Cystic Fibrosis 2

Question 1

what patient population with CF do you use high dose ibuprofen in?

Answer 1

patients that are under 18 year old with FEV1 > 60%

Patients over 18 years old – Evidence insufficient to recommend for or against chronic use

Question 2

The effect of high dose ibuprofen is a lower decline in which systems function?

Answer 2

pulmonary

Question 3

benefits of high dose ibuprofen exceed the risk of what

Answer 3

GI complications and. nephrotoxicity

Question 4

high dose ibuprofen allows for patients to maintain weight and leads to less what?

Answer 4

hospital admission

Question 5

for those taking high dose ibuprofen what must be monitored with blood draws?

Answer 5

if the patient is at a specific therapeutic level of ibuprofen (pharmacokinetic monitoring)
Target 50-100 μg/ml

Question 6

one MOI for Anti-inflammatory Therapy-Ibuprofen is to reversibly inhibits cyclooxygenase-1 and 2 (COX-1 and 2) enzymes, what does his lead to?

Answer 6

decreased formation of prostaglandin precursors

it is an antipyretic, analgesic, and has anti-inflammatory properties

Question 7

other mechanisms for Anti-inflammatory Therapy-Ibuprofen are?

Answer 7

inhibiting chemotaxis
altering lymphocyte activity
inhibiting neutrophil aggregation/activation
decreasing proinflammatory cytokine levels

Question 8

Anti-inflammatory Therapy-Ibuprofen ADEs

Answer 8

edema, headache, fluid retention, epigastric pain, gi bleed, tinnitus

Question 9

Lumacaftor is usually used in combination with what drug?

Answer 9

Ivacaftor

Question 10

Ivacaftor MOI

Answer 10

Improves the gating abnormality, effects G551D mutation

Question 11

Pancreatic Enzyme Supplementation ADEs

Answer 11

cause ulcers in oral mucosa after prolonged contact

Question 12

Pancreatic Enzyme Supplementation should be administered with

Answer 12

food, and mouth should be rinded after administration

Question 13

Excessive doses of pancreatic enzymes can cause what?

Answer 13

fibrosing colonopathy, characterized by inflammation and strictures

risk reduced by limiting dose

Question 14

Pancreatic Enzyme
Replacement Therapy
Dose is calculated using which units?

Answer 14

Lipase units

Question 15

______ insufficiency affects up to 85% of patients in their lifetime

Answer 15

pancreatic

Question 16

_______ lipase units per kilogram (kg) of body weight per meal
or ______ units per kg per day

Answer 16

500 to 2,500 lipase units per kilogram (kg) of body weight per meal
or 10,000 units per kg per day

Question 17

which disease is 6 to 8 times more common in CF children in whose without CF

Answer 17

Gastroesophageal Reflux Disease

Question 18

Omeprazole is which type of drug?

Answer 18

a proton pump inhibitor
that act by irreversibly blocking the hydrogen/potassium adenosine triphosphatase enzyme system

also called H+/K+ ATPase, or more commonly, the gastric proton pump

Question 19

Omeprazole ADEs

Answer 19

acid regurgitation, constipation, back pain, weakness, cough

Question 20

People with 2 copies of which mutation represent the largest group of people with CF?

Answer 20

F508del mutation

Question 21

Patients treated with lumacaftor/ivacaftor experienced statistically significant improvements in which function?

Answer 21

lung function

Question 22

patients with CF taking Lumacaftor/Ivacaftor also experienced what two benefits?

Answer 22

reductions in pulmonary exacerbations and improvements in body mass index

Question 23

nasopharyngitis is an ADE of which drug in more than 10% of patients?

Answer 23

lumacaftor/Ivacaftor

Question 24

ADEs experienced by 1 to 10% of patients taking lumacaftor/Ivacaftor?

Answer 24

fatigue, menstrual disease, Increased creatine phosphokinase, Upper respiratory tract infection, rhinorrhea

Question 25

G542X mutation

Answer 25

no synthesis of CFTR

Question 26

F508del mutation

Answer 26

reduced trafficking of CFTR

CFTR is misprocessed in ER

Question 27

G551D mutation

Answer 27

reduced gating of CFTR

unstable at membrane

Question 28

he CFTR gene is mutated, this function is

Answer 28

impaired and ions cannot be transported across membrane

Question 29

in when ions cannot be transported across membrane what are the results

Answer 29

higher electrolyte concentration inside the cell and water stays there, causing mucus to get thicker can block channels of the lung, Leads to Obstruction, Infection, and Inflammation

Question 30

Normally the CFTR’s function is to what?

Answer 30

to transport chloride ions from the inside of the cell to the outside, creates negative charge on the outside of the cell allowing positively charged sodium ions to follow charge out and water follows this makes mucus thinner

Question 31

Chromosome abnormality in CF

Answer 31

on chromosome 7 there are over 1750 mutations in the CFTR gene
accounts for 88% of the disease-causing alleles

Question 32

CFTR gene mutations result in various pathologies such as

Answer 32

primary lung disease to minor gastrointestinal (GI) involvement

Question 33

which class of mutations is most common in caucasians

Answer 33

class III , G551D, getting mutation

Question 34

Ivacaftor major interactions

Answer 34

Substrate of CYP3A4

Question 35

major Ivacaftor ADEs

Answer 35

headache, abdominal pain, nasopharngitis, hyperglycemia, Transaminases increased, arthralgia

Question 36

Tezacaftor/Ivacaftor should be taken with

Answer 36

fat containing food

Question 37

reduce Tezacaftor/Ivacaftor dose in those with

Answer 37

• moderate and severe hepatic impairment

- or when co-administered with drugs that are moderate or strong CYP3A inhibitors

Question 38

what must be monitored in patients taking Tezacaftor/Ivacaftor

Answer 38

ALT and AST (every 3 months for the first year)

also monitor for any signs of cataract

Question 39

liver disease ADEs

Answer 39

Alopecia, leukopenia, thrombocytopenia, and serum creatinine increased