Cystic Fibrosis Flashcards by Carter Berg

To get cystic fibrosis what type of genetics must you have?

2 recessive copies

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In cystic fibrosis what gene is affected?

CFTR- version F508del

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What does the CFTR gene do?

regulates how Cl ions cross= if issue mucus and secretions can’t get cleared in airways

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If I was class 1 CF what does this mean?

NO CFTR gene

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If I was class 4 CF what does this mean?

impair function of CFTR

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What race gets more CF?

White

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Which sex gets infertility in CF?

males

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If you have thick mucus that can’t get cleared what may happen as a complication?

infections/inflammation

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What bacteria are common in lungs that may cause infection if given the chance?

P aeruginosa
Staph
H influenzae
S maltophilia
B cepacian

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How can we track pulmonary function in CF?

PFTs

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If mucus becomes an issues in gi system what complications can there be?

GERD, obstruction, SIBO, pancreatic insufficiency, less fat vitamins

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What are the fat vitamins?

ADEK

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When does pancreatic insufficiency present in CF?

at birth

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Consequences of pancreatic insufficiency?

can’t absorb lipids and some vitamins= osteoporosis, CF related diabetes, malnutrition

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What does diabetes due to CF happen?

unknown but maybe pancreatic scarring

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How does CF impact male fertility?

no or abnormal vas deferens

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Can females get infertility? How?

thick cervical mucus can act as a spermicide

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What other complication of CF can happen? Hint on face.

nasal polyps

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If exacerbation or worsening of symptoms what must you always check?

ADHERENCE

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What is the administration order of inhaled therapies?

bronchodilator
Saline
dornase alfa
chest physio
inhaled antibiotics
inhaled CS

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What do all inhaled therapies do for the patient?

improve FEV1, QOL and decrease chances of exacerbation

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What bronchodilators do we use?

salbutamol, terbutaline, ipratropium

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Why are bronchodilators used in CF?

decrease risk of bronchospasm with other drugs

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Why use saline for CF?

mucolytic

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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?

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?

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