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Unusual Respiratory Disorders

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Title: Unusual Respiratory Disorders


1
Unusual Respiratory Disorders
  • Steve Cole
  • Paramedic, CCEMT-P

2
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.

3
Unusual Respiratory Disorders
  • Vocal Chord Dysfunction (VCD)
  • Cystic Fibrosis (CF)
  • Adult Respiratory Distress Syndrome (ARDS)

4
Vocal Chord Dysfunction (VCD)
5
VCD- Introduction
  • First suspected in the early 80s, 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.

6
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).

7
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

8
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.

9
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)

10
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

11
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

12
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

13
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.

14
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

15
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 DXed
  • 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.

16
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

17
Cystic Fibrosis
18
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 20s, and
    tend to have a poor quality of life.

19
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

20
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

21
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

22
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)

23
Cystic Fibrosis-Common Tx
  • Good Respiratory Hygiene
  • Physical Therapy (Percussion,)
  • Antibiotic Therapy
  • Nebs
  • Experimental Therapies are common and underway
  • Lung Transplants (cadaveric and living)

24
Cystic Fibrosis- Common Tx
  • Intubation (if no DNR) Remember to allow
    increased expiratory times.
  • Frequent Suctioning
  • Nebulizers
  • Steroids

25
Cystic Fibrosis- The Vest
26
Cystic Fibrosis TOBI an inhaled antibiotic
27
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.

28
Adult Respiratory Distress Syndrome (ARDS)
29
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 60s and 70s by Dr. R
    Cowley (yes THAT Cowley)and by Dr. Ash Baugh and
    coworkers, in 1967

30
ARDS-Common Causes
  • There is ALWAYS a precipitating event
  • Sepsis
  • bronchial aspiration of gastric contents
  • multiple trauma
  • massive blood transfusions
  • low-perfusion states (SHOCK)

31
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.

32
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

33
ARDS- 3 stages of ARDS
  • Exudative Phase( phase of injury and
    inflammation)
  • Fibroproliferative phase
  • Fibrotic Phase

34
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

35
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, ABGs and Swan Ganz
    Cath.
  • Physical signs are acute respiratory failure,
    decreased PaO2, Increased PCO2, decreased lung
    compliance, and non cardiogenic pulmonary edema.

36
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.

37
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

38
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 pts long term survival

39
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.

40
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.
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