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Flashcards in CysticfibrosisFC Deck (24)
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1
Q

Inheritance pattern

A

Autosomal recessive

2
Q

Chromosomal & mutations causing CF

A
  1. Chromosome 7
  2. CFTR mutation
3
Q

CFTR mutation causes what?

A

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

4
Q
  1. Gold standard test
  2. Value to be diagnostic
  3. What age can this be used?
A
  1. Sweat Test
  2. > = 60 mEq/L
  3. 7 months
5
Q

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

A

Chromosomal test

6
Q

First indicators to test - Clinical status.

A
  1. Chronic/repeated respiratory conditions
  2. Exocrine pancreatic insufficiency
  3. Family history
  4. Meconium ileus
7
Q

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

A
  1. Reproductive
  2. Exocrine/endocrine
  3. GI
  4. Pulmonary
8
Q

Reproductive system effects

A
  1. Late maturation - high energy phase
  2. Sterility
9
Q

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

A
  1. Nutrition/Pulmonary status should be monitored closely.
  2. Discuss birth control measures before puberty.
  3. Depot injection
10
Q

Exocrine/Endocrine system is focused on what organ?

A

Pancreas

11
Q

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

A

insulin deficient, T2DM

12
Q
  1. Treatment for Exocrine/Endocrine disorder?
  2. Inpatient
  3. Outpatient
A
  1. Insulin
  2. Humulin R/ Humalog
  3. Humulin N, R, Lantus
13
Q

GI system effects

A

pancreatic deficiency

14
Q

Pancreatic deficiency sx

A
  1. Failure to thrive
  2. Steatorrhea
  3. Malabsorption
15
Q

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

A

A, D, E, K

16
Q

Treatment of GI problems - combination of all these

A
  1. Nutrition support
  2. Vitamin replacement
  3. Pancreatic Enzyme replacement
17
Q

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

A
  1. 2,000 - 4,000 units lipase / 120ml bottle
  2. 1000 units Lipase/kg
  3. 500 units Lipase/kg
18
Q

How do you choose Pancreatic Enzyme Replacement?

A
  1. Meet # of lipase needed w/ lowest # of capsules needed.
  2. Can choose 1 prod for meal & 1 prod for snack
19
Q

Acute Pulmonary exacerbation
1. What it causes
2. Bug

A
  1. Easier to catch pneumonia
  2. P. aeruginosa
20
Q

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

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

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

A
  1. Obstruction of gas exchange in lungs
  2. Percussion/Postural drainage
22
Q

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?

A
  1. Chest percussive therapy (CPT)
  2. 2-4 x daily
  3. Pulmozyme nebulization - 2.5mg QD/BID
23
Q

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

A

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

24
Q

Other forms of tx for Chronic pulmonary patients to supplement care

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