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Nur-425
Exam #4 - Cystic Fibrosis
| Term | Definition |
|---|---|
| This is defined as an exocrine disorder of multisystems that results in increased viscosity of mucous | Cystic fibrosis |
| What two systems are most affected with cystic fibrosis? | Respiratory and gastrointestinal |
| What does steatorrhea indicate? | Fatty stools; indicates that body is not absorbing fats from foods |
| How are the stools of someone with cystic fibrosis described? | Foul-smelling, large, loose and bulky |
| What are some early manifestations of respiratory issues with someone with cystic fibrosis? | Wheezing, history of respiratory infections, dry, non-productive cough |
| What are some later manifestations of respiratory issues with someone with cystic fibrosis? | Dyspnea, atelectasis, air trapping (can't expel air), paroxysmal cough |
| What are some progressive manifestations of respiratory issues with someone with cystic fibrosis? | Looks a lot like emphysema; barrel chest, clubbing of fingers and toes, cyanosis, repeated bronchitis and pneumonia |
| What is the hereditary process of cystic fibrosis? | Autosomal recessive disorder |
| When a KUB is performed on a child suspected to have cystic fibrosis, what are they looking for? | Meconium ileus |
| What is the hallmark test for cystic fibrosis? | Chloride sweat test |
| What is the normal value for the chloride sweat test? | <40 mEq/L |
| What value for the chloride sweat test would be diagnostic for cystic fibrosis? | >60 mEq/L |
| What are two red flags for suspected cystic fibrosis in a child? | Frequent upper respiratory infections and low-end growth development |
| What type of diet is used for children with cystic fibrosis? | High protein,high calorie, unrestricted fat |
| What enzyme supplement is taken with every meal/snack by a child with cystic fibrosis? | Pancrease |
| What vitamins do children with cystic fibrosis have difficulty absorbing? | Fat soluble vitamins - A, D, E and K |
| For years, this procedure was done with hand clapping | Chest physiotherapy and postural drainage |
| This can be used in place of CPT to mobilize secretions | Oscillating vest |
| This device uses positive expiratory pressure to mobilize secretions | Flutter or Acapella device |
| This medication is used prior to CPT to open-up the airways | Bronchodilators |
| This medication decreases viscosity/thickness of secretions and is available by aerosol or nebulizer treatments | Pulmozyme (Dornase alfa) |
| When is oxygen used in the treatment of respiratory issues associated with cystic fibrosis? | Only during acute episodes; could knock-out their stimulus to breathe |
| What is a long-term effect of cystic fibrosis? | Pneumothorax |
| Name several signs/symptoms of a pneumothorax? | Tachypnea, tachycardia, dyspnea, pallor or cyanosis |
| Administration of these is a priority intervention in a child who has pneumonia (with cystic fibrosis) | Antibiotics |
| When looking at the ABGs of a child in respiratory distress (with cystic fibrosis), what are you looking at? | PaCO2 and pH; indicating respiratory acidosis |
| With a child in respiratory distress (with cystic fibrosis), what medications could be given for fever? | Tylenol or ibuprofen |
| What percentage of males with cystic fibrosis are sterile? | 95% |
| What determines prognosis in a person with cystic fibrosis? | Pulmonary involvement |
| True or False. Organ transplants have proven effective to prolong life of those with cystic fibrosis | False; overall survival rate after 5 years is only 60% |
| True or False. Using immunosuppressant medications is a promising treatment for a person with cystic fibrosis | False; giving immunosuppressant medications to an already immunocompromised person does not produce promising outcomes |
| What type of gland is an exocrine gland? | A gland with ducts |
| Several gastrointestinal-related signs/symptoms of cystic fibrosis include | Distended abdomen (from inadequate protein), weight loss, anemia, sallow skin, steatorrhea, voracious appetite to loss of appetite |
| Why is it necessary for a child with cystic fibrosis to use pancreatic enzymes with their food intake? | To help with the absorption of fatty foods |
| Use of these medications can decrease lung damage over time, but they also put the child at greater risk for infection | Corticosteroids |
| What causes the formation of a pneumothorax in a child with cystic fibrosis? | Pockets form in distal airways due to air trapping and the pockets can rupture and cause a pneumothorax |
Created by:
ssbourbon
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