1What is cystic fibrosis?
disease of exocrine gland function that involves multiple organ systems but chiefly results in chronic respiratory infections, pancreatic enzyme insufficiency and associated complications of untreated patients
2What are the primary goals of CF treatment?
maintaining lung function as near normal as possible by controlling respiratory infection and clearning airways of musus
administering nutritional therapy to maintain aqequate growth
managing complications
3What systems are impacted by cystic fibrosis?
lungs
sinuses
intestines
pancreas
liver
urogenital
4What is conventional chest physiotherapy?
postural drainage with percussion and/or clapping
5What are other alternative airway clearance methods?
high frequency chest compression
airway oscillating devices
positive expiratory pressure devices
6What are the 3 mucolytics used for cystic fibrosis?
hypertonic saline
dornase alfa
N-acetyl cysteine
7What is the MOA for donase alfa?
cleaves and depolymerizes extracellular DNA and seperates DNA from proteins which allows endogenous proteolytic enzymes to break down the proteins thus decreasing viscoelasticity and surface tension of prurulent sputum
8What is the MOA of hypertonic saline?
increases sodium in airways and pulls water to help thin out mucus
9What is the MOA of N-acetyl cysteine?
reduces the disulfid bonds of mucoproteins
10What are the AEs of dornase alfa and hypertonic saline?
chest pain
pharyngitis
rash
voice alteration
rhinits
fever
11Aside from mucolytics, CFTR modulators and pancrelipase what other medications can be given for CF patients?
high dose ibuprofen
SABA and LABA
NaCl tablets
12When do you want to avoid high dose ibuprofen?
in patients with hx of hemoptysis
13What are examples of pancrelipase?
creon
pancreaze
ultrese
zenpep
14What do pancrelipase medications combine and what do they assist with?
contains lipase, amylase and protease
assists in digestion protein, starch and fat
15What are some AE of pancrelipase?
abdominal cramp
abdominal pain
h/a
16What vitamins should be given to patients with CF and what are they essential for?
AEDK
antioxidant effects
coenzymes for biological pathways
neurodevelpment
bone development
coagulation
17What is the MOA for CFTR modulators?
improve CFTR protein function by targeting underlying protein defects
18What do the CFTR protein potentiators do?
increase CFTR Cl- channel gate opnening and conductance and improve Cl- transport
19What do the CFTR protein correctors do?
improve:
protein folding
protein stability
transport of functional CFTR protein to the cell surface
20What is good about CFTR modulcators?
improve FEV1
help patients gain weight
reduce exacerbations
decrease sweat CI-concentration
21What do you treat MSSA with?
nafcillin or oxacillin
22What do you treat MRSA with?
vancomycine or linezolid
23What do you treat pseudomonoas aeruginosa with in outpatient?
floroquinolones like ciproflaxacin
24What are you treating pseudomonas aeruginoas with inpatient?
select two of the following:
antipseudomonal beta-lactam (ceftazidime or meropenem)
floroquinolones (cipro)
aminoglycosides (tobramycin or amikacin)
25CF patients benefit from
a regular airway clearance routine
26What is an airway clearance seesion generally begin with?
SABA to open the airways followed by mucolytics
27How do you treat children with TB?
isoniazid and rifampin for 6 months plus pyrazinamide for the first 2 months
28Can treatment be longer or shorter in children?
longer
29Pregnant women with TB are treated with
isoniazid
rifampin
ethambutol
30Pyrazinamide is reserved for
women with suspected multidrug-resistant TB
31What is the intensive pahse of TB treatment?
2 months rifampin plus isoniazide, pyrazinamide and ethambutol
32What is the continuation phase of TB treatment?
4 months of rifampin plus isoniazid
33What is the MOA for rifampin?
inhibits DNA-dependent RNA polymerase activity in bacterial cells
34What is the patient education that should be provided with rifampin?
should take 1-2 hours after meal with full glass of water
may produce a discoloration of teeth, urine, sweat, sputum and tears
35What is the drug of choice for use in preventative therapy?
isoniazid
36What is the MOA of isoniazid (INH)?
bactericidial- may inhibt the synthesis of mycolic acid, resulting in disruption of the bacterial cell wall
37What are the serious AE of isoniazid?
peripheral neuropathy
n/v
stomach pain
loss of appetitie
thrombocytopenia
elevated ALT/AST
optic neuritis
38What is coadministered to prevent peripheral neuropathy with isoniazid?
pyridoxine 25-50 mg qd
39What are the serious AE of rifampin?
elevated LFTs
rash
epigastric distress
anorexia
n/v/d
cramps
pseudomemraneous colitis
pancreatitis
40What may rifampin decrease the effectiveness of?
OCP
41What is a caution with rifampin?
hx of alcoholism
42What should not be done while taking isoniazid?
drinking
43What PE should be provided for patients taking isoniazid?
s/s of hepatitis
big box warning is that severe and fatal hepatits may occur within the first 3 months of tx and many months after tx
44What are the CI of pyrazinamide?
severe hepatic damage
acute gout
photosensitivity
45What are the AE of pyrazinamide?
malaise
n/v
anorexia
arthralgia
myalgia
46What is the MOA of ethambutol?
impairs cell metabolism by inhibiting the synthesis of one or more metabolites which in turn causes cell death
47What should you not take with ethambutol?
aluminum containing antacids- wait four hours
48What are the DDI with ethambutol?
BCG live vaccine
49What are the AE of ethambutol?
optic neuritis
n/v
gout/hyperuricemia
abdominal pain
anorexia
fever
h/a
lft abnormalities
pruritus
rash
50What should be done monthly while taking ethambutol?
eye exams
51What are the second line drugs with TB infections?
ethionamide
cycloserine
para-aminosalicylic acid
aminoglycoside
fluoroquinolones
52What are the AE of streptomycin (an aminoglycoside)?
nephrotoxicity
ototoxicity
vestibulotoxicity
53What are the AE of amikacin (an aminoglycoside)?
nephrotoxicity and ototoxicity
54What are PDE5 inihibitors DDI?
nitrates
55Riociguat is a?
soluble guanylate cyclase stimulator
56Ambrisentan, bosentan and macitentan are?
endothelin receptor antagonists
57What is the MOA for ERAs?
help prevent blood vessels from narrowing
58What are the AE of ERAs?
fluid retention
elevated LFTs
59When should you avoid tadalafil?
patients with severe liver or kidney dysfunction
60What is the MOA for the PDE5 inhibitors?
allow lungs to produce more of their own natural vasodilators
61What is the MOA of sGC stimulators?
increase the interaction of sGC with nitric oxide to help the blood vessels in the lungs relax
62What is an AE of riociguat?
systemic hypotension
63When should you avoid riociguat?
patients with severe liver or kidney dysfunction
64What is a selective IP receptor agonist?
selexipag
65What is the MOA of selexipag?
target and activate prostacyclin receptor helping the blood vessels in the lungs relax
66Treprostinil is a?
prostcyclin analog
67What is the MOA of prostacyclin analogs?
allow the blood vessels in the lungs to relax
68What is an AE of treprostinil?
low systemic blood pressure
69What is a caution with treprostinil?
abrupt withdrawal/large dose reduction may worsen symptoms of PAH
70What is a pE for prostacyclin analogs?
take with food
71What are the two parenteral prostacyclin analogs?
treprostinil
epoprostenol
72Which PAH class are the parental prostacyclin analogs used for?
class III-IV
73What is the inhaled prostacyclin analog?
tyvaso
iliprost
74What is common AE of iliprost?
orthostatic hypotension
bronchospasm
75What are the first line therapies for PH?
CCB
digoxin
diuretics
warfarin
oxygen
76What CCB can be used to treat PAH?
nifedipine
diltiazem
amlodipine