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Clinical Nutrition 1 > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

CF is ___ and affects what?

A

Purely genetic

lungs pancreas, other organs

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2
Q

____ Canadians are CF carriers

A

1/25

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3
Q

How many canadians hav CF?

A

1/3600

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4
Q

How many mutations of CFTR gene?

A

Over 2,000

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5
Q

(T/F) CF is more prominent on females, who tend to be underweight

A

T

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6
Q

What are the chances that two carrier have a child with CF? Without CF? Carrier of CF gene?

A

With: 25%
Without: 25%
Carrier: 50%

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7
Q

What are chances that one carrier and one non-carrier have a child with CF? Carrier of CF?

A

0% with CF but all carriers

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8
Q

Cf is a ___ gene

A

recessive

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9
Q

Class I?

A

No protein made

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10
Q

Class II?

A

Protein is abnormal, and destroyed before it reached the membrane

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11
Q

Class III?

A

Protein reached membrane, but channel is blocked

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12
Q

Class IV?

A

Protein reached membrane, but channel does not work effectively

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13
Q

Class V?

A

Protein reaches membrane, channel works but not enough produced

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14
Q

Most common mutation?

A

Deltaf508 - Class II

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15
Q

What happens when the chloride channel is blocked?

A

Salt and chloride cannot leave the intracellular space, inhibiting water to follow and created the thick mucus layer as water is trapped in the cell

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16
Q

Why is thick mucus as problem?

A

Will physically block organs, and exocrine glands and cause inflammation

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17
Q

What organs does the the thick mucus affect?

A
  • Pancreas
  • Sinuses
  • Reproductive
  • Intestines
  • Airways in lungs
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18
Q

Why do CF patients often have frequent infections?

A

Ineffective clearing of mucus, pathogens and bacteria will become trapped

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19
Q

What happens in the sweat glaands?

A

More NaCl will be excreted, and 2-3 times more sodium is excreted. Can be impacted during hot day’s, fevers, sickness

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20
Q

Symptoms?

A
  • Persistent cough
  • Difficulty with growth (Pancreas affected)
  • Abdominal pain
  • Bloated stomach
  • Salty tasting skin
  • Clubbing of fingers
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21
Q

What is the main reason for malnutrition in CF?

A

Pancreatic insufficiency

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22
Q

How does pancreatic insufficiency lead to malnutrition?

A

Thick mucus blocks endocrine cells, no hormones/enzymes can leave, not uptake of nutrients

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23
Q

What should a CF patient take to combat pancreatic insufficiency?

A

Oral pancreatic enzymes –> Lipase, protease, amylase

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24
Q

When should oral pancreatic enyzmes be taken?

A

Just before eating, and amount may need to be increased if eating more

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25
Q

Are enzymes 100% efficient?

A

No

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26
Q

What is the dosage of pancreatic enzymes based on? Why?

A

Lipase units (LU), as lipids are the hardest to digest

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27
Q

General recommendation for LU?

A

500-4000 LU/gram of fat ingested/day

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28
Q

Pulmozyme =

A

inhaled, thinner mucos

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29
Q

TOBI/Cayston =

A

inhaled antibiotics, alternate to reduce antibiotic resistance

30
Q

Bronchodilators and corticosteroids=

A

Reduce blockage of airways, and reduce inflammation

31
Q

Multivitamins

A

Risk of malnutrition and malabsorption, supplements recommended

32
Q

Vit D and calcium

A

supplements recommended due risk of osteoporosis

33
Q

What other supplements are recommended?

A

-Iron, Zinc, omega-3

34
Q

Prednisone is often prescribed to reduce inflammation and help breathing, why exert caution?

A

Bone thinning, impaired glucose control, water retention

35
Q

What is difficult for Cf patients?l

A

Compliance with medications (normal for all pulmonary disease)

36
Q

What are 3 predictors of non-compliance?

A

1) Long-term treatment
2) Individual is asymptomatic
2) If treatment is complex

37
Q

What are some complications of CF?

A
  • Frequent infections (hospitalization)
  • Nutrient deficiencies (malabsorption)
  • GERD
  • IBS
  • Osteoporosis
  • Liver, heart
  • CF related diabetes
38
Q

Why higher risk of osteoporosis?

A
  • Constantly on steroids

- May not absorb all calcium and Vit D from supplements

39
Q

What is DIOS?

A

Distal Intestinal Obstructive Syndrome, major complication of CF

40
Q

Risk factors for DIOS?

A
  • Severe genotype (first 3 classes)
  • Pancreatic insufficiency
  • Fat malabsorption
  • Dehydration
  • History of meconium ileus or DIOS
  • Organ transplantation
41
Q

What is meconium ileus?

A

First stool thats blocked at birth

42
Q

Symptoms of DIOS?

A
  • Acute onset of sever abdominal pain
  • N/V
  • Inability to pass gas or stool
43
Q

Treatment for an incomplete DIOS?

A

-Oral rehydration with stool softeners and laxatives (PegLyte)

44
Q

Treatment for complete DIOS?

A
  • IV rehydration
  • Nasogastric aspiration
  • Enemas, laxatives
  • May need surgery
45
Q

How should we refeed patient after DIOS?

A

NPO until resolved, and start with clear liquids, full liquids then transition to regular diet

46
Q

What is the issue with DIOS?

A

Patient may have up to a week of suboptimal nutrition

47
Q

Impact on patient after DIOS?

A
  • Anxiety towards food
  • Changes in oral enzymes
  • Keep track of water/fibre
  • Add laxatives
48
Q

How many Canadians with CF have CF related diabetes?

A

22.4%

49
Q

What is the cause of CFRD?

A

Mucus blocks endocrine ducts of pancreas –> pancreatic insufficiency –> no insulin –> diabetes

50
Q

What is the standard for detecting CFRD?

A

Oral glucose tolerance test 1 hour post ingestion

51
Q

What is another symptom of CFRD?

A
  • Inexplicable decline in breathing

- Inability to gain weight (or weight loss)

52
Q

What is malabsorbed in CF?

A

Fat soluble vitamins

53
Q

CF patients often have low Hgb, Hct, Fe and B12 leading to anemia - why?

A
  • Blood loss (IBS)

- Anemia of chronic disease

54
Q

Malabsorption of which nutrient causes acrodermatitis enteropathica, severe diaper rash, decreased appetite and delayed growth?

A

Zinc

55
Q

Live function tests should be obtained yearly for all patients with CF - what values are indicative of CF related liver disease?

A
  • ALT
  • AST
  • Alkaline phosphatase
  • GGT
56
Q

Energy requirement is CF is ___ of that of a healthy individual of same age and gender

A

110-200%

57
Q

Why are E requirements higher in CF?

A
  • Work of breathing/coughing
  • Presence of inflammation
  • Malabsorption
  • Complications
58
Q

There is a positive association between ____ and lung function

A

BMI

59
Q

If there is rapid weight loss, what may we be concerned about in CF patients?

A

Decreased lung function

60
Q

Target healthy BMI females?

A

2

61
Q

Target healthy BMI males?

A

23

62
Q

Target healthy BMI children?

A

> 50% percentile

63
Q

What is the main dietary issue with CF?

A

Increase E requirement with compounding factors that increase difficulty in eating, decreased appetite and absorption

64
Q

What influences eating in CF?

A

-Dyspnea (SOB)
-SInus problems
-Steroids (increase or decrease appetite)
-GI problems
-Slow-intestinal motility
-History of DIOS
Psychological and financial history

65
Q

What is the paradox in infection on nutrition status?

A

Decreased appetite while calorie needs are increases, leading to potential weight loss

66
Q

Nutritional intervention?

A
  • High energy/protein
  • Supplements as snack
  • Adding enteral to oral nutrition
  • Potentially increase sodium
67
Q

(T/F) There has been a major increase in life expectancy amongst CF patients due to better nutrition and treatment

A

T

68
Q

(T/F) Most patients with CF are teenagers

A

F, due to higher survival rates, 60.9% of CF patients are adults

69
Q

Median age of survival?

A

52.3 years

70
Q

How do new medications act?

A

On cellular level to help proper folding and insertion into channels

71
Q

Drugs to treat CF?

A
  • Kalydeco
  • Orkambi
  • Symdeko
72
Q

Which drug specifically treats F508del mutation?

A

Orkambi

Clinical Nutrition 1 (30 decks)

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