T3 - Cystic Fibrosis and Celiac Disease Flashcards
CF is characterized by — gland dysfunction.
exocrine (mucus-producing)
T/F: CF is inherited.
True
CF is seen primarily in –
white children (male and female)
Where is the location of the mutated gene w/ CF?
long arm of chromosome 7
What number of the population are carriers for CF?
1 in 25 caucasions
With each pregnancy, two carrier parents have a – chance of having a child w/ CF.
1: 4
- there is a 50% chance child will be a carrier
- there is a 25% chance child will neither have CF or be a carrier
How CF is passed from parents.
see the pic in ppt
A – – is useful for calculating the chances of having a child w/ CF.
Punnett Square
Patho of CF
Mucous glands secrete thick mucoprotein that accumulates and dilates the glands.
Small passages in organs (esp. pancreas and bronchioles) become obstructed when secretions coagulate and become hard in the glands and ducts leading to fibrosis and failure.
How does CF affect the GI tract?
impairs absorption of nutrients (fat-soluble vitamin absorption is decreased)
CF leads to what type of stools?
frothy stools from undigested fat
What else occurs in GI tract due to CF?
Isles of Langerhans decreases
Fibrosis in liver and Biliary obstruction
Prolapse of rectum
Intestinal obstruction
S/S of CF
Short Stature
Sinusitis
FTT
Nonproductive Cough
Increased AP chest diameter
Protuberant abdomen
Digital Clubbing
What would CF stool’s look like (ie: the Four F’s)?
Frothy (bulky and large quantity)
Foul Smelling
Fat Containing
Float (due to fat content)
What type of tests are used to measure for CF?
two positive SWEAT TESTS
- presence of high NaCl concentrations in sweat is a unique characteristic of CF
Parents will sometime say their child tastes — with CF.
salty
- presence of high NaCl concentrations in sweat is a unique characteristic of CF
Early Signs of CF in airway
Wheezing
Dry, nonproductive cough
Late signs of CF in airway
dyspnea
paroxysmal cough
obstructive
emphysema’patchy areas of atelactasis
frequent pulmonary infections
overinflated, barrel-shaped chest
cyanosis
clubbing of fingers and toes
Early sign of CF in GI tract
meconium ileus
Late signs of CF in GI tract
large, bulky frothy, foul-smelling stools
increased appetite early in disease (w/ no weight gain)
decreased appetite–later
failure to grow and anemia
fat soluble vitamin deficiency
thin extremities and tissue wasting
Which type of diet for CF?
high protein, high calorie
- may need up to 150% of daily requirements
What is Flutter Therapy for CF?
Looks similar to a fat pipe that has a steel ball encased in it.
The patient blows on the pipe, the ball vibrates and sends the vibrations down the airways and into the lungs.
The vibrations loosen the mucus and help it move up the airways.
Works 3 x better than chest physiotherapy.
– is an enzyme that chops up DNA and causes liquefied form of mucous (which is easier to cough out).
Pulmonzyme
Celiac Disease results from what?
sensitivity to gluten (protein found in grains)
In CD, the villi of the small intestine do what?
flatten out
- reduces absorptive surface
- reduces fat digestion
- reduces absorption of vitamins and carbs
CD clients first present symptoms that look like –
CF
Digestive disturbances from CD usually seen at what age?
6-12 mths
S/S of Celiac’s Disease
Irritability
Anorexia
Chronic diarrhea
Steatorrhea
Vit deficiencies
Abdominal distention
Malnutrition
FTT
CD clients can eat —
CD clients cannot eat —
Can eat grains (rice, corn, soybean flours)
Cannot eat wheat, barley, rye, oats
