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Clinical Nutrition 1 > Cystic fibrosis > Flashcards

Cystic fibrosis Flashcards

1
Q

Define cystic fibrosis

A
  • genetic disease
  • affects lungs and pancreas
  • can cause other complications and affect other organs
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2
Q

How many Canadians are:

  • carriers of CF
  • have CF
A
  • carriers= 1/25

- have CF= 1/3600

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3
Q

What is the common mutation of the CFTR gene called

A

deltaF508

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4
Q

T/F

In 2018, 11.7% of female adults and 7.5% of male adults with CF are classified as overweight

A

F

Classified as underweight

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5
Q

If 2 parents are carrier of CF gene, what is the probability that child:

  • develops CF
  • carries CF gene
  • doesn’t have CF
A
  • 25% prob develops CF
  • 50% prob carries CF gene
  • 25% prob doesn’t have CF
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6
Q

Can a person with CF and a no,-carrier of CF have a normal child?

A

Yes

0% prob that the child will have CG (will always be carrier)

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7
Q

What are the 5 classes of CFTR mutations and the effect on protein

A

I- no protein made
II- protein is abnormal and destroyed before it reaches membrane
III- protein reaches membrane but channel is blocked
IV- protein reaches membrane but channel doesn’t work properly
V- protein reaches membrane and works properly but not many are made

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8
Q

Which mutation classes are the most severe?

A

Classes I, II, III

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9
Q

Why is thick mucus a problem?

A
  • thick mucus can block several organs (pancreas, sinuses, intestines, reproductive, airways)
  • thick mucus can accumulate, get infected and cause inflammation
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10
Q

Symptoms of a patient with CF

A
  • persistent cough
  • difficulty with growth
  • abdominal pain
  • bloated stomach
  • salty tasting skin
  • clubbing of fingers
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11
Q

What is the main reason for malnutrition in CF patients?

A

Pancreas insufficiency

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12
Q

What do pancreatic enzymes contain

A

lipase, protease, amylase

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13
Q

Different brands of pancreatic enzymes available

A

Creon 5,10,25, Pancrease, Viokase, Ultrase

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14
Q

When should pancreatic enzymes be taken?

A

Just before eating

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15
Q

Other management tools available for CF patients

A
  • Pulmozyme
  • Tobramycin and Cayston
  • Azithromycin
  • Bronchodilators or corticosteroids
  • Multivitamins
  • Vitamin D and Calcium
  • Iron supplements, Zinc, Omega 3
  • Prednisone as needed
  • Lax-a-day, nasal wash as needed
  • Physiotherapy
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16
Q

Predictors for non-compliance

A
  • Long-term
  • Asymptomatic
  • Complex
17
Q

Complications that can occur in CF patients are:

A
  • frequent infections requiring hospitalization
  • nutrient deficiencies due to malabsorption
  • gastro-oesophageal reflux disease
  • Inflammatory Bowel diseases
  • osteoporosis
  • intestinal obstruction (DIOS)
  • liver disease
  • kidney disease
  • heart problems
  • CF related diabetes
18
Q

What are risk factors for developing DIOS (Distal Intestinal Obstructive Syndrome)

A 
severe genotype
pancreatic insufficiency 
fat malabsorption
dehydration
history of meconium ileum
organ transplantation
19
Q

Symptoms related to DIOS (Distal Intestinal Obstructive Syndrome)

A

ACUTE (fast) onset of severe abdominal pain, nausea, vomiting (including bilious material) and inability to pass gas or stool

20
Q

Treatment for incomplete DIOS

A

-oral rehydration with stool softeners and laxatives

21
Q

Treatment for complete DIOS

A
  • IV rehydration
  • nasogastric aspiration
  • enemas, laxatives
  • surgery may be required
22
Q

What changes after DIOS in the patient

A

-more anxiety towards food and eating

23
Q

What medical/dietary changes should be made after a DIOS

A
  • changes on enzyme dosage
  • keep track of water or fiber intake
  • add laxatives
24
Q

2 symptoms that help determine a plan for CF related-diabetes

A
  • inexplicable decline in breathing functions

- inability to gain weight (or weight loss)

25
Q

Assessments tests performed on CF patients

A
  • breathing test at each visit
  • weight monitoring at each visit
  • X-ray of lungs or abdomen
  • blood tests yearly and/or during exacerbations
  • Dexa scan
  • ultrasound
  • ECG
  • OGTT yearly
  • colonoscopy if IBD present
26
Q

Which possible vitamin deficiencies could occur when you have CF?

A

-vitamin A, D, E, K deficiencies because are fat soluble

27
Q

What is the energy requirement of an individual with CF?

A

110-200% of that of a healthy individual of same age and gender

28
Q

Why are energy needs higher in CF patients ?

A
  • due to higher work of -breathing/coughing
  • presence of inflammation
  • malabsorption
  • complications
29
Q

What BMI should you aim for a women and a man with CF

A

Women: 22
Man: 23

30
Q

Which factors might influence eating in patients with CF

A
  • presence of dyspnea
  • sinus problems
  • medications (steroids
  • GI problems
  • slow intestinal motility
  • history of DIOS
31
Q

Why is there weight loss in patients with CF

A

Infection –> increased kcal needs and decreased appetite –> potential weight loss

32
Q

Typical meal plan for CF patient

A
  • high protein, high energy diet

- supplements added to diet as snacks

33
Q

What should be taught to CF patients?

A

Teach ways to increase energy/protein in each recipes (ex: adding milk powder to shakes)

34
Q

What is the median age of survival of patient with CF

A

52.1 years

35
Q

What should always be monitored in a patient with CF

A
  • weight

- nutrient levels in blood

36
Q

What drug has been approved for 11 different mutations and by Health Canada ?

A

Kalydeco (ivacaftor)

37
Q

What is a new drug combination to help bpateints with 2 comes of the F508 delta mutation

A

Orkambi

also Symdeko more recently

38
Q

What is the problem with Symdeko and Trikafta

A

Very $

39
Q

On a hot summer day what would be the cancer for a patient with CF?

A
  • sodium levels decreasing in the body

Clinical Nutrition 1 (13 decks)

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