bronchodilator, hypertonic saline, Pulmozyme, anti-pseudomonal ABs (if indicated)
What is the order of therapy for CF? (4)
heel prick at birth, genetic testing, sweat test
How do we diagnose CF? (3)
>50% for age
What is the pediatric weight goal?
>=23; >=22
Goal BMI for Men?
Goal BMI for women?
test dose in clinic; can cause airway hyper-reactivity
When prescribing Hypertonic saline, what must be done prior?
Creon, zenpep, pertzye
What are the PERTs? (3)
lipase units per kg
How do we dose PERTs?
constipation; colonic stricture (rare)
What can occur when PERTs are dosed too high? (2)
Pertzye; acid suppression therapy
If a patient is still having malabsorption, what else can we consider adding?
twice daily with fat-containing meal + enzymes
How do we dose CFTR modulators?
lung function; weight
CFTR modulators can improve ________ & increase ________.
chest tightness, increased intra-ocular pressure, liver dysfunction
What are AE's of CFTR modulators? (3)
annual eye exam (patients <18), LFTs
What monitoring is done for CFTR modulators? (2)
Tobramycin, aztreonam
What are the treatment options for Pseudomonas? (2)
tobramycin
______ is the only AB approved for pseudomonas in CF.
cayston
aztreonam
bethkis, kitabis
Tobramycin Nebulizer (2)
Tobi Podhaler
Tobramycin DPI =
tobramycin nebulizer
_________ provides a more rapid inhalation.
can't be taken with other CF therapies, fastest neb
What is unique about Aztreonam? (2)
>=60
What chloride levels indicate a CF diagnosis?
30-59
What chloride levels indicate further testing required?
A,D,E,K
What are the fat-soluble vitamins? (4)