CYSTIC FIBROSIS EXAM QUESTIONS WITH
100% CORRECT ANSWERS!!
What are the diagnostic test for cystic fibrosis ?
Sweat chloride test - normal less than 40 mEq/L
DNA testing to isolate mutation
Pulmonary Function Tests
Chest X-rays
Abdominal X-rays
What would be the nursing care of a cystic fibrosis child with pulmonary issues
General care of Vitals, in particular lung sounds, IV, get sputum cultures (B. capacia and P.
aeruginosa), provide support ot child and family
Perform chest physiotherapy (CPT) with posterual drainage (traditional or vest)
Administer aerosol therapy
Administer IV antibiotics (tobramycin, ticarcillian or gentamicin)
Encourage physical exercise
Provide Oxygen as necessary
What are the complications of Cystic Fibrosis
Respiratory Complications - infections, bronchial cysts, emphysema, pneumothorax, nasal polys
Gastrointestional complications meconium ileus, prolapse of the rectum, distal intestional
obstruction syndrome, GERD
Endocrine complications - Diabetes mellitus
What would be nursing care for gastrointestional complications of a cystic fibrosis patient?
Give multiple small meals a day with snacks
Encourage fluid intake
Administer pancreatic enzymes as prescribed (with meals or 30 within 30 minutes)
Administer vitamin supplements (multivitamin (especially A, D, E, K)
Administer polyethylene-glycol electrolyte solution via nasogastric tube for constipation
, Administer histamine-receptor antagonist and motility medications for GERD
Administer possible formula supplements via gastric tube
Consult dietitian
diagnosing CF at birth
At birth, some babies have a terminal Ileus that blocks feces from being excreted. This is highly
suggestive of cystic fibrosis - s/s abdominal distinction , n/v and inability to pass meconium
S/s cystic fibrosis pink puffer
Fatigue, chronic cough, thick yellow mucous, sob, cyanosis, difficulty in exhaling, hyperinflation
of chest, barrel shaped chest, clubbing of fingers, +sputum culture , distended abdomen, thin
arms and legs with a bloated belly
Sweat test
Pilocarpine is applied to skin on forearm and covered with electrodes. The electrodes are
stimulated (to get sweat) and the test is checked for NaCl levels
CF reproductive and endocrine
men are usually sterile. There is a delay in puberty, viscous cervical mucous
- glucose monitoring may be needed because of insulin resistance or deficiency.
CF meds and teaching
Albuterol - teaching about proper use and monitor for tremors or tachycardia
ipratropium (bronchodilator)- will cause dry mouth, suck on hard candies, nebulizer treatment
teaching - breaks up mucus
antibiotics- screen for allergies, take entire course
pancreatic enzymes- take with meals; swallow whole or sprinkle on food
Therapeutic vest- use on an empty stomach- coughing spasms can cause vomiting
Diet-High calorie, let child choose, ADEK supplements, stool softeners
Fluticasone propionate/salmeterol - steroid
cor pulmonale in CF
100% CORRECT ANSWERS!!
What are the diagnostic test for cystic fibrosis ?
Sweat chloride test - normal less than 40 mEq/L
DNA testing to isolate mutation
Pulmonary Function Tests
Chest X-rays
Abdominal X-rays
What would be the nursing care of a cystic fibrosis child with pulmonary issues
General care of Vitals, in particular lung sounds, IV, get sputum cultures (B. capacia and P.
aeruginosa), provide support ot child and family
Perform chest physiotherapy (CPT) with posterual drainage (traditional or vest)
Administer aerosol therapy
Administer IV antibiotics (tobramycin, ticarcillian or gentamicin)
Encourage physical exercise
Provide Oxygen as necessary
What are the complications of Cystic Fibrosis
Respiratory Complications - infections, bronchial cysts, emphysema, pneumothorax, nasal polys
Gastrointestional complications meconium ileus, prolapse of the rectum, distal intestional
obstruction syndrome, GERD
Endocrine complications - Diabetes mellitus
What would be nursing care for gastrointestional complications of a cystic fibrosis patient?
Give multiple small meals a day with snacks
Encourage fluid intake
Administer pancreatic enzymes as prescribed (with meals or 30 within 30 minutes)
Administer vitamin supplements (multivitamin (especially A, D, E, K)
Administer polyethylene-glycol electrolyte solution via nasogastric tube for constipation
, Administer histamine-receptor antagonist and motility medications for GERD
Administer possible formula supplements via gastric tube
Consult dietitian
diagnosing CF at birth
At birth, some babies have a terminal Ileus that blocks feces from being excreted. This is highly
suggestive of cystic fibrosis - s/s abdominal distinction , n/v and inability to pass meconium
S/s cystic fibrosis pink puffer
Fatigue, chronic cough, thick yellow mucous, sob, cyanosis, difficulty in exhaling, hyperinflation
of chest, barrel shaped chest, clubbing of fingers, +sputum culture , distended abdomen, thin
arms and legs with a bloated belly
Sweat test
Pilocarpine is applied to skin on forearm and covered with electrodes. The electrodes are
stimulated (to get sweat) and the test is checked for NaCl levels
CF reproductive and endocrine
men are usually sterile. There is a delay in puberty, viscous cervical mucous
- glucose monitoring may be needed because of insulin resistance or deficiency.
CF meds and teaching
Albuterol - teaching about proper use and monitor for tremors or tachycardia
ipratropium (bronchodilator)- will cause dry mouth, suck on hard candies, nebulizer treatment
teaching - breaks up mucus
antibiotics- screen for allergies, take entire course
pancreatic enzymes- take with meals; swallow whole or sprinkle on food
Therapeutic vest- use on an empty stomach- coughing spasms can cause vomiting
Diet-High calorie, let child choose, ADEK supplements, stool softeners
Fluticasone propionate/salmeterol - steroid
cor pulmonale in CF
