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Exam #4 - Cystic Fibrosis

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