cystic fibrosis Flashcards
(29 cards)
1
Q
cystic fibrosis
A
- A respiratory disorder from inheriting a mutated gene.
- Characterized by mucus glands that secrete an increase in the quantity of thick, tenacious mucus, which leads to mechanical obstruction of organs:
pancreas, lungs, liver, small - Autonomic nervous system abnormalities.
2
Q
what is increased
A
- An increase in organic and enzymatic constituents in the saliva
- An increase in the sodium and chloride content of sweat
3
Q
risk factors
A
Both biological parents carry the recessive trait for cystic fibrosis.
Caucasian ethnicity
1/4
4
Q
clinical findings
earliest indiction of CF
A
- Medical history of respiratory infections, growth failure
- Meconium ileus at birth manifested as distention of the abdomen, vomiting, and inability to pass stool.
- Meconium ileus is the earliest indication of cystic fibrosis in the newborn.
5
Q
early manifestations respiraotry
A
- Wheezing, rhonchi
- Dry, nonproductive cough
- Increased involvement
- Dyspnea
- Paroxysmal cough
- Obstructive emphysema and atelectasis on chest x-ray
6
Q
advanced symptoms
A
- Cyanosis
- Barrel-shaped chest
- Clubbing of fingers and toes
- Multiple episodes of bronchitis or bronchopneumonia
7
Q
gastro findings
A
- Large, frothy, bulky, greasy, foul-smelling stools (steatorrhea)
- Voracious appetite (early)
- Loss of appetite (late)
- Failure to gain weight or weight loss
- Delayed growth patterns
- Distended abdomen
- Thin arms and legs
- Deficiency of fat-soluble vitamins
- Anemia
8
Q
integumentary findings
A
Sweat, tears, and saliva have an excessively high content of sodium and chloride.
9
Q
endocrine findings
A
- Viscous cervical mucus
- Decreased or absent sperm
10
Q
blood specimen
A
Nutritional panel to detect a deficiency of fat-soluble vitamins (A, D, and E).
11
Q
sputum culture
A
For detection of infection: Pseudomonas aeruginosa, Haemophilus influenzae, Burkholderia cepacia, Staphlococcus aureus, Escherichia coli, or Klebsiella pneumoniae
12
Q
diagnsotic tests
A
- DNA testing: To isolate the mutation
- Pulmonary function tests (PFTs)
- Chest x-ray: Can indicate diffuse atelectasis and obstructive emphysema
- Abdominal x-ray: Detect meconium ileus
Stool analysis
- For presence of fat and enzymes
- 72 hr sample with documented food intake
13
Q
sweat chloride test
A
- The child must be well hydrated.
- A device uses an electrical current to stimulate sweat production.
- Collection of sweat from two different sites for adequate sample
- Expected reference range is chloride content less than 40 mEq/L and sodium content less than 70 mEq/L.
14
Q
sweat chloride numbers
A
Chloride greater than 40 mEq/L for infants less than 3 months of age
greater than 60 mEq/L for all others; sodium greater than 90 mEq/L
15
Q
start nursing care
A
- Assess lung sounds and respiratory status.
- Vital signs with oxygen saturation.
- Obtain IV access.
- Use of a peripherally inserted central catheter allows for home IV antibiotic therapy.
- Obtain sputum for culture and sensitivity.
16
Q
pulmonary management nursing care
A
- Airway clearance therapy (ACT)
- Usually prescribed twice a day in the morning and evening.
- Avoid ACT immediately before or after meals.
- Chest physiotherapy (CPT)
- Positive expiratory therapy (PEP)
- Active-cycle-of-breathing techniques, such as “huffing” or forced expiration, are encouraged.
- Autogenic drainage uses an electronic chest vibrator or handheld percussor along with breathing techniques.
High-frequency chest compression - uses a device, such as a flutter mucus clearance device, to encourage the client to breathe with forceful exhalations.
17
Q
pulmonary management nursing care med admin
A
- Administer aerosol therapy as prescribed (bronchodilator, human deoxyribonuclease).
- Often recommended prior to ACT.
- Administer IV or aerosolized antibiotics.
- Encourage physical aerobic exercise.
- Provide oxygen as prescribed (assess for carbon dioxide retention).
18
Q
gastro nursing care
A
- Give three meals a day with snacks.
- Encourage oral fluid intake.
- Administer pancreatic enzymes within 30 min of eating a meal or snack.
- Administer vitamin supplements.
- Administer laxatives or stool softeners for constipation.
- Administer histamine-receptor antagonist and motility medications for GERD.
- Administer possible formula supplements in addition to breast feedings or via gastric tube.
Consult a dietitian. - Provide a well-balanced diet high in protein and calories. Polyethylene-glycol electrolyte solution is administered orally or via nasogastric tube.
19
Q
endocrine nursing care
A
- CF related diabetes (CFRD) necessitates monitoring of blood glucose levels.
- Administer insulin.
- Oral glycemic medications are not effective for CFRD.
20
Q
short acting beta 2 agonists (abuterol)
A
- Cholinergic antagonists (anticholinergics)
- ipratropium bromide (dry mouth)
- Fluticasone propionate/salmeterol
- Monitor for tremors and tachycardia
Dornase alfa - Decreases the viscosity of mucus and improves lung function
- Monitor sputum thickness and ability of client to expectorate.
- Monitor the child for improvement in PFTs.
- Instruct the child how to use a nebulizer.
- Instruct the child to administer once or twice a day.
21
Q
antibiotics
A
- Administer through IV or aerosol.
-Specific to treat pulmonary infection.
-Common medications include tobramycin, ticarcillin,
or gentamicin.
-Assess for allergies.
-High doses may be prescribed.
-Collect blood specimens before and after some IV antibiotics to maintain therapeutic levels.
22
Q
pancreatic enzymes: pancrelipase
A
- Monitor stools (1 to 2 stools/day).
- Administer capsules with all meals and snacks.
- Client can swallow or sprinkle capsules on food.
- Increase dosage of enzymes when eating high-fat foods.
23
Q
vitamins
A
Daily multivitamin and vitamins A, E, D, and K
24
Q
interprofessional care
A
- Respiratory and physical therapy
- Social services
- Pulmonologist
- Pharmacist
- Pediatrician
- Infectious disease specialists
- Dietitians
- Transplantation of heart, lung, pancreas, and liver for clients who have advanced disease can be a consideration.
25
education
- Ensure family has information regarding access to medical equipment and medications.
- Provide teaching about equipment and medications prior to discharge.
- Instruct the family about ways to provide CPT and breathing exercises.
- Promote regular provider visits.
- Emphasize the need for up-to-date immunizations and a yearly influenza vaccine.
- Teach about diet and ways to increase calorie intake.
- Teach indications of infection and when to call the provider.
26
education
- Teach parents about ways to manage chronic illness in children.
- Promote regular physical activity.
- Encourage the family to participate in a support group and use community resources.
- Identify specific needs based on the client’s developmental level.
- Provide home palliative care for the child or adolescent in the terminal stages of CF.
27
respiratory complications
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Respiratory infections, colonizations,
Bronchial cysts
Emphysema
Pneumothorax
Nasal polyps
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28
gi complications
Meconium ileus, prolapse of the rectum, intestinal obstruction
GERD
29
endocrine complications
Diabetes mellitus
