36 terms

Cystic Fibrosis RSCC RT Pathology I

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Classical CF Triad
Pancreatic exocrine insufficiency, pulmonary disease, elevated sweat chloride concentration
Avg life expectancy
30 yrs
Common Bacteria
S. aureus, hemophilia influenza, P. aeruginosa
Bronchectasis
Chronic dilation and distortion of one or more bronchi
Cause of bronchiectasis
response to extensive inflammation and destruction of bronchial tree cartilage, blood vessels, elastic tissue, and smooth muscle
mucociliary clearing mechanism
impaired due to bronchial wall destruction
3 types of bronchiectasis
varicose, cylindrical, saccular
varicose bronchiectasis
bronchi are dilated and constructed in an irregular fashion similar to varicose veins
cylindrical bronchiectasis
bronchi are dilated and have regular outlines similar to a tube
saccular bronchiectasis
bronchi progressively increase in diameter until ending in large cyst like sac in lung parenchyma
most damaging bronchietasis
saccular
CF and bronchiectasis etiology
because of problems associated with increased sputum and bronchial obstruction leading to infection and causing increase in inflammation leading to bronchiectasis
diagnosis of bronchiectasis
diagnosed by bronchogram: injection of opaque contrast materieal into trachobronchial tree
sweat glands
may secrete up to 4 times the normal amount of NaCl
upper airway
recurrent sinusitis
intestines
Meconium ileus: obstruction of small intestines of newborn caused by thick, dry, tenacious impaction
Autosome chromosomes
all chromosomes besides sex chromosomes
gene responsible for CF
chromosome #7
appearance of recessive trait
both genes must be identical
Mendelian Pattern
autosomal recessive inheritance pattern
homozygous
must have recieved a CF gene from both parents
signs of chronic hypoxemia
polycythemia, digital clubbing
lab eval for polycythemia
complete cbc to look for increased RBC count
barrel chest
increased A-P diameter of chest
standard test for CF
sweat chloride test
most significant health threat to CF
recurrent respiratory infections
CF treatment
O2 therapy, mucolytics, bronchial hygiene therapy, CPT, postural drainage, pickle, PEP therapy, vest, antibiotics, bronchodilator therapy
Mucomyst
acetylcysteine, breaks down disulfide bounds in mucous
Pulmozyme
Dornase alfa, breaks up DNA debris in sputum. Lowers viscocity
Tobramycin
Antibiotic, given in seperate nebulizer after bronchodilator
ventolin
short acting beta 2 agonist, bronchodilator, sympathomimetic
foradil
Formoterol - Long acting adrenergic bronchodilator
flovent
fluticasone, corticosteroid
Spiriva
Tiotropium Bromide
Anticholinergic
Respiratory Antimuscarinic
atrovent
Ipratropium Bromide - anticholinergic parasympatholytic bronchodilator
Extended home care
education, hydration, O2 use, aerosol treatments, cough training, brochial hygiene maneuvers, watch sputum, pursedlip breathing and diapragmatic breathing