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37 Cards in this Set

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What is the gold standard diagnostic test for cystic fibrosis?
The Sweat Test
_______ mEq/L or higher is diagnostic
?
Most CF patients can be diagnosied using the sweat test by _____ of age
7months
What alterations in clinical status may indicate cystic fibrosis?
1)chronic or repeated respiratory conditions
2)exocrine pancreatic insufficiency
3)meconium ileus
4)family history
What are the 4 physiologic systems affected by cystic fibrosis?
1)reproductive
2)exocrine/endocrine
3)gastrointestinal
4)pulmonary
Reproductive system effects
1)late maturation/delayed puberty
2)90% of males sterile
3)60% of females are unable to have children
Exocrine/Endocrine
1)18 years of age they may clinically appear to be insulin deficient (CFDM)
2)presents as if a type2 diabetic
Treatment for CFDM
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
GI system pathophysiology from cystic fibrosis
1)increased viscosity of mucus secretions
2)deficiency of pancreatic enzymes to aid in digestion
GI system effects
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
Pancreatic enzyme deficiency leads to _____, protein, and carbohydrate malabsorption
fat?
Malabsorption also leads to decreased absorption of 4 fat soluble vitamins which are
ADEK
Treatment for GI effects
1)nutrition
2)vitamin replacement
3)pancreatic enzyme replacement
Specific nutrition treatment
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
Vitamin replacement
1)standard multivitamin BID or have patient take ADEK@QD
2)educated on sxs of fat soluble deficiency
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
Pancreatic replacement therapy
b)how is dose based?
1)number of lipase units with corresponding ratio of Protease and Amylase
Pancreatic replacement therapy
c)what products are preferred
1)microencapsulated-protect compound from stomach acid degradation
For INFANTS: Give _______ units lipase per ____mL bottle
1)2000-4000units
2)120mL
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)?
Pulmonary system effects
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
Pulmonary system-Acute exacerbation common pathogens
1)PSEUDOMONAS AERUGINOSA
2)staph aureus
3)burkholderia capacia
4)haemophilus influenza
Acute exacerbation treatment
COMBO TREATMENT
1)Extended-spectrum penicillin (betalactamase inhibition) AND tobramycin
2)3rd or 4th gen cephalosporin (antipseudomonal) AND tobramycin
Aminoglycoside dosing in CF patients
1)INCREASED ability to clear aminoglycosides
Tobramycin dosing
1)initial 7.5-9mg/kg/day divided q8h or q12h
Target peaks for tobramycin dosing should be _____mcg/mL
?
What should be monitored closely with tobramycin therapy?
CPK-dosing interval may need to be as close as q6h
Duration of antibiotic treatments
14-21days
What are the goals of chronic care for the pulmonary system?
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
What is the cornerstone of pulmonary therapy?
percussion and postural drainage
Prior to ONE OF THE CPT SESSIONS the patient should receive a nebulized dose of __________.
Pulmozyme (DNase or Dornase Alpha)
Pulmozyme effects
1)reduces visosity of CF sputum by breaking down DNA and other proteins in mucus
2)lengthens time between acute exacerbations and improves QOL
Pulmozyme dose
2.5mg QD
What bacteria is the predominate organism for colonization in CF patients
Pseudomonas aeruginosa
When is chronic supressive therapy indicated in CF patients?
1)4 exacerbations in 6months or 6 in 1year
What is used for chronic suppressive therapy?
tobramycin nebulizations (TOBI)
Dose of tobramycin for chornic suppressive therapy
300mg BID for 28 days on then 28 days off therapy, then repeat