cystic fibrosis

Question 1

cystic fibrosis

Answer 1

• 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.

Question 2

what is increased

Answer 2

• An increase in organic and enzymatic constituents in the saliva
• An increase in the sodium and chloride content of sweat

Question 3

risk factors

Answer 3

Both biological parents carry the recessive trait for cystic fibrosis.
Caucasian ethnicity
1/4

Question 4

clinical findings

earliest indiction of CF

Answer 4

• 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.

Question 5

early manifestations respiraotry

Answer 5

• Wheezing, rhonchi
• Dry, nonproductive cough
• Increased involvement
• Dyspnea
• Paroxysmal cough
• Obstructive emphysema and atelectasis on chest x-ray

Question 6

advanced symptoms

Answer 6

• Cyanosis
• Barrel-shaped chest
• Clubbing of fingers and toes ­
• Multiple episodes of bronchitis or bronchopneumonia

Question 7

gastro findings

Answer 7

• 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

Question 8

integumentary findings

Answer 8

Sweat, tears, and saliva have an excessively high content of sodium and chloride.

Question 9

endocrine findings

Answer 9

• Viscous cervical mucus

- Decreased or absent sperm

Question 10

blood specimen

Answer 10

Nutritional panel to detect a deficiency of fat-soluble vitamins (A, D, and E).

Question 11

sputum culture

Answer 11

For detection of infection: Pseudomonas aeruginosa, Haemophilus influenzae, Burkholderia cepacia, Staphlococcus aureus, Escherichia coli, or Klebsiella pneumoniae

Question 12

diagnsotic tests

Answer 12

• 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

Question 13

sweat chloride test

Answer 13

• 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.

Question 14

sweat chloride numbers

Answer 14

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

Question 15

start nursing care

Answer 15

• 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.

Question 16

pulmonary management nursing care

Answer 16

• 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.

Question 17

pulmonary management nursing care med admin

Answer 17

• 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).

Question 18

gastro nursing care

Answer 18

• 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.

Question 19

endocrine nursing care

Answer 19

• CF related diabetes (CFRD) necessitates monitoring of blood glucose levels.
• Administer insulin.
• Oral glycemic medications are not effective for CFRD.

Question 20

short acting beta 2 agonists (abuterol)

Answer 20

• 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.

Question 21

antibiotics

Answer 21

• 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.

Question 22

pancreatic enzymes: pancrelipase

Answer 22

• 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.

Question 23

vitamins

Answer 23

Daily multivitamin and vitamins A, E, D, and K

Question 24

interprofessional care

Answer 24

• 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.

Question 25

education

Answer 25

• 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.

Question 26

education

Answer 26

• 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.

Question 27

respiratory complications

Answer 27

Respiratory infections, colonizations, 
Bronchial cysts
Emphysema
Pneumothorax
Nasal polyps

Question 28

gi complications

Answer 28

Meconium ileus, prolapse of the rectum, intestinal obstruction
GERD

Question 29

endocrine complications

Answer 29

Diabetes mellitus