class 5 cystic fibrosis Flashcards

jan 23

1
Q

how is CF inherited

A

autosomal recessive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what causes CF

A

a deletion on chromosome 7
this causes a mutation in the cystic fibrosis transmembrane conductance regulator protein (CFTR) which transports chloride out of the cells do no sodium or H2O getting thru too

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

how is CF diagnoses

A

-newborn screening
-gene testing
-chloride sweat test (>60 positive, <30 negative)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what happens in the lungs with CF

A

-increased secretions lead to obstruction, leading to repeated infections, bronchiectasis, and decline in lung function, causing respiratory failure
-compromised mucociliary and cough clearance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what happens in the pancreas and GI with CF

A

-pancreatic ducts are obstructed by thick secretions, leading to decreased excretion of enzymes into the intestine= dec breakdown of fat and protein
-vitamin ADEK deficiency
-pancreas is autodigested and replaced by scar tissue
-can develop CF-related diabetes
-inc risk for celiac disease & colon cancer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what happens with the liver with CF

A

-irritation & inflammation of bile ducts
-liver cirrhosis= usually presents earlier in life
-hepatomegaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

infection control for CF patients

A

-patient-to-patient transmission is an issue in this population
-standard precautions, transmission-based precautions including contact and droplet
-hand & equipment hygiene
-inc risk for infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

objectives of CF care

A

-focus CF care on lung function, nutritional status, and CFRD
-emphasize early detection for tx
-provide education

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

treating CF with antibiotics

A

-tx aggressively to prevent lung decline
-oral/home IV/ inpatient
-with chronic therapy
-azithromycin usually used as chronic abx
-exercise
-antibiotic resistance prevalent

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

order of medications given

A

1.bronchodilator
2.mucolytic
3.airway clearance (PEP/percussion/huff and couch)
4.inhaled antibiotics (need clean airway)
5.inhaled antiinflammatories (need clean airway)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what is airway clearance with CF

A

-daily/multiple times daily
-enhances clearance of mucus after puffer/neb
-postural drainage & percussion
-positive expiratory pressure therapy
-deep coughing
-high-frequency chest wall

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

nutritional therapy for cf patients

A

high fat and protein diet
-pancreatic enzyme replacement (PERT)
-nurtitional supplements
-vitamin & mineral supplements
-enteral feeds
-BMI should be 22-23

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what is pancreatic enzyme replacement therapy (PERT)

A

-needed to break down fat, CHO, proteins
-different enzymes break down different nutrients
-takes 45-60min to work, needed before meals/enteral feeds
-lipase, proteases, amylases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Nutrition supplementation with Cf

A

Used if struggling to gain weight
Oral protein drinks(boost)
Ng and peg tube feeds
Enzymes given before tube feeds too

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Fat soluble vitamins with CF

A

-lvls measured annually
-vit A D E K need fat for absorption
-taken at mean time (fat)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Calcium and CF

A

Risk for osteoporosis
-supplement 1500mg/day
Maintain vit D >75mmol/L

17
Q

CFRD (CF related diabetes)

A

-main cause is insulin deficiency
-chest infections/steroids may cause glucose intolerance when body cannot control BG
-most require insulin

18
Q

Test for CFRD

A

75g OGTT
definitive test
Screened annually
Gluc Bid for first 72h after admission

19
Q

HEMT (highly effective modulator treatment)

A

Kalydeco (ivacaftor), orkambi (lumacaftor/ivacaftor), symdeko (tezacaftor/ivacaftor) trikafta (elecaftor/tezacaftor/ivacaftor)
-changes defective protein & thins mucus
-taken daily
-very expensive without insurance

20
Q

Lung transplants and CF

A

of transplants decreasing due to medication

Life expectancy increasing