CF robles
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- 3 imp goals; NO CURE
- maintain lung fxn, prevent and manage pulmonary infection, optimize nutritional status for growth
- panc enz point
- do not sub generics
- CF orgs initial infection
- H flu, SA, P aeruginosa
- mucolytic: hypertonic saline
- (3, 6, 7, 10%) 7% 4 ml inhaled BID
- cholelithiasis tx
- ursodiol 15-30 mg/kg/d: reduce bile viscosity and prevent obstruction
- anti-inflam CS
- prednisone/prednisolone; use if inflam complications; take with food
- cacute CF tx
- anti-infectives and non pharm and B agonist
- TOBI
- refrigerate; 28 d RT
- max lipase titrate
- 2500
- anti-inflam
- azithromycin (zithromax); for sx PA > 6 y/o
- anti-inflam ibuprofen
- for lung inflammation; take with food; 5-13 years
- panc sxs
- 90%: failure to thrive, weight loss, GI sxs (steatorrhea etc)
- ABX
- PO for mild, IV for sev exacerbation, resist org, PO failure; nebulized if resist PO org
- long term pulm fxn improvement with CSs
- if LT treat QOD
- acute exacerbation tx
- infectious therapy
- most courses of ABX
- 2-3 weeks
- mucolytic DNAse (dornase) MOA
- digests EC DNA and so reduces viscosity of mucus; do not dilute/mix
- TOBI CF peak
- 10-14
- breakfast
- enz, multivit, ABX
- snack dose of lipase
- half
- mucolytics: N-acetylcysteine (mucomyst)
- >6; 1-2 mL 20% soln INH BID-TID
- PA
- colonization late in childhoon; use combo tx with dif MOAa
- Colistimethate
- do NOT use premixed products!!
- colistimethate
- use immediately
- CF gent
- 2.5-3.3 mg/kg q 6-8 hrs
- bronchodilators: B2 agonists
- albuterol, levalbuterol nebules
- ADR of B2 agonists
- hypokalemia, tachycardia
- TOBI nonCF peak
- 6-10
- IV dif PK
- if have inc V and CL; dec abs; if higher peak/tr goals, larger doses or shorter intervals
- mucolytics
- dornase alpha (pulmozyme); reduce infection frequency and improve lung function
- sxs
- basilar crackles, expiratory wheezing, increased chest diameter, digital clubbing (adv)
- use of B2 agonists
- dilate airways BEFORE other CF tx and possible sputum clearance
- nonCF gent
- 2.5mg/kg q 8 hrs
- pm
- bagonist; advair
- mucolytic considerations
- $$, >5, refrigerate
- chronic mgmnt of CF
- anti-inflammatory, bronchodilators, mucolytics
- ABX duration
- 10-14 days
- malnutrition vit tx
- fat sol ADEK, multivit
- H flu
- most common at ages 2-5
- saline
- use bronchodilator first
- SA
- most common 6-17
- TOBI
- do NOT mix with other products; will precipicate; > 6 y/o
- lipase dose if < 4
- 1000u/kg/meal
- am and pm
- beta agonist; advair
- TOBI
- INH
- am
- neb meds, TOBI, hypertonic saline, vest, advair, bagonist
- sxs of exacerbation
- SHB, inc freq cough, chest congestion, change in sputum, fever
- lipase dose if > 4
- 500 u/kg/meal
- pilocarpin iontophoresis
- sweat test > 60/70 is positive
- inhaled antipseudo
- colistimethate, TOBI
- thru day
- enzs with food
- treatment
- B agonist, acute and chronic; breathing methods, chest tx with percussion, chest oscillation, anti-infectives
- chronic mgmnt: nut/GI
- panc replacement and high calorie diet
- CF orgs late infection
- burk. cepacia, stenotro maltophilia, acine. xylosoxidams: mult ABX resist
- panc enzs
- indiv dose on lipase based on ingested fat of weight
- colistimethate
- nebulizer
- liver disease in CF
- cholelithiasis: second leading COD