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.
• I have chosen these because these three conditions
are all something I have seen myself.
Unusual Respiratory Disorders
• Vocal Chord Dysfunction (VCD)
• Cystic Fibrosis (CF)
• Adult Respiratory Distress Syndrome
(ARDS)
Vocal Chord Dysfunction (VCD)
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- 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?
• 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 it?
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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
• 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
• 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 DX
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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
• 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
• 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/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.
• 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
• 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
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?
• 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?
• 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
• 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
• 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
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Good Respiratory Hygiene
Physical Therapy (Percussion,)
Antibiotic Therapy
Nebs
Experimental Therapies are common and
underway
• Lung Transplants (cadaveric and living)
Cystic Fibrosis- Common Tx
• Intubation (if no DNR) Remember to allow
increased expiratory times.
• Frequent Suctioning
• Nebulizers
• Steroids
Cystic Fibrosis- The Vest
Cystic Fibrosis – TOBI an
inhaled antibiotic
Cystic Fibrosis- What does all
this mean to you?
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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.
Adult Respiratory Distress
Syndrome (ARDS)
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
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
• 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 byproducts 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 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
• Exudative Phase( phase of injury and
inflammation)
• Fibroproliferative phase
• Fibrotic Phase
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 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
• 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
• 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 you
1. Don't over-ventilate Spirometry and peak
pressure valves are helpful.
2. Allow for a longer inspiratory time.When
bagging the patient deliver air slowly and
evenly.
3. Consider sedation or pain management This
will increase respiratory compliance.
4. ETCO2 detector is highly recommended
5. Assess the patient frequently for barotrauma.
ARDS -Summary
• By introducing an understanding of
ventilator management, pathophysiology of ARDS, and impact of
therapies we will be better pt. care
advocates and providers.