Title: Unusual Respiratory Disorders
1Unusual Respiratory Disorders
- Steve Cole
- Paramedic, CCEMT-P
2Unusual 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.
3Unusual Respiratory Disorders
- Vocal Chord Dysfunction (VCD)
- Cystic Fibrosis (CF)
- Adult Respiratory Distress Syndrome (ARDS)
4Vocal Chord Dysfunction (VCD)
5VCD- 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.
6VCD- 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).
7VCD- 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
8VCD- 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.
9VCD-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)
10VCD- 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
11VCD- 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
12VCD-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
13Speech 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.
14Abdominal 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
15VCD- 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.
16VCD- 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
17Cystic Fibrosis
18Cystic 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.
19Cystic 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
20Cystic 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
21Cystic 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
22Cystic 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)
23Cystic Fibrosis-Common Tx
- Good Respiratory Hygiene
- Physical Therapy (Percussion,)
- Antibiotic Therapy
- Nebs
- Experimental Therapies are common and underway
- Lung Transplants (cadaveric and living)
24Cystic Fibrosis- Common Tx
- Intubation (if no DNR) Remember to allow
increased expiratory times. - Frequent Suctioning
- Nebulizers
- Steroids
25Cystic Fibrosis- The Vest
26Cystic Fibrosis TOBI an inhaled antibiotic
27Cystic 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.
28Adult Respiratory Distress Syndrome (ARDS)
29ARDS- 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
30ARDS-Common Causes
- There is ALWAYS a precipitating event
- Sepsis
- bronchial aspiration of gastric contents
- multiple trauma
- massive blood transfusions
- low-perfusion states (SHOCK)
31ARDS-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.
32ARDS- 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
33ARDS- 3 stages of ARDS
- Exudative Phase( phase of injury and
inflammation) - Fibroproliferative phase
- Fibrotic Phase
34ARDS-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
35ARDS- 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.
36ARDS- 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.
37ARDS- 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
38ARDS- 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
39ARDS- 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.
40ARDS -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.