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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

Unusual Respiratory Disorders

Steve Cole

Paramedic, CCEMT-P

unusual respiratory disorders2
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.
unusual respiratory disorders3
Unusual Respiratory Disorders
  • Vocal Chord Dysfunction (VCD)
  • Cystic Fibrosis (CF)
  • Adult Respiratory Distress Syndrome (ARDS)
vcd introduction
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.
vcd introduction6
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).
vcd what causes it
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”
vcd what causes it8
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.
vcd what s going on
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)
vcd making a dx
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
vcd making a dx11
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
vcd common tx regimens
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
speech tricks
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.
abdominal breathing
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
vcd what this means to you
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.
vcd summary
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
cystic fibrosis introduction
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.
cystic fibrosis what causes it
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
cystic fibrosis what s going on
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
cystic fibrosis making a dx
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
cystic fibrosis common presentations
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)
cystic fibrosis common tx
Cystic Fibrosis-Common Tx
  • Good Respiratory Hygiene
  • Physical Therapy (Percussion,)
  • Antibiotic Therapy
  • Nebs
  • Experimental Therapies are common and underway
  • Lung Transplants (cadaveric and living)
cystic fibrosis common tx24
Cystic Fibrosis- Common Tx
  • Intubation (if no DNR) Remember to allow increased expiratory times.
  • Frequent Suctioning
  • Nebulizers
  • Steroids
cystic fibrosis what does all this mean to you
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.
ards introduction
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
ards common causes
ARDS-Common Causes

There is ALWAYS a precipitating event

  • Sepsis
  • bronchial aspiration of gastric contents
  • multiple trauma
  • massive blood transfusions
  • low-perfusion states (SHOCK)
ards what s going on
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.
ards what s going on32
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
ards 3 stages of ards
ARDS- 3 stages of ARDS
  • Exudative Phase( phase of injury and inflammation)
  • Fibroproliferative phase
  • Fibrotic Phase
ards making a dx
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
ards making a dx35
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.
ards x ray
  • 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.
ards common tx regimens
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
ards what this means to you
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
ards what this means to you39
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.
ards summary
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.