T3-Blueprint: CF and Celiac Flashcards
Exocrine gland dysfunction leading to increased mucous production
CF
CF: Mucus glands secrete thick _____ that accumulates and ___ the gland
Secrete thick MUCOPROTEIN that accumulates and DILATES the glands
CF: Small passages in organs (especially ____ & ____) become obstructed when secretions coagulate and become hard in the glands and ducts, leading to fibrosis and failure
Pancreas and bronchioles
Results from sensitivity to gluten
Celiac
What is gluten?
Protein found in grains
Celiac: Vili of the small intestine _____, reducing the absorptive surface leading to decreased digestion of ___ and decreased absorption of ___ & ___
Villi of small intestine FLATTEN out, reducing the absorptive surface leading to decreased digestion of FAT and decreased absorption of VITAMINS AND CARBS
What are the early respiratory S/S of CF?
Wheezing
Dry, non-productive cough
What are the late respiratpory signs of CF?
- Dyspnea
- Paroxysmal cough
- Obstructive emphysema
- Patchy areas of atelectasis
- Frequenty pulmonary infections
- Overinfalted, barrel shaped chest
- Cyanosis
- Clubbing of fingers and toes
What are the early GI s/s of CF?
Meconium ileus
What are the late GI s/s of CF?
- 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
What are s/s of celiac disease?
- Irritability
- Anorexia
- Chronic diarrhea
- Steatorrhea
- Vit. deficiencies
- Abdominal distention
- Malnutrition
- FTT
How do we test for celiac?
Biopsy of small intestine
How do we test for CF?
Sweat tests–presence of high NaCl concentrations in sweat is a unique characteristic of CF
CF: What is a normal sweat chloride test?
Less than 40 mEq/L
CF: What is the level for infants with CF who had sweat chloride test?
Greater than 60 mEq/L
CF: What is the level for all other ages with CF who had a sweat chloride test?
Greater than 40 mEq/L
What do stools look like for CF patients?
- Frothy
- Foul smelling
- Fat containing
- Float (due to fat content)
What are the fat soluble vitamins?
A, D, E, K
*CF patients have fat containing stools
One of the early signs for CF is meconium ileum. What is this?
Bowel obstruction
CF: Describe meconium ileus characteristics.
Thick, puttylike, tenacious, mucilaginous meconium that blocks the lumen of the small intestine
CF: Meconium ileum blocks the lumen of the small intestine. Where does this usually happen and what does that mean?
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)
What is the diet for CF?
High protein, high calorie
What is the diet for celiac?
Rice, corn, soybean flours
T/F: It is okay to give a child with celiac disease foods with wheat, barley, rye, or oats
NO
What respiratory meds do we give for CF?
- SABA: albuterol
- Anticholinergics: Ipratroprium
- Combo: fluticasone/salmaterol
- Human deoxyribonuclease
- Dornase alfa (decreases viscosity of mucous)
What are some antibiotics we give CF patients?
Tobramycin
Tircarcillin
Gentamycin
What GI meds do we give CF patients?
- Pancreatic enzymes
- Vitamines: multi and ADEK
- Constipation meds: Polyethylene glycol (golytely)
- H2 receptor agonist and motility meds for GERD
Does the US have universal newborn screening for CF?
No, only 2 states offer this
CF Screening: Children who were identified and treated early in infancy with aggressive nutritional support had improved ___ & ___ well into adolescence
Height and weight
If a newborn were to be screened for CF, how is it done?
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
CF: Prevention of pulmonary infection involves a daily routine of CPT to maintain pulmonary hygiene. What is CPT?
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
How often is CPT done on CF patients?
On average twice failing, on rising and in the evening
*and more frequently during pulmonary infection
CF: A small, handheld plastic pipe with a stainless steel ball on the inside that facilitates removal of mucus
Flutter Mucus clearance device
What are the advantages of the flutter mucus clearance device?
Increasing septum expectoration and being used without assistance
CF: What is the cornerstone of pulmonary therapy?
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
The term celiac disease is often used to describe a symptom complex with 4 characteristics. What are those 4 characteristics?
- Steatorrhea
- General malnutrition
- Abdominal distention
- Secondary vitamin deficiencies
Celiac: What does steatorrhea mean?
Fat, frothy, foul, bulky stool
