Term
| 75% pts with CF diagnosed when |
|
Definition
2 years old
(Autosomal recessive disorder) |
|
|
Term
|
Definition
CFTR gene mutation
Most common mutation=F508 |
|
|
Term
Patho is decreased?
Patho is increased? |
|
Definition
Decreased SECRETION OF CL & H2O
Increased REABSORPTION of Na+ |
|
|
Term
|
Definition
|
|
Term
| Positive sweat chloride test for Dx |
|
Definition
|
|
Term
|
Definition
Pseudomonas
-causes cough, recurrent pneumonia,mucus |
|
|
Term
|
Definition
|
|
Term
|
Definition
-Cipro
-piptazo,aminoglyc,
-ceftazidime,cefepime,
-imipenem,meropenem |
|
|
Term
| Aerosols indicated in who |
|
Definition
| >/=6 yrs & chronis Pseudomonas colonies |
|
|
Term
|
Definition
Tobramycin dry powder or nebulizer
Aztreonam nebulizer
Colistimethate nebulizer |
|
|
Term
| Mucokinetics (for >/=6yrs) |
|
Definition
Hypertonic saline
Dornase Alfa [Pulmozyme]
Albuterol 2 puff po before inhale Tx (opens airways) |
|
|
Term
| Dornase Alfa [Pulmozyme] MOA |
|
Definition
| Digests extracellular DNA sputum from neutrophil breakdown |
|
|
Term
| Sequence of Airway ckearing Tx |
|
Definition
Brochodilator(albuterol)
Hypertionic Saline
Dornase Alfa
Aerosol ABXs |
|
|
Term
| PO Anti inflamatory agents |
|
Definition
-AZITHRO 250-500mg po tiw
-Ibuprofen 20-30mg/kg/dose bid |
|
|
Term
|
Definition
| >/= 6 yrs old & chronic pseudomonas colonization |
|
|
Term
| IBUprofen antiinflamm TX is for |
|
Definition
6-17 yrs old &
FEV >/= 60% predicted |
|
|
Term
|
Definition
| Slow loss of lung function |
|
|
Term
|
Definition
Fatty stools
rectal prolappse Deficient in ADEK |
|
|
Term
|
Definition
Pancreatic Lipase
-Zenpep
-Creon
-Pancreaze
-Pertzye |
|
|
Term
|
Definition
High calorie
High protein
Supplement w/ ADEK Vitamins |
|
|
Term
|
Definition
| 10 yrs old annual OGTT test in CF related DM |
|
|
Term
|
Definition
Insulin basal and bolus combo
-0.5 to 1 unit rapid insulin for q 15g of carbs |
|
|
Term
|
Definition
|
|
Term
|
Definition
>/=2yrs old w/ at least 1 CFTR mutation that is responsive
{IVACAFTOR = CFTR potentiator} |
|
|
Term
| KALYDECO (Ivacaftor) counseling |
|
Definition
-take w/ full meal
-beware of CYP3A4 inhibitors
-Monitor LFTs q 3 months for first year then annually |
|
|
Term
| ORKAMBI (Ivacaftor/Lumacaftor) indicated in |
|
Definition
-F508 homozygous pts
->/=6 years
-take with fatty meal |
|
|
