Exam #4 - Cystic Fibrosis
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| This is defined as an exocrine disorder of multisystems that results in increased viscosity of mucous | Cystic fibrosis
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| What two systems are most affected with cystic fibrosis? | Respiratory and gastrointestinal
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| What does steatorrhea indicate? | Fatty stools; indicates that body is not absorbing fats from foods
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| How are the stools of someone with cystic fibrosis described? | Foul-smelling, large, loose and bulky
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| What are some early manifestations of respiratory issues with someone with cystic fibrosis? | Wheezing, history of respiratory infections, dry, non-productive cough
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| What are some later manifestations of respiratory issues with someone with cystic fibrosis? | Dyspnea, atelectasis, air trapping (can't expel air), paroxysmal cough
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| 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
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| What is the hereditary process of cystic fibrosis? | Autosomal recessive disorder
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| When a KUB is performed on a child suspected to have cystic fibrosis, what are they looking for? | Meconium ileus
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| What is the hallmark test for cystic fibrosis? | Chloride sweat test
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| What is the normal value for the chloride sweat test? | <40 mEq/L
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| What value for the chloride sweat test would be diagnostic for cystic fibrosis? | >60 mEq/L
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| What are two red flags for suspected cystic fibrosis in a child? | Frequent upper respiratory infections and low-end growth development
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| What type of diet is used for children with cystic fibrosis? | High protein,high calorie, unrestricted fat
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| What enzyme supplement is taken with every meal/snack by a child with cystic fibrosis? | Pancrease
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| What vitamins do children with cystic fibrosis have difficulty absorbing? | Fat soluble vitamins - A, D, E and K
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| For years, this procedure was done with hand clapping | Chest physiotherapy and postural drainage
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| This can be used in place of CPT to mobilize secretions | Oscillating vest
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| This device uses positive expiratory pressure to mobilize secretions | Flutter or Acapella device
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| This medication is used prior to CPT to open-up the airways | Bronchodilators
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| This medication decreases viscosity/thickness of secretions and is available by aerosol or nebulizer treatments | Pulmozyme (Dornase alfa)
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| 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
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| What is a long-term effect of cystic fibrosis? | Pneumothorax
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| Name several signs/symptoms of a pneumothorax? | Tachypnea, tachycardia, dyspnea, pallor or cyanosis
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| Administration of these is a priority intervention in a child who has pneumonia (with cystic fibrosis) | Antibiotics
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| 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
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| With a child in respiratory distress (with cystic fibrosis), what medications could be given for fever? | Tylenol or ibuprofen
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| What percentage of males with cystic fibrosis are sterile? | 95%
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| What determines prognosis in a person with cystic fibrosis? | Pulmonary involvement
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| 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%
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| 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
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| What type of gland is an exocrine gland? | A gland with ducts
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| 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
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| 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
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| Use of these medications can decrease lung damage over time, but they also put the child at greater risk for infection | Corticosteroids
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| 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
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