class 5 cystic fibrosis

Question 1

how is CF inherited

Answer 1

autosomal recessive

Question 2

what causes CF

Answer 2

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

Question 3

how is CF diagnoses

Answer 3

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

Question 4

what happens in the lungs with CF

Answer 4

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

Question 5

what happens in the pancreas and GI with CF

Answer 5

-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

Question 6

what happens with the liver with CF

Answer 6

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

Question 7

infection control for CF patients

Answer 7

-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

Question 8

objectives of CF care

Answer 8

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

Question 9

treating CF with antibiotics

Answer 9

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

Question 10

order of medications given

Answer 10

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

Question 11

what is airway clearance with CF

Answer 11

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

Question 12

nutritional therapy for cf patients

Answer 12

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

Question 13

what is pancreatic enzyme replacement therapy (PERT)

Answer 13

-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

Question 14

Nutrition supplementation with Cf

Answer 14

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

Question 15

Fat soluble vitamins with CF

Answer 15

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

Question 16

Calcium and CF

Answer 16

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

Question 17

CFRD (CF related diabetes)

Answer 17

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

Question 18

Test for CFRD

Answer 18

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

Question 19

HEMT (highly effective modulator treatment)

Answer 19

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

Question 20

Lung transplants and CF

Answer 20

of transplants decreasing due to medication

Life expectancy increasing