Cystic Fibrosis Flashcards
To get cystic fibrosis what type of genetics must you have?
2 recessive copies
In cystic fibrosis what gene is affected?
CFTR- version F508del
What does the CFTR gene do?
regulates how Cl ions cross= if issue mucus and secretions can’t get cleared in airways
If I was class 1 CF what does this mean?
NO CFTR gene
If I was class 4 CF what does this mean?
impair function of CFTR
What race gets more CF?
White
Which sex gets infertility in CF?
males
If you have thick mucus that can’t get cleared what may happen as a complication?
infections/inflammation
What bacteria are common in lungs that may cause infection if given the chance?
P aeruginosa
Staph
H influenzae
S maltophilia
B cepacian
How can we track pulmonary function in CF?
PFTs
If mucus becomes an issues in gi system what complications can there be?
GERD, obstruction, SIBO, pancreatic insufficiency, less fat vitamins
What are the fat vitamins?
ADEK
When does pancreatic insufficiency present in CF?
at birth
Consequences of pancreatic insufficiency?
can’t absorb lipids and some vitamins= osteoporosis, CF related diabetes, malnutrition
What does diabetes due to CF happen?
unknown but maybe pancreatic scarring
How does CF impact male fertility?
no or abnormal vas deferens
Can females get infertility? How?
thick cervical mucus can act as a spermicide
What other complication of CF can happen? Hint on face.
nasal polyps
If exacerbation or worsening of symptoms what must you always check?
ADHERENCE
What is the administration order of inhaled therapies?
bronchodilator
Saline
dornase alfa
chest physio
inhaled antibiotics
inhaled CS
What do all inhaled therapies do for the patient?
improve FEV1, QOL and decrease chances of exacerbation
What bronchodilators do we use?
salbutamol, terbutaline, ipratropium
Why are bronchodilators used in CF?
decrease risk of bronchospasm with other drugs
Why use saline for CF?
mucolytic
How do administer saline?
nebulizer
What strength of saline?
7% if not tolerated 3 %
s/e of saline?
cough, sore throat, bronchospasm
How does dornase alfa work?
deoxyribonuclease that cleaves DNA to lower viscosity and inflammation
Can Dornase alfa and saline be combined in nebulizer?
NO
Regimen of inhaled antibiotics? Why is it done this way?
28 days on then 28 days off
less resistance
When do we use inhaled antibiotics?
if aeruginosa on culturw
Antibiotics used in CF?
levofloxacin, tobramycin, aztreonam, colistimethate
What antibiotic doesn’t HAVE to be nebulizer?
tobramycin
What form of antibiotics are used?
IV solution can be nebulized
Since patients have pancreatic insufficiency what must we give?
pancreatic enzymes= lipase
Rae different pancreatic enzyme brands interchangeable?
NO
S/e of pancreatic enzymes?
bloating, dyspepsia, steatorrhea (fatty stool)
How can we help with fatty stools?
MCT oil
What product has all the vitamins needed for CF?
MVW complete formulation
Who can get the MVW formulation?
special access only
When should CFTR modulators be started?
at youngest age possible
How does trikafta work?
fix CFTR protein= opens channel and holds it open for chloride to move
Which drugs in trikafta are the corrector and potentiator?
corrector= elexacftor, tezacaftor
potentiator= ivacaftor
Special admin instructions with trikafta?
with fat food
S/e of trikafta?
LFTs, psych changes, cataracts
DI with trikafta?
3A4
Monitoring of trikafta?
LFTS and eye exam
efficacy of trikafta?
increases FEV1 alot by 8 weeks, sustains it, may lower other therapies
How to treat DM if caused by CF?
INSULIN
When would we give azithromycin for CF?
if staph and H influenzae
Is azithromycin used for prophylaxis of infection?
NO but maybe for antiinflammatory
How should a CF diet be?
high calorie and protein
How may ibuprofen work for CF?
at high doses it may slow lung loss of function
If using ibuprofen for CF what monitoring do we need?
plasma []
If in a CF exacerbation what will a patient get as therapy?
> 2 weeks of IV antibiotics
If starting IV antibiotics what changes happen to therapy?
STOP inhaled antibiotics during this time
What antibiotics need TDM?
Aminoglycosides
If we do not have a culture what is empiric therapy for exacerbation and why?
pip/taz or ceftazidime AND tobramycin
DUE to coverage of most pathogens and double aeurginosa coverage
How do we dose antibiotics for CF?
USE GUIDE B/C DIFF PK
How do PK change in CF?
absorption may be delayed due to less motility and pancreas
distribution change due to leaner and hypo albumin
increased free drug= more clearance
What other CFTR modulators are there?
Kalydeco (only potentiator
orkambi (Lumacaftor +potentiator)
symdeko (teza+ potentiator)
What type of cough does CF patients have?
productive with sputum and blood
Which CF drugs have risk of bronchospasm?
hypertonic saline and antibiotics
What programs are there for help with CF?
EDS
Special support
SAIL - CF program
