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 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.

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 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.

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 20’s, 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 60’s and 70’s 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, ABG’s 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 pt’s 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.