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53 Cards in this Set

  • Front
  • Back
What glands does Cystic Fibrosis Affect?
The Exocrine glands
How is Cystic Fibrosis genetically transmitted?
It is transmitted by an autosomal recessive gene that both parents must carry for the child to be affected.

When both parents are carriers of the gene, then there is a 25% chance of the child contracting the disease.

It is located on chromosome 7
What is the most common lethal genetic disease for Caucasians in the US?
Cystic Fibrosis (1 in 2000 white neonates)
Primary pathophysiology of cystic fibrosis...
it is an abnormal accumulation of viscous mucous, which leads to obstruction and dilation of many glands and organs.

The lungs and pancreas present the greatest challenge. The reproductive organs can also be affected by the mucous.

Block the pancreas from releasing pancreatic enzymes (lipase, amylase and trypsin)

Salt depletion may also occur during hot weather and heavy exercise.

Portal HTN related to cirrhosis of hte liver can result in esophageal verices.
What are some signs that a newborn may have CF?
•Meconium Ileus
•Steatorrhea - greasy, foul smelling stols that contain fat and undigested food.
•Failure to Thrive (FTT) - due to malabsorption
Distended abdomen - along with thin arms and legs.
•Voracious appetite - because food not being properly digested.
•Salty taste on skin -
•Respiratory distress
•Chronic Obstructive Respiratory Disease - chronic, productive cough
How is CF diagnosed?
Sweat Test - pilocarpine iontophoresis test - quantification of chloride concentration in sweat.
•normally done between 4-6 weeks of age
• > 60mEq/L of chloride = + CF
- >45 is suggestive of CF
•Can also take a Chest Xray
•Can take stool samples for trypsin and fat content (Trypsin would be markedly DIMINISHED in child with CF)
•3 day collection of stool to document steatorrhea
What are is an important nursing intervention to provide during meals for a child with CF?
•administer pancreatic enzymes with meals and snacks.
--Make sure to not get enzymes on lips, or wipe off face, can be caustic to skin
What type of diet should a child with CF receive?

What type of formula should an Infant with CF receive?
Moderate Fat, High Protein, High Calorie, with FREE USE of Salt and Fat soluble vitamins (A,E,D, & K)

Infants should receive PREDIGESTED formula, such as progestimil
What type of respiratory interventions should be performed on children with CF?
Should perform pulmonary Hygiene, such as Chest Percussion and Postural Drainage, 2-4 times a day, which should be PRECEDED by a mucolytic, bronchodilator or antibiotic nebulizer inhilation treatment.
What type of OTC drugs should be avoided for a child with CF?
Cough Suppressants - child should attempt to cough up mucous.
at what age do a child's lungs fully mature? Full # of alveoli present?
age 8
at what age do children tend to stop being abdominal breathers?
age 7, if persists after age 7, then this usually indicates a problem
What respiratory condition might a prolonged expiratory phase indicate?
What respiratory condition might a prolonged inspiratory phase indicate?
Upper airway obstruction

Ex: Croup, Foreign body in airway

If resps > 60 then that is an emergency!
What does clubbing of the nails indicate?
chronic hypoxemia
What are some important signs of respiratory distress?
•Grunting - may indicate impending resp. failure
•Tachycardia To Bradycardia - late sign of respiratory distress
•Severe retractions
diminished or absent breath sounds
•apnea or gasping aspirations
•Poor system perfusion/mottling
•Decreased O2 saturation
What is ALTE? and what are some imminent signs of it?
Apparent Life Threatening Event

•nasal flaring
What do retractions indicate during respiratory distress?
They suggest an obstruction to INSPIRATION at any point in the resp. tract.
What respiratory disorder may hyperinflation of the lungs indicate?
What may be the cause of Unilateral retractions?
it could possibly be a foreign body in one or the other bronchi. It usually occurs in the right side though due to broader bore and more vertical placement.
What are signs of severe respiratory distress?
•diminished breath sounds
•nasal flaring
•decreased oxygen saturation
What types of food should a child who's received a tonsilectomy AVOID?
•Milk - can make one cough by coating throat
•Red colored liquids - need to see if there is bleeding, this can mask bleeding, i.e. cherry juice, fruit juice
•Anything acidic, i.e. orange juice
What is some important post operative care for a patient post tonsilletomy?
•be in side lying position or prone
•wear an ice collar
•watch for swallowing - could indicate bleeding
•drink copious amounts of cold fluids and a soft diet.
What are some physiological symptoms of Croup?
Inflammation of the following:
•vocal cords
•subglottic tissue
of Laryngotrachealbronchiolitis and epiglottitis, which occurs in younger and which occurs in older children?
In genera, LTB occurs in very young children, and epiglottitis is more common in older children.
How soon before feeding should chest PT be administered?
30 minutes before feeding time
What are some precautions one should take when treating Croup?
-always watch airway for signs of closure
-get an x-ray to visualize the airway
-Have blow by O2 ready
-Have an IV antibiotic ready
-keep the child calm
What are some physical symptoms of Croup? (LTB)
•inspiratory stridor
•barking cough
•child may be drooling because unable to swallow
What are the main causes of Croup?
Hemophillis Influenza B
What are some hospital and home treatments for croup?
Home care:
•cool mist
•Mist tent
•racemic epinephrine inhalant
What are some symptoms of epiglotitis?
•child sits in tripod position to breath
•It has a sudden onset
•high fever
•dysphasia and drooling
•epiglottis is cherry red and swollen
What are some important managment points in providing nursing care to a patient with epiglotitis?
-No tongue blade in mouth
•have an emergency tracheostomy set nearby
•keep child calm and quiet
What are some physical symptoms of CF?
•Harsh, dry cough
•low grade fever
•feeding difficulties
•respiratory distress with apnea
•thick mucous
What are some important nursing management points in dealing with a patient with CF?
•give oxygen to maintain SpO2 >95%
•constant pulse ox monitoring
•use normal saline nose drops before suctioning
•perform deep suctioning, especially before feedings
•perform chest PT and inhalation therapies 30min - 1 hr before feeding
•use mechanical ventilation as needed
What are some important types of medications used for patients with CF?
•enzymes to help digest food
•antibiotics to help control infections, especially respiratory infections
•bronchodilators to open airways
•vitamin C to improve absorption of other meds
•Vitamin E,A,K, D / all fat soluble vitamins
What are some long term complications of CF and what is the average life expectancy?
•avg life expectancy is 30 years

Long term complications are:
•nasal polyps
•rectal polyps/ prolapse
•hyperglycemia/ diabetes
What virus most commonly causes Bronchiolitis?
Respiratory Syncytial virus
What is bronchiolitis?
It is an acute obstruction and inflammation of the bronchioles.

The bronchioles become narrowed or occluded as a result of inflammatory process, edema, mucous and cellular debris clog alveoli
At what period during the year is Bronchiolitis most prevelent?
from Fall to April
What are some important management points in treating a patient with bronchiolitis?
•Contact Precautions - can be easily spread by contact!
•assess respiratory distress/ added lung sounds - rales, rhonchi, wheezing
•Pt. should be NPO in hospital (if RR is > 60 then increased chance of aspiration)
•give IV fluids
•give O2 to keep ox sat above 95%
•perform CPT, deep suctioning
•give Tylenol for fever and give antibiotics to fight infection
How do you test for Bronchiolitis?
ALISA assay
suction nose for a sample, then test for RSV virus
What is the physiology of asthma?
chronic inflammatory lung disease involving recurrent breathing problems

it's caused by a complex, multicellular reaction to airway characterized by:
•airway inflammation
•airway hyper-responsiveness to a variety of triggers
What is the most common chronic health problem among children?
What are some symptoms of asthma?
•tightness of chest
•prolonged expiratory phase
•hyper expansion of lungs (as seen in x-ray)
What position should an asthma patient be placed in bed?
High Fowler's position
What are some important management points for a pt with asthma?
•bed rest
•constant pulse ox
•give Nebulized Albuterol
•perform CPT
•Give IV methylprednisone/Solu-Medrol steroid.
•give IV fluids
•Give oxygen to keep SpO2 > 95%
What is the main home "rescue drug" for asthma?
Short or long acting Bronchodilators: Albuterol (ST) beta 2 agonist or Serevent (ST)
Up until what age must children use facial masks when being given bronchodilator treatment?
up until 3 years old
When using Intal or Servent anti inflammatory drugs to treat asthma, what must children remember to do afterward?
They must rinse their mouths out after treatment to prevent thrush.
What is the most common reason for children to come to the pediatrician or emergency room?
Otitis Media
What is a clear sign of otitis media witnessed in infants and younger children?
tugging at ear, fever, accompanied by history of cold or congestion
What do the following acronyms mean?ROM, LOM, BOM, OME?
Right otitis media
left otitis media
bilateral otitis media
Otitis Media with Effusion
Why is Otitis Media and OME a problem?
an infection in the inner ear can lead to rupture of the ear drum. Chronic effusion can lead to hearing loss.