T3-Blueprint: CF and Celiac

Question 1

Exocrine gland dysfunction leading to increased mucous production

Answer 1

CF

Question 2

CF: Mucus glands secrete thick _____ that accumulates and ___ the gland

Answer 2

Secrete thick MUCOPROTEIN that accumulates and DILATES the glands

Question 3

CF: Small passages in organs (especially ____ & ____) become obstructed when secretions coagulate and become hard in the glands and ducts, leading to fibrosis and failure

Answer 3

Pancreas and bronchioles

Question 4

Results from sensitivity to gluten

Answer 4

Celiac

Question 5

What is gluten?

Answer 5

Protein found in grains

Question 6

Celiac: Vili of the small intestine _____, reducing the absorptive surface leading to decreased digestion of ___ and decreased absorption of ___ & ___

Answer 6

Villi of small intestine FLATTEN out, reducing the absorptive surface leading to decreased digestion of FAT and decreased absorption of VITAMINS AND CARBS

Question 7

What are the early respiratory S/S of CF?

Answer 7

Wheezing

Dry, non-productive cough

Question 8

What are the late respiratpory signs of CF?

Answer 8

• Dyspnea
• Paroxysmal cough
• Obstructive emphysema
• Patchy areas of atelectasis
• Frequenty pulmonary infections
• Overinfalted, barrel shaped chest
• Cyanosis
• Clubbing of fingers and toes

Question 9

What are the early GI s/s of CF?

Answer 9

Meconium ileus

Question 10

What are the late GI s/s of CF?

Answer 10

• Large, bulky, frothy, foul-smelling stools
• Increased appetite early in disease (with no weight gain)
• Decreased appetite later–> failure to grow and anemia
• Fat soluble vit. deficiency
• Thin extremities
• Tissue wasting

Question 11

What are s/s of celiac disease?

Answer 11

• Irritability
• Anorexia
• Chronic diarrhea
• Steatorrhea
• Vit. deficiencies
• Abdominal distention
• Malnutrition
• FTT

Question 12

How do we test for celiac?

Answer 12

Biopsy of small intestine

Question 13

How do we test for CF?

Answer 13

Sweat tests–presence of high NaCl concentrations in sweat is a unique characteristic of CF

Question 14

CF: What is a normal sweat chloride test?

Answer 14

Less than 40 mEq/L

Question 15

CF: What is the level for infants with CF who had sweat chloride test?

Answer 15

Greater than 60 mEq/L

Question 16

CF: What is the level for all other ages with CF who had a sweat chloride test?

Answer 16

Greater than 40 mEq/L

Question 17

What do stools look like for CF patients?

Answer 17

• Frothy
• Foul smelling
• Fat containing
• Float (due to fat content)

Question 18

What are the fat soluble vitamins?

Answer 18

A, D, E, K

*CF patients have fat containing stools

Question 19

One of the early signs for CF is meconium ileum. What is this?

Answer 19

Bowel obstruction

Question 20

CF: Describe meconium ileus characteristics.

Answer 20

Thick, puttylike, tenacious, mucilaginous meconium that blocks the lumen of the small intestine

Question 21

CF: Meconium ileum blocks the lumen of the small intestine. Where does this usually happen and what does that mean?

Answer 21

Usually at or near the ileocecal valve, which gives rise to intestinal obstruction (abdominal distention, vomiting, failure to pass stools, rapid dehydration with electrolyte imbalance)

Question 22

What is the diet for CF?

Answer 22

High protein, high calorie

Question 23

What is the diet for celiac?

Answer 23

Rice, corn, soybean flours

Question 24

T/F: It is okay to give a child with celiac disease foods with wheat, barley, rye, or oats

Answer 24

NO

Question 25

What respiratory meds do we give for CF?

Answer 25

• SABA: albuterol
• Anticholinergics: Ipratroprium
• Combo: fluticasone/salmaterol
• Human deoxyribonuclease
• Dornase alfa (decreases viscosity of mucous)

Question 26

What are some antibiotics we give CF patients?

Answer 26

Tobramycin
Tircarcillin
Gentamycin

Question 27

What GI meds do we give CF patients?

Answer 27

• Pancreatic enzymes
• Vitamines: multi and ADEK
• Constipation meds: Polyethylene glycol (golytely)
• H2 receptor agonist and motility meds for GERD

Question 28

Does the US have universal newborn screening for CF?

Answer 28

No, only 2 states offer this

Question 29

CF Screening: Children who were identified and treated early in infancy with aggressive nutritional support had improved ___ & ___ well into adolescence

Answer 29

Height and weight

Question 30

If a newborn were to be screened for CF, how is it done?

Answer 30

Immunoreactive trypsinogen analysis performed on a dried sport of blood…which may be followed by direct analysis of DNA for presence of mutations in the same dried spot of blood

Question 31

CF: Prevention of pulmonary infection involves a daily routine of CPT to maintain pulmonary hygiene. What is CPT?

Answer 31

Patient is dressed in a light shirt and placed in a postural drainage position. The practitioner gently but firmly strikes the chest wall with a cupped hand. A popping, hollow sound, NOT a slapping sound, should be the result. This procedure is done over the RIBCAGE ONLY

Question 32

How often is CPT done on CF patients?

Answer 32

On average twice failing, on rising and in the evening

*and more frequently during pulmonary infection

Question 33

CF: A small, handheld plastic pipe with a stainless steel ball on the inside that facilitates removal of mucus

Answer 33

Flutter Mucus clearance device

Question 34

What are the advantages of the flutter mucus clearance device?

Answer 34

Increasing septum expectoration and being used without assistance

Question 35

CF: What is the cornerstone of pulmonary therapy?

Answer 35

CPT—CF patients have been found to REGRESS when CPT is discontinued!–although it is time consuming for child and family, it is the cornerstone treatment

Question 36

The term celiac disease is often used to describe a symptom complex with 4 characteristics. What are those 4 characteristics?

Answer 36

1. Steatorrhea
2. General malnutrition
3. Abdominal distention
4. Secondary vitamin deficiencies

Question 37

Celiac: What does steatorrhea mean?

Answer 37

Fat, frothy, foul, bulky stool