Cystic Fibrosis: Case Study Flashcards by Holly Grey

What is done at birth?
1st

Heel prick test
= test for IRT levels
= immuno reactive trypsinin

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What is trypsinin?

Pro-enzyme
= enzyme that is NOT active
Activated by trypsin in small intestine

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What is then done if IRT levels come back elevated?

Sweat chloride test

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What is the level of sweat chloride if CF?
2nd

> 60 mmol/L

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What is then done if sweat test is elevated?
3rd

Genetic test to identify if genetic mutation is present

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What is the pancreas’ endocrine function?

Release hormones
eg. insulin

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What is the pancreas’ exocrine function?

Release enzymes

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What happens to pancreas in CF patient?

Pancreatic duct blocked by mucus
= enzymes blocked
= accumulate

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What happens if enzymes are stuck in pancreas?

Carbs, proteins + fats cannot be broken down
= nutrients cannot be absorbed

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What therapy is immediately offered?

Early nutrition
Liquid multivitamins
Replacement of enzymes
Prophylactic antibiotics

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What is an example of replacement of enzymes drug?

Creon

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What is the problem with Creon?

Enteric coating
= need to be given with acidic food
= or protease will degrade the mouth
Babies can’t be given tablets

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What do you monitor in patient’s with CF?

Height + weight
Pulmonary status
Oropharyngeal cultures
Glucose tolerance testing
Bone density

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How is pulmonary status tested?

FEV1
= forced expiratory vol in 1s

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Why is oropharyngeal cultures collected?

Testing for bacteria

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Why is glucose tolerance tested?

Pancreas NOT working
= chance of developing diabetes

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Why is bone density tested?

NO nutrition
= chance of osteoporosis

What % of FEV1 is considered respiratory failure?

30%

Why is inhalation of hypertonic saline sometimes given to CF patients?

Help liquify mucus
= can cough it up

What is Dornase alfa?

Enzyme
Recombinant form of human deoxyribonuclease

What does Dornase alfa do?

Breaks up cellular DNA from neutrophils
= decreases mucus viscosity

How does cellular DNA of neutrophils end up in lungs?

Bacteria trapped in lungs
= neutrophils stimulated
= trapped
= die (apoptosis)
= DNA released
= increase viscosity of mucus

What is Pseudomonas aeruginosa?

Gram negative
CF patients will eventually get it at one point

What is the problem with Pseudomonas aeruginosa?

Difficult to treat
= resistant to most antibiotics

What is often prescribed to treat Pseudomonas aeruginosa in CF patients?

Oral ciprofloxacin for 3 weeks Nebulised tobramycin 300mg for 1 month After negative, ciprofloxacin stopped BUT tobramycin continued for 1 month

What is the problem with quinolones?

Caution in children = joint problems

Why are quinolones used then in CF paediatric patients?

CF outweighs the problems

What type of antibiotic is ciprofloxacin?

Quinolone

What type of antibiotic is tobramycin?

Aminoglycosides

What type of antibiotic is azithromycin?

Macrolide antibiotic

Why is azithromycin sometimes used as "500mg 3x a week"?

Has inflammatory properties at that dose + regime

What is the normal level for 2-hr OGTT plasma glucose?

<11mmol/L = higher diabetes/pancreas not working?

What is the normal level for 2-hr OGTT plasma glucose?

<11mmol/L = higher diabetes/pancreas not working?

What is HbA1c?

Sugar stuck on Hb

What is the normal level for HbA1c?

<6%

What does microalbuminuria suggest?

Protein in urine = signs of renal failure

Which gene is mutated in CF?

CFTR

What does CFTR code for?

Cl channels

What happens when we sweat?

Release Na+, Cl- + H2O Na channel = Na+ reabsorbed Cl channel = Cl- reabsorbed = NORMAL person

What happens because Cl- is NOT reabsorbed?

= Na+ NOT reabsorbed = NaCl excreted out = salty sweat = H2O follows salt = dehydration

What happens because H2O leaves in CF patients?

Mucus gets thick + sticky = mucus cannot be cleared = bacteria cannot be cleared = stuck in lungs