T3 - Cystic Fibrosis and Celiac Disease

Question 1

CF is characterized by — gland dysfunction.

Answer 1

exocrine (mucus-producing)

Question 2

T/F: CF is inherited.

Answer 2

True

Question 3

CF is seen primarily in –

Answer 3

white children (male and female)

Question 4

Where is the location of the mutated gene w/ CF?

Answer 4

long arm of chromosome 7

Question 5

What number of the population are carriers for CF?

Answer 5

1 in 25 caucasions

Question 6

With each pregnancy, two carrier parents have a – chance of having a child w/ CF.

Answer 6

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

Question 7

How CF is passed from parents.

Answer 7

see the pic in ppt

Question 8

A – – is useful for calculating the chances of having a child w/ CF.

Answer 8

Punnett Square

Question 9

Patho of CF

Answer 9

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.

Question 10

How does CF affect the GI tract?

Answer 10

impairs absorption of nutrients (fat-soluble vitamin absorption is decreased)

Question 11

CF leads to what type of stools?

Answer 11

frothy stools from undigested fat

Question 12

What else occurs in GI tract due to CF?

Answer 12

Isles of Langerhans decreases

Fibrosis in liver and Biliary obstruction

Prolapse of rectum

Intestinal obstruction

Question 13

S/S of CF

Answer 13

Short Stature

Sinusitis

FTT

Nonproductive Cough

Increased AP chest diameter

Protuberant abdomen

Digital Clubbing

Question 14

What would CF stool’s look like (ie: the Four F’s)?

Answer 14

Frothy (bulky and large quantity)

Foul Smelling

Fat Containing

Float (due to fat content)

Question 15

What type of tests are used to measure for CF?

Answer 15

two positive SWEAT TESTS

• presence of high NaCl concentrations in sweat is a unique characteristic of CF

Question 16

Parents will sometime say their child tastes — with CF.

Answer 16

salty

• presence of high NaCl concentrations in sweat is a unique characteristic of CF

Question 17

Early Signs of CF in airway

Answer 17

Wheezing

Dry, nonproductive cough

Question 18

Late signs of CF in airway

Answer 18

dyspnea

paroxysmal cough

obstructive

emphysema’patchy areas of atelactasis

frequent pulmonary infections

overinflated, barrel-shaped chest

cyanosis

clubbing of fingers and toes

Question 19

Early sign of CF in GI tract

Answer 19

meconium ileus

Question 20

Late signs of CF in GI tract

Answer 20

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

Question 21

Which type of diet for CF?

Answer 21

high protein, high calorie

• may need up to 150% of daily requirements

Question 22

What is Flutter Therapy for CF?

Answer 22

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.

Question 23

– is an enzyme that chops up DNA and causes liquefied form of mucous (which is easier to cough out).

Answer 23

Pulmonzyme

Question 24

Celiac Disease results from what?

Answer 24

sensitivity to gluten (protein found in grains)

Question 25

In CD, the villi of the small intestine do what?

Answer 25

flatten out

• reduces absorptive surface
• reduces fat digestion
• reduces absorption of vitamins and carbs

Question 26

CD clients first present symptoms that look like –

Answer 26

CF

Question 27

Digestive disturbances from CD usually seen at what age?

Answer 27

6-12 mths

Question 28

S/S of Celiac’s Disease

Answer 28

Irritability

Anorexia

Chronic diarrhea

Steatorrhea

Vit deficiencies

Abdominal distention

Malnutrition

FTT

Question 29

CD clients can eat —

CD clients cannot eat —

Answer 29

Can eat grains (rice, corn, soybean flours)

Cannot eat wheat, barley, rye, oats