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37 Cards in this Set
- Front
- Back
What is the gold standard diagnostic test for cystic fibrosis?
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The Sweat Test
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_______ mEq/L or higher is diagnostic
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?
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Most CF patients can be diagnosied using the sweat test by _____ of age
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7months
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What alterations in clinical status may indicate cystic fibrosis?
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1)chronic or repeated respiratory conditions
2)exocrine pancreatic insufficiency 3)meconium ileus 4)family history |
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What are the 4 physiologic systems affected by cystic fibrosis?
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1)reproductive
2)exocrine/endocrine 3)gastrointestinal 4)pulmonary |
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Reproductive system effects
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1)late maturation/delayed puberty
2)90% of males sterile 3)60% of females are unable to have children |
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Exocrine/Endocrine
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1)18 years of age they may clinically appear to be insulin deficient (CFDM)
2)presents as if a type2 diabetic |
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Treatment for CFDM
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1)Humulin R or Humalog on SSI when in hospital
2)Split-mixed dosing w/ Humulin N and Humulin R or 1-dose lantus w/ premeal Humalog for outpatient management |
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GI system pathophysiology from cystic fibrosis
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1)increased viscosity of mucus secretions
2)deficiency of pancreatic enzymes to aid in digestion |
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GI system effects
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1)initially may present as meconium ileus or as distal intestinal obstructive syndrome (DIOS) if later in life
2)lifelong complications related to pancreatic deficiency presenting as maldigestion and malnutrition |
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Pancreatic enzyme deficiency leads to _____, protein, and carbohydrate malabsorption
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fat?
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Malabsorption also leads to decreased absorption of 4 fat soluble vitamins which are
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ADEK
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Treatment for GI effects
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1)nutrition
2)vitamin replacement 3)pancreatic enzyme replacement |
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Specific nutrition treatment
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1)foods/energy sources high n calories and easily absorbed
2)Ensure like products PO throughout day or via NG drip at night 3)cyclical TPN if problems persist |
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Vitamin replacement
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1)standard multivitamin BID or have patient take ADEK@QD
2)educated on sxs of fat soluble deficiency |
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Pancreatic replacement therapy
a)how should doses be titrated |
To patient response
1)decrease stools 2)less complains of steatorrhea and bloating 3)weight gain |
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Pancreatic replacement therapy
b)how is dose based? |
1)number of lipase units with corresponding ratio of Protease and Amylase
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Pancreatic replacement therapy
c)what products are preferred |
1)microencapsulated-protect compound from stomach acid degradation
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For INFANTS: Give _______ units lipase per ____mL bottle
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1)2000-4000units
2)120mL |
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For all others: preferred dosing is by weight-
1)Give ____ units lipase per Kg prior to each meal 2)Give ____ units lipase prior to each snacks |
1)?
2)? |
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Pulmonary system effects
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1)thickened mucus in lungs
a)chronic issue: mucus makes gas exchange difficult resulting in hypoxia and COPD-like syndrome; Cor pulmonale (rt sided HF) b)bacteria growth medium |
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Pulmonary system-Acute exacerbation common pathogens
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1)PSEUDOMONAS AERUGINOSA
2)staph aureus 3)burkholderia capacia 4)haemophilus influenza |
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Acute exacerbation treatment
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COMBO TREATMENT
1)Extended-spectrum penicillin (betalactamase inhibition) AND tobramycin 2)3rd or 4th gen cephalosporin (antipseudomonal) AND tobramycin |
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Aminoglycoside dosing in CF patients
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1)INCREASED ability to clear aminoglycosides
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Tobramycin dosing
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1)initial 7.5-9mg/kg/day divided q8h or q12h
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Target peaks for tobramycin dosing should be _____mcg/mL
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?
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What should be monitored closely with tobramycin therapy?
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CPK-dosing interval may need to be as close as q6h
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Duration of antibiotic treatments
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14-21days
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What are the goals of chronic care for the pulmonary system?
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1)minimize obstruction/gas exchange symptomatology while lengthening time between acute exacerbations
2)leads to decreased systemic consequences and lower degrees of airway inflammation/destruction |
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What is the cornerstone of pulmonary therapy?
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percussion and postural drainage
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Prior to ONE OF THE CPT SESSIONS the patient should receive a nebulized dose of __________.
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Pulmozyme (DNase or Dornase Alpha)
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Pulmozyme effects
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1)reduces visosity of CF sputum by breaking down DNA and other proteins in mucus
2)lengthens time between acute exacerbations and improves QOL |
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Pulmozyme dose
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2.5mg QD
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What bacteria is the predominate organism for colonization in CF patients
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Pseudomonas aeruginosa
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When is chronic supressive therapy indicated in CF patients?
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1)4 exacerbations in 6months or 6 in 1year
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What is used for chronic suppressive therapy?
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tobramycin nebulizations (TOBI)
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Dose of tobramycin for chornic suppressive therapy
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300mg BID for 28 days on then 28 days off therapy, then repeat
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