Pulmonary Diseases Flashcards
classifications of pulmonary disease
1: obstructive
2: restrictive
3: infectious
4: vascular
5: pleural
what is chronic obstructive pulmonary diseases (COPD)?
=generic term for group of diseases that rap air in the lungs
- preventable & treatable but not fully reversible
- # 1 risk factor=cigarette smoking (responsible for 80-90% COPD cases)
- associated with abnormal inflammatory response of the lungs to noxious particles/gases (cigs)
- although COPD affects lungs, also produces systemic consequences
- 3rd leading cause of death in US; 6th worldwide
- 2nd leading cause of disability
- estimated cost $42.6 billion in 2007
2 major categories of COPD
1: non- septic
2: septic
*the difference between the two will help determine if bronchial hygiene will be a primary goal of the PT plan of care
non septic COPD diseases
emphysema
alpha1 antitrypsin deficiency
bronchiolitis obliterans
asthma
septic COPD diseases
cystic fibrosis
chronic bronchitis
brochiectasis
*septic COPD typically produces large volume of sputum, productive chronic cough, colonization of bacteria and fungus associated with chronic infections
mechanisms of COPD
current thinking on the pathophysiology of obstructive diseases, involving mast cells, eosinophils and T lymphocytes, which release a wide range of inflammatory mediators
these mediators act on cells in the airway, leading to contraction of smooth muscle, edema due to plasma leakage and mucus plugging
classifications by PFTs
Obstructive diseases: can’t get air out so residual volume increases
Restrictive: the residual volume is less than normal
COPD: emphysema & chronic bronchitis
- includes small airways disease and parenchymal disease
- Chronic bronchitis clinical dx: cough & sputum production for >3 mon to >2years
- Emphysema pathologic dx: destruction of gas-exchanging surface of lung (alveoli & capillaries)
*most pts have components of both
non-septic obstructive diseases
emphysema, alpha1 antitrypsin (AAT) deficiency, bronchiolitis obliterans, and asthma
- hyperinflation of the lungs
- barrel chest deformity
- excessive accessory muscle use (shortened; elevated shoulders)
- increased lung compliance
- larger lung volume with trapped air due to premature airway closure
- ABGs: show hypoxia w/ or w/out hypercapnia
- PFTs: increased TLC, IRC, RV; decreased FVC, FEV1, carbon monoxide diffusion capacity (DLCO) and ratio of FEV1/FVC
Secondary sequelae from medication & limited activity:
- type I and II muscle atrophy and weakness
- osteopenia and osteoporosis
- development of R sided heart failure
emphysema
- 2nd most prevalent COPD disease after asthma
- abnormal, non-reversible, airway dilation distal to terminal bronchioles
- decrease in driving & intraluminal pressures leading to impaired expiratory airflow & patent airway during inspiration
- premature airway closure and air trapping, bullae
- increased residual volume
- hyperinflation
- inspiratory muscles are shortened, have decreased sarcomeres and are hypertrophied
pretease-antiprotease hypothesis for emphysema
- emphysema is result of destruction of CT matrix of alveolar walls by proteolytic enzymes (elastase) released by alveolar neutrophils
- elastase normally inactivated by alpha1 antitrypsin
- smoking upsets balance between these two enzymatic processes
signs and symptoms of emphysema
- cough and phlegm
- dyspnea on exertion
- increased A-P diameter on CXR (barrel chest)
on auscultation:
- decreased distant breath sounds
- prolonged expiratory phase
- wheezing/ronchi due to secretons
signs and symptoms of advance emphysema
cachexia R sided heart failure peripheral pitting edema weight gain JVD (jugular venous distention) decreased appetite RUQ (R upper quadrant) discomfort ventricular gallop S3 heart sound osteoporosis depression CVD
emphysema medication management
smoking cessation drugs
Bronchodilators
- antichoinergics
- B2 agonists
- inhaled corticosteroids
methylxanthines: may improve respiratory muscle strength & endurance
during exacerbations:
- inhaled systemic corticosteroids to relieve SOB
- antibiotics for bacterial infection (acute bronchitis and pneumonia)
emphysema treatment
Smoking cessation (behavioral management)
Supplemental oxygen:
- usually low flow O2 via NC is adequate
- especially important in pulmonary HTN and polycythemia (excessive RBC)
- only therapy proven to increase survival
BiPAP to decrease work of breathing
mucus clearing devices
AAT replacement therapy
pulmonary rehab
influenza & pneumococcal vaccine
surgery: bullectomy, LVRS (lung volume reduction surgery) or transplant
alpha 1 antitrypsin (AAT) deficiency
- most common autosomal recessive genetic liver disease in children
- 2nd to CF in genetic pulm diseases
- lungs lack alpha 1 antitrypsin coating. leaving them open to damage by neutrophil elastase. enzyme is synthesized in the liver & inhibits neutrophil elastase which breaks down bacteria from airway -> causes liver and lung damage
- by second decade of life it is primarily a pulmonary disease
- leads to early development of emphysema in 3rd or 4th decade
bronchiolitis obliterans (OB)
- acute inflammatory injury with diffuse destruction of bronchioles associated with underlying inflammatory pathology
- increase in fibroproliferation in bronchioles and collagen deposition in cartilaginous airways
most common cause is associated with lung transplants- often there is a loss of graft function due to chronic rejection
-post transplant incidence is 80% after 5 years with onset of 16-20 months. more than 50% who survive transplant beyond 3 months will develop OB
also found in children and infants following severe respiratory infections
bronchiolitis obliterans (OB) signs and symptoms
- SOB with exertion
- wheezing, crackles, ronchi
- persistent cough
- hypoventilation
- hypercapnia (too much CO2)
- intercostal retraction
- tachypnea (fast/ hyperventilation)
- grunting
CXR: hyperinflation w/ patchy atelectasis
PFT: decreased FVC (forced vital capacity) and FEV1; increased RV
CT scan: mosaic perfusion, vascular attenuation, and central bronchiactasis
bronchiolitis obliterans treatment
- prevention is key
- supplemental oxygen
- antivirals
- corticosteroids
- bronchodilators
asthma definition
- chronic disease
- airway inflammation
- reversible airway limitation (normal lung function between between episodes)
- bronchial hyper-responsiveness
- unknown cause (associated with maturing immune system & lung tissue)
asthma risk factors
- maternal smoking
- second hand smoke
- family history
- genetics
- atopy (allergic hypersensitivity)
- childhood asthma
- occupational exposures
- environmental exposures
- gender
- early infection (RSV)
asthma prevalence
-prevalence rate in 2000 was 7.5% in the US
can occur at any age- most common chronic disease in children
- 80% have age of onset before 5 y/o and 50-70% outgrow it
- 35-40% of children with asthma limited in activity & play
- majority have a family hx
- occupational exposure is related to adult onset
- disproportionately affects those in lower socio-economic levels
- worldwide asthma prevalence and death rates are increasing
asthma signs and symptoms
- wheezing
- dyspnea
- chest pain/tightness especially at night
- facial distress
- non-productive cough
- decreased breath sounds
- cyanosis
- exhaustion
- hypercapnia
- pending respiratory failure
- status asthmaticus= severe attack that doesn’t respond to bronchodilators
asthma triggers/stimulants
- indoor irritants (cigs, CO, nitric oxyide) from poorly vented heating systems
- **air pollution (diesel exhaust, ozone, smoke)
- allergens (pollen)
- pesticides
- mold
- dust mites, rodents, cockroaches, animal dander
- respiratory infection
- exertion and/or exercise (usually 10-15 minutes into exertion, resolves with rest over 30-16 min)
- cold air
- **medications: NSAIDS, ACE inhibitors, aspirin, beta blockers
exercise induced asthma (EIA)
- results from loss of water and heat from lower respiratory system (mouth breathing)
- loss of heat causes hyperemia (^ BF), vascular engorgement & bronchial edema (narrows bronchioles)
- bronchoconstriction may present 6-8 hours post exercise
diagnosis of exercise induced asthma
- history of symptoms in children
- in adults a increase in FEV1 > 15% following use of a bronchodilator
- 15% drop in peak expiratory flow following exercise
severity is determined by minute ventilation during exercise, temperature, humidity of air and baseline airway reactivity
exercise induced asthma trigger
inflammatory process -> bronchoconstriction and airway obstruction
asthma medication management
-short and long acting beta2 agonists bronchodilators
B adrenergic agonists: increase in smooth muscle relaxation resulting in bronchodilation & inhibits release of mediators
Cromolyn: used to prevent attack; not a rescue drug
Leukotriene modifiers: block proinflammatory mediators that promote smooth muscle contraction, vascular leakage, mucous secretion & airway hyperactivity (montelukast, zafirlukast)
Immunosuppressive meds: (methotraxate & cyclosporine) used in chronic, severe asthma
antibiotics not usually indicated*
asthma treatment & management
- EIA can be reduced by meds and warm ups 45-60 minutes prior to exercise; warm up usually consists of 30 second bouts with 2 min rest periods to reduce severity of s&s
- goal is prevention first and minimizing exacerbation second
- routine exercise program has been shown to increase minute ventilation, increase O2 consumption and decrease blood lactate
- objective measure of lung function
- environmental control measures to avoid allergens and irritants and avoidance of triggers
- pharmacologic therapy
- patient education
septic obstructive diseases
chronic bronchitis, cystic fibrosis, bronchiectasis
- purulent sputum & high incidence of pulmonary infection
- hallmark is productive cough with excessive secretion production
PFTs: (similar to non-septic); decrease in expiratory effort with increased TLC, hypercapnia which leads to pulmonary hypertension and cor pulmonale (R heart failure)
pulmonary HTN (high BP in pulmonary arteries and lungs)
high pressure & narrowing of vessels causes R heart enlargement and failure (cor pulmonale)
chronic bronchitis
clinical diagnosis= cough and sputum production for >3 months to >3 years
- smoking is the #1 risk factor as leads to inflammation and airway destruction
- increased size and number of bronchial mucous glands leads to excess airway mucus
- increased glands, plus airway inflammation thickens airway walls
- structural and functional effects on cilia lead to decreased mucociliary clearance
- small airways (<2 mm) develop narrowing, inflammation, fibrosis
chronic bronchitis medications
- often on various antibiotics for bacterial and fungal infection
- short acting beta agonists
- long acting bronchodilators
- inhaled corticosteroids
- smoking cessation drug therapy
- expectorants and mucolytics
- antiprotease treatment (to decrease destruction of elastase by inflammation)
chronic bronchitis treatment
- supplemental O2
- bronchial hygiene
- pulmonary rehab
- early mobilization
- smoking cessation behavioral therapy
- influenza and pneumococcal vaccine
“pink puffer”
descriptive name for COPD pt with predominant emphysema
major symptom: dyspnea appearance: thin, wasted, not cyanotic PO2: low PCO2: normal or low elastic recoil of lungs: low diffusing capacity: low hematocrit: normal cor pumonale: infrequent
“blue bloater”
descriptive term for COPD pt with predominant bronchitis
major symptom: cough and sputum appearance: obese, cyanotic PO2: really low PCO2: normal or high elastic recoil of lung: normal diffusing capacity: normal hematocrit: often high cor pulmonale: common
mucus clearing device
1: flutter mucus clearing device
2: acapella mucus clearance devices (high and low flow)
cystic fibrosis prevalence
=autosomal recessive
- most common and lethal genetic disease in US
- incidence is 1 in 3900 live white births
- more than 10 million americans are symptomless carriers of the gene
what is cystic fibrosis?
- autosomal recessive genetic disease
- defect involves the epithelial cells of the exocrine glands
- defect in chloride ion transfer: Na+ and Cl- transport disturbed, pulling water back into cell making secretions thicker and drier
- lungs: mucociliary clearance mechanism is hindered, secretions settle in lungs & provide good medium for bacteria to grow
- sets up a cycle of inflammation, infection and destruction of tissues
- in other organs the thickened secretions lead to obstruction and malabsorption (GI tract)
- hallmark of CF is that baby tastes “salty”
- chronic disease with median life span ~ 38 years
target organs of cystic fibrosis
sinuses lungs pancreas intestines biliary tracts vas deferens uterine cervix sweat glands
primarily affects the lungs and digestive systems
cystic fibrosis signs and symptoms
- meconium ileus at birth (intestinal obstruction due to overly thick meconium-(should be passed in feces after birth))
- failure to regain birth weight/failure to thrive
- recurrent respiratory infections (PNA)
- chronic productive cough (hemoptysis)
- “salty kiss” (increased Cl)
- pancreatic insufficiency, pancreatitis, DM
- nasal polyps
- GERD
- SOB with excess inspiratory accessory muscle use
- inspiratory crackles and wheezing
- clubbing of nails
- weight loss; nutrient deficit: pancreatic enzymes can’t function properly
- decreased activity tolerance
- osteoporosis
- muscle wasting
- chronic low back pain
- developmental delays
cystic fibrosis medical treatment
- early detection & tx important to start aggressive nutritional support
- aerobic & strengthening exercise
- energy conservation techniques
increased life expectancy due to:
- pancreatic enzyme replacement
- vitamin supplements
- high caloric diets
- airway clearance techniques
- antibiotics and antifungal treatment
- mucolytic & bronchodilator meds
cystic fibrosis treatment complications
- respiratory failure
- R sided heart failure
- severe hemoptysis
- spontaneous pneumothorax (collection of air/gas in the lung causing it to collapse)
cystic fibrosis medications
- glucocorticoid steroids (anti-inflammatory)
- bronchodilators
- antibiotics
- pancreatic enzymes (assist with digestion)
bronchiectasis
- permanent dilation of bronchi due to destruction of muscular and elastic properties of lungs
- usually associated with other pulmonary diseases (CF, emphysema,etc)
CXR: nonspecific
CT: high resolution, gold standard for dx
characteristics of bronchiectasis
- thickening of bronchial walls
- impairment of the mucociliary blanket
- hypersecretion of purulent sputum
2 key causes of bronchiectasis
1: intense chronic inflammation
2: inadequate defense to minimize infection resulting in tissue damage
