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?

• 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

• 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

• 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?

• 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 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 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, patho-physiology of ARDS, and impact of therapies we will be better pt. care advocates and providers.