CysticfibrosisFC

Question 1

Inheritance pattern

Answer 1

Autosomal recessive

Question 2

Chromosomal & mutations causing CF

Answer 2

1. Chromosome 7
2. CFTR mutation

Question 3

CFTR mutation causes what?

Answer 3

Cl will stay in the cell & draw H2O/NA in -> concentrated solution on one side = Dehydration of mucosa

Question 4

1. Gold standard test
2. Value to be diagnostic
3. What age can this be used?

Answer 4

1. Sweat Test
2. > = 60 mEq/L
3. 7 months

Question 5

What if the value is 50mEq/L - What do you do then?

Answer 5

Chromosomal test

Question 6

First indicators to test - Clinical status.

Answer 6

1. Chronic/repeated respiratory conditions
2. Exocrine pancreatic insufficiency
3. Family history
4. Meconium ileus

Question 7

What other areas of the body does CF effect (excluding respiratory system?)

Answer 7

1. Reproductive
2. Exocrine/endocrine
3. GI
4. Pulmonary

Question 8

Reproductive system effects

Answer 8

1. Late maturation - high energy phase
2. Sterility

Question 9

What if a CF+ woman becomes pregnant -
1,2. What should you do and tell her?
3. What form of Birth control?

Answer 9

1. Nutrition/Pulmonary status should be monitored closely.
2. Discuss birth control measures before puberty.
3. Depot injection

Question 10

Exocrine/Endocrine system is focused on what organ?

Answer 10

Pancreas

Question 11

As the patient ages (18yr/old), they will appear __ & if symptomatic will present as ___

Answer 11

insulin deficient, T2DM

Question 12

1. Treatment for Exocrine/Endocrine disorder?
2. Inpatient
3. Outpatient

Answer 12

1. Insulin
2. Humulin R/ Humalog
3. Humulin N, R, Lantus

Question 13

GI system effects

Answer 13

pancreatic deficiency

Question 14

Pancreatic deficiency sx

Answer 14

1. Failure to thrive
2. Steatorrhea
3. Malabsorption

Question 15

Malabsorption of Fats = Decreased absorption of what 4 fat soluble vitamins?

Answer 15

A, D, E, K

Question 16

Treatment of GI problems - combination of all these

Answer 16

1. Nutrition support
2. Vitamin replacement
3. Pancreatic Enzyme replacement

Question 17

Pancreatic enzyme replacement dosing.
1. Infants
2. Everyone else prior to each meal
3. Everyone else prior to each snck

Answer 17

1. 2,000 - 4,000 units lipase / 120ml bottle
2. 1000 units Lipase/kg
3. 500 units Lipase/kg

Question 18

How do you choose Pancreatic Enzyme Replacement?

Answer 18

1. Meet # of lipase needed w/ lowest # of capsules needed.
2. Can choose 1 prod for meal & 1 prod for snack

Question 19

Acute Pulmonary exacerbation
1. What it causes
2. Bug

Answer 19

1. Easier to catch pneumonia
2. P. aeruginosa

Question 20

Acute Pulmonary exacerbation - tx
1. option 1
2. option 2
3. Target peak for Tobramycin
4. Antibiotic duration

Answer 20

1. Aminoglycoside (tobra) + ESP
2. Aminoglycoside (tobra) + Cefepime/Ceftazidime
3. 10-14 mcg/ml
4. 14-21 days

Question 21

Chronic Pulmonary
1. What causes it
2. Gold standard of tx

Answer 21

1. Obstruction of gas exchange in lungs
2. Percussion/Postural drainage

Question 22

Percussion/Postural drainage
1. What are they
2. How many times a day
3. What should you do before one of the CPT sessions? Dose?

Answer 22

1. Chest percussive therapy (CPT)
2. 2-4 x daily
3. Pulmozyme nebulization - 2.5mg QD/BID

Question 23

What if the pt. is >= 6 yr/old + high number of exacerbations in a short period (4-6 months or 6 in 1 year)
1. What do you give & dose

Answer 23

TOBI (tobramycin) nebulization
300 mg BID 28 days -> 28 days off therapy -> repeat for rest of life

Question 24

Other forms of tx for Chronic pulmonary patients to supplement care

Answer 24

1. Long-term NSAID
2. Azithromycin (M,W,F dosing)
3. Ivacaftor - Kalydeco (G551D mutation patient only)