cf2

Question 1

what is meconium ileus

Answer 1

blockage in inestines present at birth in many CF patients

Question 2

what is Distal Intestinal Obstruction Syndrome (DIOS)

Answer 2

Thick intestinal secretions that is painful

Question 3

How is Distal Intestinal Obstruction Syndrome (DIOS) treated

Answer 3

Golytely, Miralax, hydration, chronic laxatives

Question 4

what should be monitored for GI symptoms of CF

Answer 4

1. symptoms at every visit
2. BMI
3. oral glucose tolerance test
4. annual liver enzymes
5. fat soluble vitamin levels once a year

Question 5

what are some other prevalent complicatons in CF other than pulmonary and GI problems

Answer 5

1. CF-related bone disease
2. depression
3. pain

Question 6

what is the screening for CF-related bone disease? how often is it done?

Answer 6

DEXA

every 5 years starting at age 10

Question 7

what type of treatment do all CF patients receive 2-5 times daily

Answer 7

chest physiotherapy (CPT)

Question 8

if a patient has a hyperactive airway, what should be used daily in CF patients

Answer 8

albuterol (beta agonist)

Question 9

name a mucolytic agent used to help airway clearance

Answer 9

dornase alfa (Pulmozyme)

Question 10

what is dornase alfas MOA

Answer 10

recombinate human deoxyribonuclease; cleaves DNA to help thin out mucus

Question 11

how is dornase alfa given

Answer 11

nebulization (over about 10-15 minutes)

Question 12

what are common side effects of dornase alfa

Answer 12

hoarseness, cough, rash

Question 13

when should dornase alfa be recommended

Answer 13

1. all patients older than 6 years old

2. symptomatic younger patients

Question 14

what else is used to help with airway clearance other than dornase alfa?

Answer 14

hypertonic saline (7%)

Question 15

what must be given before hypertonic saline can be given

Answer 15

bronchodilator (albuterol) due to bronchial irritation caused by saline

Question 16

what is geiven before and after first use of hypertonic saline nebulization for CF patients? why?

Answer 16

pulmonary function tests

it is very irritating and if PFT drops by 15%; not a candidate for the treatment

Question 17

Why is azithromycin used in CF patients

Answer 17

for its anti-inflammatory properties, not its antibiotic properties

Question 18

what side effect of azithromycin is considered a good thing

Answer 18

weight gain

Question 19

what are the indications for azithromycin in CF patients

Answer 19

1. CF patients older than 6 infected with pseudomonas (also those without P. aeruginosa, but not as good of evidence)

Question 20

what is a negative side effect of azithromycin in CF patients

Answer 20

increases clindamycin resistant S. aureus