Cystic Fibrosis

Question 1

Normal infant weight gain

Answer 1

1. Lose up to 10% after birth –> regain by 2 weeks
2. Should be gaining 20-30 g/day
3. Weight doubles by 4 months, triples by 12 months

Question 2

Maternal causes of low weight gain

Answer 2

1. maternal HTN
2. Diabetes
3. Lupus
4. Malnutrition
5. Exposure to substances

Question 3

Failure to Thrive

Answer 3

poor weight gain = lack of physiologic growth and development (weight

Question 4

Etiology of FTT

Answer 4

1. Chronic diarrhea/vomiting
2. CHF
3. Food allergy
4. Poorly prepared formula or not enough
5. GERD, malabsorption
6. Neglect

Question 5

Caloric requirements during first 4 months of life

Answer 5

100-110 Cal/kg/24 hrs

- improperly mixed formula can result in excessive or inadequate intake and electrolyte abnormalities

Question 6

Help make informed decisions

Answer 6

1. Identify concerns
2. Provide adequate information
3. Don’t be judgmental
4. Be impartial

Question 7

DDx for FTT

Answer 7

CHF - difficult feeding and respiratory distress
formula allergy - fussiness, vomiting
gastroenteritis - vomiting, diarrhea
hypothyroidism - poor feeding and constipation
malabsorption - poor weight gain despite good intake

Question 8

Diagnostic Eval for FTT

Answer 8

CBC, UA, BUN/Cr, CXR, stool test, newborn screen, thyroid tests

• heart murmur? -> cardiology eval
• vomiting/diarrhea? -> electrolytes
• no clear etiology? -> sweat test for CF

Question 9

Anemia in infants

Answer 9

Hemoglobin at birth 16.5 –> 2 months 11.5 (short half-life of fetal RBCs)

• iron deficiency
• chronic disease
• hemolysis
• blood loss

Question 10

Sweat chloride

Answer 10

used to diagnose CF since 1959
Sensitivity - 99%, Specificity - 90%
- is apart of a lot of states newborn screens

Question 11

Communicating difficult news

Answer 11

Show empathy, be knowledgeable, private quiet room, ask their perspective, don’t get too in detail, let them ask questions

Question 12

Inheritance of CF

Answer 12

autosomal recessive -> both parents must be heterozygous or have the disease
- genetic counseling may be needed

Question 13

CF management and prognosis

Answer 13

some treatments target the airway inflammation, some treat the genetic abnormalities
Tx = nutritional supplementation, enzymes and extra calories, airway clearance, treatment of airway infections

Question 14

ID individuals with CF

Answer 14

1. Index of suspicion - 10-15% don’t present with pancreatic insufficiency (steatorrhea) –> need high index of suspicion (repeated sinusitis)
2. All 50 states have CF in newborn screen

Question 15

CF centers

Answer 15

Pulmonologist
Nutritionist
Social workers
Respiratory therapists - education on airway clearance and importance of lung health