventilation types Mouthpiece “sip” ventilation Biphasic cuirass ventilation Invasive ventilation Rates of tracheostomy placement for ventilation vary by disease state and prognosis E.g. Duchenne MD vs. ALS Special consider
Pulmonary hypertension mean pulmonary artery pressure (mPAP) 25 mmHg at rest 8 Key Ppa mean pulmonary arterial pressure Ppv mean pulmonary venous pressure CO right-sided cardiac output PVR pulmonary vascular resistance. 3/4/2016 3 Pathophysiology Ppa (CO x PVR) PCWP 9
Symposium on pulmonary hypertension, pulmonary hypertension is defined as mPAP 20 mm Hg and its subgroup Pulmonary arterial hypertension (PAH) is defined as mPAP 20 mm Hg, PCWP 15 mm Hg and PVR 3 Woods Units. Table 1 : Haemodynamic definitions of pulmonary hypertension, 6th world symposium on pulmonary hypertension, Nice, France.
1.5 Persistent pulmonary hypertension of the newborn 1 . Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH) 2. Pulmonary hypertension owing to left heart disease 2.1. Systolic dysfunction 2.2. Diastolic dysfunction 2.3. Valvular disease 3. Pulmonary hypertension owing to lung diseases and/or hypoxia 3.1.
Pulmonary episodes represent 1.16B over two years ASTHMA, 879M, 76% COPD, 276M, 24% Cost Composition of Pulmonary Episodes Total Pulmonary Costs: 1.16B in two years (2012-2013) ASTHMA, 443K, 79% COPD, 120K, 21% Volume Makeup of Pulmonary Episodes Total Pulmonary Episodes: 564K in two years (2012-2013) 8 Costs Included:
Pulmonary hypertension occurs when there is a vasoconstriction of the pulmonary blood vessels. This leads to right to left shunting across the foramen ovale and ductus arteriosus and subsequent hypoxia. Pulmonary hypertension can be primary (rare) or secondary. Secondary pulmonary hypertension can occur with a number of conditions including:
Pulmonary function has relevance for well-being and longevity (1, 2), and, according to World Health Organization data, chronic obstructive pulmonary dis-ease (COPD) was number three in the top 10 global causes of death in 2019 (3). To achieve the goal of early diagnosis and reliable management of obstructive and fibrotic pulmonary diseases .
ICD-10 Diagnosis Code ICD10 Diagnosis Code Description . Bilateral H628X1 Other Disorders of Right External Ear in Diseases Classified Elsewhere . I2601 Septic Pulmonary Embolism with Acute Cor Pulmonale I2690 Septic Pulmonary Embolism
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