Ch. 19: CF Flashcards
CF is a respiratory disorder that results from inheriting a ____
Mutated gene
What is CF characterized by?
- Mucus glands that secrete an increase in the quantity of thick, tenacious mucous–> Leads to mechanical obstruction of organs
- Increase in organic and enzymatic constituents in the saliva
- Increase in Na and Cl in sweat
- CNS abnormalities
One of the things CF is characterized by is mucus glands that recreate an increase in the quantity of thick, tenacious mucous, which leads to obstruction of organs. What organs are affected?
Pancreas Lungs Liver S. Intestine Reproductive system
One or both biological parents carry the recessive trait for CF?
BOTH
Physical assessment findings:
______ at birth manifested as distention of the abdomen, vomiting, and inability to pass stool
Meconium ileus
Physical assessment: Respiratory
What are the early signs?
Wheezing
Dry, non-productive cough
Physical assessment: Respiratory
What are the increased involvement signs?
Dyspnea
Paroxysmal cough
Mucus plugs and atelectasis on xray
Physical assessment: Respiratory
What are the advanced involvement signs?
Cyanosis
Barrel-shaped chest
Clubbing of fingers and toes
Multiple episodes of bronchitis or bronchopneumonia
Physical assessment: GI
What kind of stools?
Large, loose, fatty, sticky, foul-smelling
Physical assessment: GI
What are the early signs for appetite? late?
Early: Voracious appetite
Late: Loss of appetite
Physical assessment: GI
Do they gain weight easily?
No–failure to gain weight; weight loss
Physical assessment: GI
How are their growth patterns? What type of abdomen, arms, and legs?
Growth pattern= delayed
Arms and legs= thin
Physical assessment: GI
Deficiency of what?
Fat soluble vitamins
Physical assessment: GI
What kind of blood problems?
Anemia
Physical assessment: Integumentary
How are the sweat, tears, and saliva?
Abnormally salty
Physical assessment: Endocrine and reproductive
What goes on here?
- Viscous cervical mucus
- Decreased/absent sperm
How is CF diagnosed?
Sweat chloride test
What is a sweat chloride test?
A special device that stimulates sweat production
How many sites need to have sweat collected for it to be an adequate sample for the sweat test?
Sweat from 2 different sites
Sweat chloride test: Expected reference range is the presence of chloride less than ____
40 mEq/L
**Sweat chloride test: What is the diagnostic confirmation for CF?
Normal= 40 mEq/L
CF= Chloride greater than 60 mEq/L for infants less than 3 months of age & greater than 40 mEq/L for all others
Why would a chest x ray be done?
May indicate atelectasis and obstructive emphysema
Why would an abdominal x ray be done?
Detect meconium ileus
Lab tests: What are we testing for detection of infection?
- Pseudomonas aeruginosa
- Haem influenzae
- Burkholderia cepacia
- S. aureus
- E. Coli
- Klebsiella pneumonia
What 2 types of infection need contact isolation?
B. cepacia and P. aeruginosa
For pulmonary management for CF, one nurse care is to perform chest physiotherapy (CPT) with postural drainage as prescribed. When should we avoid doing CPT?
Before and after meals
For pulmonary management for CF. one nurse care is to perform airway clearance therapy. What do we use to do this and how many times a day do we do this?
Use flutter mucus clearance device
Twice daily
Pulmonary management. We should encourage physical exercise. Ex?
Stationary bike
GI management. What kind of diet? How many meals a day?
Diet: well balanced, high in PROTEIN and CALORIES
3 meals a day with snacks
GI management. What do we administer as prescribed 30 min within eating?
Pancreatic enzymes
GI management: What vitamins may we give (as prescribed)?
Multivitamins; vit. A, D, E and K
GI management. What if the child is constipated?
Give GoLYTELY (polythylene-glycol electrolyte solution)
Medications. What med decreases the viscosity of mucus and improves lung function?
Dornase alfa
Dornase alfa is administered ____
1x a day
If a client is taking ipratropium bromide (cholinergic antagonists), what should we observe for and what should we do with client education?
Observe: Dry mouth
Client education: Wait 5 min between ipratropium bromide and other inhaled meds
If the client is taking albuterol, what do we monitor the child for?
Tremors and tachycardia
What should we teach the client about fluticasone propionate/salmeterol?
Rinse mouth after taking that
What are some common antibiotics given? How are they administered?
Tobramycin
Ticarcillin
Gentamicin
Administered through IV or aerosol
If we are giving the child with CF pancreatic enzymes, what should we be monitoring? Explain.
Adequate dosing
Loose, fatty stool= increase dose
Constipated= decrease dose
How do we give the pancreatic enzymes?
In a capsule with all meals and snacks (remember earlier, give within 30 min of eating)
Pancreatic enzymes–is it okay if the client swallows capsule whole? Is it okay if they sprinkle capsules on food?
Yes to both
Care after discharge. What should we emphasize?
Need for up-to-date immunizations and a yearly influenza vaccine
A nurse is caring for a child who is suspected of having CF. Which of the following tests should the nurse prepare to administer to confirm the diagnosis?
A. Sweat chloride
B. Pulmonary function test
C. Arterial blood gases
D. Chest percussion
A
A nurse is admitting a child who has CF. Which of the following meds should the nurse anticipate including in the plan of care? (SATA)
A. Tobramycin B. Solu-medrom C. Fat-soluble vitamins D. Albuterol E. Dornase alfa
A, C, D, E
A nurse is caring for a child who has CF. Which of the following are expected findings? (SATA)
A. Wheezing B. Clubbing of fingers and toes C. Barrel-shaped chest D. Thin, watery mucus E. Rapid growth spurts
A, B, C
A nurse is planning care of child who has CF. Which of the following interventions should she include in plan of care?
A. Provide a low calorie, low protein diet
B. Administer pancreatic enzymes with meals and snacks
C. Promote an increase in fluids after 1800
D. Restrict physical activity
B
