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52 Cards in this Set
- Front
- Back
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How do you make diagnosis of cystic fibrosis
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- Recurrent lung infections and sometimes chronic malabsorption leading to failure to thrive (85% of patients suffer from pancreatic insufficiency)
- Abnormally high levels of chloride in sweat = positive sweat test |
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What is the most serious problem in cystic fibrosis
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Obstruction of airways by viscous mucus in the bronchioles and colonization by bacteria - Staph Aureus and Pseudomonas - leads to lung destruction (bronchiectasis is common) and also cause R heart failure - most patients die from respiratory infections
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Which GI problem is present in patients with CF
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Pancreatic ducts are obstructed by mucus resulting in deficient secretion of pancreatic enzymes and indigestion of proteins and fats - failure to thrive and production of copious maloderous stools
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Describe management of CF
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- Vigorous regular chest PT with antibiotic treatment for infections
- Dietary supplementation of pancreatic enzymes and fat soluble vitamins (ADEK) - Surgical intervention for small intestinal obstruction and both liver and heart-lung transplants = extreme cases |
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Name gene defective in CF
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CFTR = cystic fibrosis transmembrane regulator, functions as chloride channel
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Which amino acid is deleted in CF and where - this mutation responsible for 70% of cases
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Phenylalanine at position 508 in the CFTR
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Which method is used for screening of carriers of CF
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PCR
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What type of protein is alpha 1 antitrypsin
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Serine protease inhibitor - major component of alpha 1 globulin of human plasma and is synthesized by hepatocytes and also macrophages
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What is the major function of alpha 1 antitrypsin
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Protect lungs from digestion by inhibition of the activity of elastase - a proteolytic enzyme produced principally by neutrophils
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Alpha 1 antitrypsin defficiency results in _
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Chronic pulmonary disease (emphysema) and liver disease
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Which mutant alleles are responsible for alpha 1 antitrypsin defficiency
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S and Z
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Pittsburgh variant antitrypsin defficiency
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Missense point mutationsubstituting an arginine for the methionine - destroys affinity for elastase but now targets thrombin causing lethal bleeding disorder
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2 causes of increased pulmonary disabilities in Americans
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Smoking and air pollutants
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Common diagnoses of patients who would benefit from pulmonary rehab
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Obstructive - asthma, COPD (bronchitis + emphysema)
Cystic fibrosis - bronchiectasis Restrictive diseases - interstitial fibrosis, pneumoconiosis, kyphoscoliosis Lung reductin and transplant candidates before and after the surgery |
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Conditions that would prevent patient from participating in a rehab
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Terminal disease states
Psychiatric problems- dementia, substance abuse Serious medical conditions - unstable CHF, post MI, CVA |
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Parameters that should be monitored during simple 6 or 12 minute walk
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SpO2
BP EKG Respiratory rate Symptoms- orthopedic as well as respiratory |
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3 possible reasons for loss of appetite in patinets with COPD
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Air swallowing, dyspnea
Using O2, loss of sense of smell and taste Taking meds and water (stomach full) |
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What is collaborative self management in pulmonary rehab
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Assesment of learning abilities and needs - involves patient, family and caregivers, hypoxemia can impair ability to leart and retain information
Educate on topics of anatomy and physiology, pathology, meds, diet/nutrition, relaxation techniques, self care, establishing realistic objectives/goals, patient interests, travel and sexuality, and end of life decision making (advanced directives) Testing on those can formal or informal, need to repeat demonstrations if needed |
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Common psychosocial issues that commonly effect pulmonary rehab candidates
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Depression
Role reversal Dependence (loss of independence) Low self esteem Anger Fear Learning abilities Coping difficulties/strategies |
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2 contraindications for Trendelenburg position if performing chest physical therapy
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Vomiting and risk of aspiration
Recent CVA or cranial surgeries |
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Describe technique for pursed lip breathing
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Inspire during diaphragmatic breathing
Exhale normally through pursed lips Emphasize slow exhalation not forced Practice is required |
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Benefits of pursed lip breathing
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Slows respiratory rate
Prevents or delays airway collapse by pressure splinting airway Decreases air trapping Decreases dyspnea |
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Describe 3 oxygen supply methods available to home care patients
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Concentrators for continuous users who mostly stay at home
Liquid oxygen for continuous users who are active Mechanical ventilation at home - conventional positive pressure ventilation, noninvasive positive pressure ventilation, bilevel positive pressure ventilation BiPAP |
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Home oxygen supply method most suited for active ambulatory patient
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Liquid oxygen for continuous users who are active
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Identify disadvantage or hazard of each home oxygen supply method
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Liquid O2 - potential hazard of thermal injury if not proporly handled
Cylinders - high pressure systems, finite amount, needs to be monitored to avoid runing out Concentrators for stay at home users - increase electric cost, preventative maintenance, need back up power supply |
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3 benefits of exercise training to a dyspnea limited COPD patient
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Desensitization to dyspnea
More mechanically efficient Psychologically - improved ego strength Carries over to ADL |
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Benefits of pulmonary rehab
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Quality of life
Reduction in symptoms Increased exercise tolerance Increase in functional activities of daily living Increased independence Improved psych function |
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Describe appropriate use of nicotine replacement along with behavior modification in smoking cessation
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Nicotine addicted smokers (> 1 pack a day) benefit from nicotine replacement
Nicotine replacement must be accompanied by behavior modification Recent studies indicate better success with nicotine replacement than "cold turkey" Increased success with bupropion (Zyban) that may also help prevent undesirable weight gain |
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Currently recommended immunizations for someone with pulmonary disability
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Pneumonia every 6 years
Influenza yearly |
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Virchows triad
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Stasis
Hypercoagulability Endothelial injury |
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Shortness of breath + clear lungs - what should you suspect
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PE
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Describe pathophysiology of PE
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Impaired gas exchange --> increased dead space leading to hypoxia
Increased pulmonary vascular resistance --> caused by obstruction and neurohormonal influence (serotonin) Increased airway resistance --> bronchoconstriction Decreased pulmonary compliance --> lung edema, hemorrhage, loss of surfactant |
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Name risk factors for PE
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DVT
BCP CA CHF Bed bound Coagulopathy |
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What is the most common coagulopathy
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Factor V Leiden mutation
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What are the common symptoms of PE
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Dyspnea
Pleuritic chest pain Cough - possible hemoptysis Leg pain and swelling - DVT Palpitations, diaphoresis, wheezing Non specific symptoms Massive cardiovascular collapse, coma, death |
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What are the signs of PE
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Tachypnea
Tachycardia Rales (crackles) or wheezes LOUD P2 Pleural friction rub Cyanosis |
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Which work up is ordered in patient with PE
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History
PE CBC, EKG, CXR, ABG CT angiogram - gold standard Lung scan - not used as much |
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Westermarks sign - decreased vascularity or focal oligemia is the sign of _
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PE
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Pleural effusion, atelectasis and decreased vascularity on x ray are signs of _
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PE
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Pleuritic chest pain + hemoptysis =
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Pulmonary infarction
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Chest x ray is suggestive of pneumonia - shows parenchymal density which extends to the pleural surface - what else should you think about?
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PE with infarction
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EKG in PE
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NSR - 30%
Sinus tachycardia Right heart strain pattern - RBBB, S1, Q3 A fib, A flutter (PAC, PVC) P pulmonale (peaked in II,III) |
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Very sensitive but not specific test for PE - gives many false positives, but very few false negatives
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D dimer
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What is the differential diagnosis of PE
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MI
Pericarditis CHF Pneumonia COPD/Asthma Pneumothorax |
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Tests for DVT
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Ultrasonogram
MRI Venography Radionuclide scanning |
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Treatment of PE
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First thing is to prevent further PE - heparin and warfarin (anticoagulants, not thrombolytics), dont dissolve blood clots
O2 IVC filter - bleeding, recurrences Thrombolytic therapy - massive PE, hemodynamic instability Intubation and/or vasopressors Embolectomy |
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Give examples of non thrombotic PE
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Fat embolism - long bone fractures, petechiae, confusion
Amniotic fluid embolism - postpartum pulmonary edema IVDA -talc |
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Bronchial breath sounds heard peripherally (where they are not supposed to be heard) - diagnosis?
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Pneumonia (consolidation)
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Egophony is present in what disease
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Pneumonia
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Wheezing in people without asthma is the sign of _
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CHF
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Crackles and rales are lung sounds heard in _
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CHF
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Silent chest + signs of respiratory distress are indicative of _
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Asthma - severe - emergency
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