Cystic Fibrosis

Question 1

What is CF

Answer 1

Affect whole body

Inherited

1 in 4 chances of having a kid with CF if both parent carry gene

Question 2

Patho of CF

Answer 2

Loss of CFTR function affects sodium and chloride ion channels, altering the flow of salt and fluids in and out of cells

With chloride not moving out mucus build up on outside bc it’s not there to break it up

Stick cells through out body

Question 3

CF affects

Answer 3

1) sinuses: infections and polyps
2) lungs : thick sticky mucus build up, bacterial infections
3) skin: sweat glands produce salty sweat “salty kiss”
4) liver: blocked biliary ducks
5) pancreas: blocked pancreatic duct
6) intestine: cannot fully absorb nutrients
7) reproductive: complications

Question 4

CF growth

Answer 4

Growth failure: deficient in Vit A E D K

Osteoarthritis bc lack vit D

Not growing as well- look younger shorter and thinner due to lack or absorption

Question 5

Laboratory and diagnostic test

Answer 5

1) Sweat chloride test
- greater than 60 (norm 35)

2) genetic testing
- done if first test pos

Pulse ox
Chest x-ray- look for pneumonia 
Pulmonary function test 
   - done regularly to see how breathing is 
   - if decrease, admit to hospital

Question 6

Respiratory Signs of CF

Answer 6

• Airway obstruction (mucus build up)
• difficulty clearing secretions
• impaired gas exchange
• chronic cough
• decreased pulmonary function
• recurrent pneumonia
• chronic sinusitis
• nasal polyps

Question 7

GI signs of CF

Answer 7

*** meconium ileus: first sign in infants don’t have first poop w/in 48 hr

• gallstones
• GERD
• altered absorption of iron, vit A D E K
  
  • failure to thrive
• hyperglycemia and development of diabetes
• reduction OR no release of digestive enzymes
  
  • constipated
  • fatty stool

Question 8

CF diet & nutrition

Answer 8

• high calorie, high fat, high salt food
• increase liquid intake ( fluids & electrolyte on days when it’s hot or when sweating)
• pancreatic enzyme supplement (take anytime eat)
• vitamin and mineral supplements (fat soluble vitamin A D E K) (iron and zinc)
• enteral feedings ( usually during sleep at night via g-tube button)

Question 9

CF medication

Answer 9

1) aerosolized & Iv antibiotic ( TOBRAMYCIN)
2) mucolytic ( PULMOZYME) inhaled to break up mucus
3) bronchodilator
4) glucocorticoids for inflammation
5) macro life for infection
6) nebulized hypertonic saline
7) pancreatic enzyme ( CREON)
8) PPI for GERD
9) insulin

Question 10

Therapy for CF

Answer 10

Flutter: breath in and vibrates

Acapella flutter valve

Vest

Positive expiratory pressure (PEP)

Postural drainage: tilt head down

Question 11

Lung transplant

Answer 11

Bilateral lung transplant

Problems:
Long wait list- most don’t make it
Long recovery
Anti rejection meds

This is not a cure bc genetic CF is still there

Question 12

CF nursing management

Answer 12

Maintain patent airway 
Prevent infection 
Maintain growth
Promote family coping 
Prepare child and fam for adult life with CF