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Unusual Respiratory Disorders. Steve Cole Paramedic, CCEMT-P. Unusual Respiratory Disorders. Discussion of unusual and interesting respiratory conditions could take a year. We have just an hour You deserve your moneys worth I have chosen three conditions to give you something to talk about.

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Unusual Respiratory Disorders

Steve Cole

Paramedic, CCEMT-P

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Unusual Respiratory Disorders

  • Discussion of unusual and interesting respiratory conditions could take a year.

  • We have just an hour

  • You deserve your moneys worth

  • I have chosen three conditions to give you something to talk about.

  • I have chosen these because these three conditions are all something I have seen myself.

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Unusual Respiratory Disorders

  • Vocal Chord Dysfunction (VCD)

  • Cystic Fibrosis (CF)

  • Adult Respiratory Distress Syndrome (ARDS)

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VCD- Introduction

  • First suspected in the early 80’s, VCD is a condition that may mimic Asthma and other reactive airway disorders.

  • Nearly 25 percent of patients who are referred to National Jewish (A major respiratory care system) with the diagnosis of asthma actually have vocal chord dysfunction (VCD)

  • VCD strikes people of all ages, though the condition is seen most often in women between the ages of 20 and 40.

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VCD- Introduction

  • Based on the similarity of presentation to asthma, and due to the relative newness of this DX, many patients are TX for asthma.

  • Complicating this is that many patients may have VCD and Asthma both.

  • Undiagnosed VCD Patients have even been seen in emergency rooms with this problem, and admitted to an intensive care unit with the diagnosis of status asthmaticus (life-endangering asthma).

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VCD- What Causes it?

  • Still figuring it out

  • Has many of the same triggers as other reactive airway disorders.

  • Many people with VCD have difficulty expressing direct anger, sadness or pleasure, and experience depression, obsessive-compulsive personality, passive-dependent personality, or a borderline personality. As such there is a theory of a possible psychological component as well.

  • Some get it “On the Job”

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VCD- What Causes it?

  • Exercise/physical activity

  • Stressful situations

  • Menses

  • Singing

  • Inhalation Injury

  • Sinus and Upper Respiratory irritation/infection

  • Pattern of VCD episodes may be unpredictable (unlike asthma which is usually readily apparent )

  • Sometimes the cause is not known.

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VCD-What's Going on

  • Vocal Cord Dysfunction: VCD is a clinical syndrome where the vocal cords decrease in size by 10-40 percent.

  • Sometimes patients experience abnormal vocal cord inhalation during the entire breathing cycle (these are the most severe)

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VCD- Making a DX

  • Characteristics of VCD include asthma-like symptoms, yet the S/S do not respond well to typical asthma therapies, or despite escalating therapies.

  • Air flow limitation in the vocal chords causes a choking sensation in the throat

  • Difficulty swallowing during episodes

  • Sometimes the wheezes can be clearly heard over the throat be auscultation, but this is not reliable

  • Distinct voice changes during attacks

  • Difficulty swallowing during normal periods

  • Always consider this disorder when a patient presents with inspiratory wheezing; expiratory wheezing is typical of asthma.

  • SEVERITY- This may present with all of the severity of a regular asthma attack

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VCD- Making a DX

  • True Dx is done by a specialist

  • May involve a “Flow Volume Loop” Test

  • Will often involve Laryngoscopy.

  • Typically involves trying to induce the symptoms (sometimes difficult)

  • It is generally considered that true VCD patients cannot produce the s/s at will.

  • Spirometry

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VCD-Common Tx regimens

  • Speech therapy

  • Relaxation (of the vocal chords)technique (very important)

  • Special Breathing techniques

  • Psychotherapy.

  • More severe attacks are treated with a mixture of helium and oxygen which promotes a less turbulent flow of air past partially obstructed vocal cords

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Speech Tricks

  • In some cases, breathing oxygen (without helium) has helped stop VCD attacks.

  • VCD patient can try EXHALING through pursed lips, whispering the sound "f f f f f", "f f f f f", "f f f f f", against a little resistance, in somewhat short, quick bursts, all in the same exhalation. (Do this, using breath & lips, without vibrating the vocal cords.) Some prefer whispering "s s s s s", or, "s h h h h". This panting/breathing/speech therapy exercise has helped stop VCD attacks in some patients.

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Abdominal Breathing

  • ABDOMINAL/diaphragmatic breathing means: While exhaling, the abdomen (belly) comes "in"/towards the "back", making the belly seem smaller; then, while inhaling, the abdomen (belly) gets pushed "out", to expand/increase the size of belly.

  • During abdominal/diaphragmatic breathing, try to NOT use chest or throat muscles.

  • Speech Therapists/Pathologists teach these important breathing techniques

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VCD- What this means to you.

  • You are not expected to DX and Tx VCD

  • You may be required to assist/Tx a pt with VCD already DX’ed

  • As more and more physicians become aware of this condition, more and more patients will have knowledge of various ways to self tx VCD. It is likely that as a field provider you will be presented with this.

  • It is important also for you to know that asthma (and similar d/o) can co exist with this disorder and are considered co-morbid. Do not delay Tx in the symptomatic.

  • It is important for us to be educated in this d/o, so we can communicate effectively with the patient and his loved ones. This will in turn make our job easier.

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VCD- Summary

  • Vocal cord dysfunction syndrome is characterized by episodes of paradoxical movements of the vocal cords, which close rather than open on inhalation, creating a wheezing-type sound.

  • Patients often have a variety of self Tx that they do which may seem odd.

  • The causes are many, the Dx is difficult, but as asthma cases grow in the US, so will the incidence of VCD

  • www.cantbreathesuspectvcd.com

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Cystic Fibrosis- Introduction

  • Cystic fibrosis (CF) is a genetic disease affecting approximately 30,000 children and adults in the United States

  • The Defective Gene was isolated in 1989

  • One in 31 Americans (one in 28 Caucasians) - more than 10 million people - is an unknowing, symptom less carrier of the defective gene

  • Patients seldom survive into the late 20’s, and tend to have a poor quality of life.

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Cystic Fibrosis- What causes it?

  • Genetic defect

  • An individual must inherit a defective copy of the CF gene from each parent

  • CF causes the body to produce an abnormally thick, sticky mucus within cells lining organs such as the lungs and pancreas

  • This mucus production leads to other systemic problems as well

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Cystic Fibrosis- What's Going on?

  • Genetic defects cause faulty transport of sodium within certain cell linings.

  • This results in thick, fibrotic Mucus production in the lungs and pancreas. This mucus makes the patient very susceptible to respiratory infections.

  • Long term inhibition of pancreatic excretion can cause diabetes in these patients.

  • Effects on the GI system make the patient prone to obstructed bowels

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Cystic Fibrosis- Making a DX

  • Dx is made by a specialist using a “Salt Test” combined with CXR.

  • salty-tasting skin

  • persistent coughing

  • wheezing or pneumonia

  • excessive appetite but poor weight gain

  • Barrel Chest, protruding abdomen

  • Elevated CO2

  • General Failure to thrive

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Cystic Fibrosis- Common Presentations

  • SOB/Respiratory Complaints (Increased cough frequency and severity followed by shortness of breath, Increase in sputum or change in color of sputum, Bloody Sputum, etc…)

  • Persistent vomiting, Excessive thirst Increased urination

  • Severe Constipation - lack of bowel movements for 2 or more days

  • Severe drug interactions/allergic reactions (i.e.; rash, hives, GI upset, joint pain, mental changes and others related to patients specific drug therapies)

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Cystic Fibrosis-Common Tx

  • Good Respiratory Hygiene

  • Physical Therapy (Percussion,)

  • Antibiotic Therapy

  • Nebs

  • Experimental Therapies are common and underway

  • Lung Transplants (cadaveric and living)

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Cystic Fibrosis- Common Tx

  • Intubation (if no DNR) Remember to allow increased expiratory times.

  • Frequent Suctioning

  • Nebulizers

  • Steroids

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Cystic Fibrosis- What does all this mean to you?

  • Get a detailed subjective Hx

  • Standard Respiratory care similar to COPD

  • Allow Percussion if possible

  • If over long distance transport, prepare to accommodate other therapy as well

  • Be aware of unusual medication interactions and/or side effects

  • Be vigilant for other associated diseases,.

  • Respiratory Hygiene is crucial.

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ARDS- Introduction

  • While ARDS was first Dx less than 20 years ago, it has been around under other names for most of the century.

  • Called Shock Lung, Post-Pump Lung, and other various names. Very few documented cases early on because few patients survives to get it.

  • Early research in the 60’s and 70’s by Dr. R Cowley (yes THAT Cowley)and by Dr. Ash Baugh and coworkers, in 1967

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ARDS-Common Causes

There is ALWAYS a precipitating event

  • Sepsis

  • bronchial aspiration of gastric contents

  • multiple trauma

  • massive blood transfusions

  • low-perfusion states (SHOCK)

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ARDS-What's Going On

  • Poor Perfusion (SHOCK) leads to increased permeability of alveolar membranes

  • This in turn destroys the alveolar epithelial barrier

  • This opens the the alveolar space to inflammatory by-products and these substances destroy surfactant.

  • surfactant deficiency is a crucial component of this syndrome

  • This eventually leads to decreased alveolar space, alveolar collapse, and respiratory failure.

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ARDS- What's going on

  • S/S usually sneak up on you, 12-48 hours post event.(Exception: Aspiration Pneumonia)

  • Patients who die of respiratory failure usually show a progressive decrease in lung compliance, worsening hypoxemia, increased respiratory effort and tiring, and progressive increase in dead space with hypercapnia

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ARDS- 3 stages of ARDS

  • Exudative Phase( phase of injury and inflammation)

  • Fibroproliferative phase

  • Fibrotic Phase

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ARDS-Making a DX

  • Many Patients are under the age of 65 with no prior HX or indication of heart /lung disease.

  • Can occur even in children

  • Pulmonary Hypertension (detected via a PA cath) is common due to increased pulmonary vascular resistance

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ARDS- Making a DX

  • There are no lab test of pulmonary endothelial/epithelial injury

  • The diagnosis of ARDS is that of exclusion.

  • Nevertheless, some laboratory and radiographic tests may be useful. CXR, ABG’s and Swan Ganz Cath.

  • Physical signs are acute respiratory failure, decreased PaO2, Increased PCO2, decreased lung compliance, and non cardiogenic pulmonary edema.

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  • Very different to tell difference on X-Ray, ARDS vs APE

  • Heart silhouette size is usually normal

  • ARDS have a more peripheral, uneven and patchy distribution of pulmonary edema when compared with the even and perihilar (bat-wing) features of cardiogenic pulmonary edema

  • pleural effusions in ARDS is less than that of cardiogenic pulmonary edema.

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ARDS- Common Tx Regimens

  • Early Intubation is recommended. Strong aggressive ventilator management is required. (That means “Transport/ICU Grade Ventilators”)

  • PEEP. PA caths help with determining PEEP

  • Inverse ratio ventilation, Permissive hypercapnia, Prone positioning of the Patient, pressure control ventilation, Hi.-frequency Jet ventilation and are all therapies that may be encountered.

  • Steroids have been used, no benefit shown on studies.

  • Tx of co-morbid infections and problems

  • Dietary support

  • Surfactant replacement

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ARDS- What this means to you

  • In the pre-hospital setting, destination choice (with good ICU care) can make a huge difference

  • Lung volume may be decreased up to 66% Standard preventive measures such as reducing Barotrauma are important

  • In the Critical Care arena, careful monitoring of patients SAO2, ETCO2,PEEP, FIO2 and other vent settings are crucial to pt’s long term survival

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ARDS- What this means to you

  • Don't over-ventilate Spirometry and peak pressure valves are helpful.

  • Allow for a longer inspiratory time.When bagging the patient deliver air slowly and evenly.

  • Consider sedation or pain management This will increase respiratory compliance.

  • ETCO2 detector is highly recommended

  • Assess the patient frequently for barotrauma.

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ARDS -Summary

  • By introducing an understanding of ventilator management, patho-physiology of ARDS, and impact of therapies we will be better pt. care advocates and providers.