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NUTR 344 > Cystic fibrosis > Flashcards

Cystic fibrosis Flashcards

1
Q

Is CF an acquired or genetic disease?

A

purely genetic

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2
Q

Which organs are affected by CF?

A

lungs and the pancreas

Can cause other complications and affect other organs

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3
Q

What is the prevalence of CF carriers

A

1 in 25

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4
Q

What is the chance of having a child with cystic fibrosis by carrier parents?

A

1/4 chance of having a child with cystic fibrosis by carrier parents

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5
Q

Is CF gene dominant or passive

A

passive

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6
Q

Outcomes of CC and cc parent (CF and non-carrier)

A

100% of a healthy child of 1 CC parent and 1 absolutely normal parent

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7
Q

What are the classes of CF?

A

First 3 are the most severe
I- No protein is 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 does not work effectively
V- Protein reaches membrane and works properly but not many are made

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8
Q

lung cells with and without CF

A

Chloride channel is affected, causing Cl to be trapped within the cell. This causes salt to stay inside the cells causing less water to be transported out of the cell (water follows Na)
This results in a sticky, concentrated mucus

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9
Q

Why is thick mucus a problem?

A

Thick mucus can block several organs:
•Pancreas
•Sinuses
•Reproductive -> infertility
•Intestines-> obstruction
•Airways in the lungs-> difficulty breathing
Thick mucus can accumulate, get infected and cause inflammation. The perfect storm for bacterial growth!

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10
Q

Symptoms of 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

A

Pancreatic Insufficiency (84% prevalence of insufficiency)

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12
Q

What is the reason of pancreatic insufficiency in CF?

A

It occurs because the thick mucus in your pancreas blocks pancreatic enzymes from entering the small intestine

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13
Q

Why are there different brands of pancreatic enzymes available?

A

various tolerance by different subjects

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14
Q

What are the enzymes in pancreatic enzyme drugs?

A

They contain lipase, protease and amylase

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15
Q

When should pancreatic enzymes be taken and what does the dose depend on?

A
  • Need to be taken just before eating

- Number of pills may need to be adjusted depending on the meal/snack

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16
Q

What are the other management tools for CF and why?

A

 Pulmozyme- sed to improve lung function in people with cystic fibrosis by thinning pulmonary secretions and reducing the risk of respiratory tract infections. Inhaled
 Tobramycin (TOBI) and Cayston- antibiotic
 Azithromycin
 Bronchodilators or corticosteroids
 Multivitamins- due to digestion problems and low absorption of vitamins
 Vitamin D and Calcium
 Iron supplements, Zinc, Omega-3 as required
 Prednisone as needed- glucocorticoid medication mostly used to suppress the immune system and decrease inflammation
 Lax-a-day, nasal wash as needed
 Physiotherapy- to clear out lung mucus

17
Q

What are the 3 factors that dictate the compliance to treatment? How do they affect CF treatment compliance

A

1) Long-term-> CF treatment is very long-term
2) Asymptomatic - CF is often asymptomatic
3) Complex

Low compliance with CF treatment

18
Q

Complications of CF

A

 Frequent infections requiring hospitalization- as mucus harbours bacterias
usually 3-4 per year
 Nutrient deficiencies due to malabsorption
 Gastro-oesophageal reflux disease
 Inflammatory Bowel diseases
 Osteoporosis- due to decreased absorption and digestion of food and supplement Vit D
plus the use of steroids to help with inflammation which are very blood thinning
 Intestinal obstruction (DIOS)
 Liver disease (ex: fatty liver)
 Kidney disease
 Heart problems
 CF related diabetes- due to thick mucus scarring on pancreas; This scarring prevents the pancreas from producing normal amounts of insulin; so, like people with type 1 diabetes, they become insulin deficient.

19
Q

What is Distal Intestinal Obstructive Syndrome (DIOS)

A

involves blockage of the intestines by thickened stool

20
Q

What are the risk factors for DIOS?

A

Risk factors include severe genotype, pancreatic insufficiency, fat malabsorption, dehydration, history of meconium ileus (obstruction at birth_ or DIOS, organ transplantation, etc.

21
Q

Speed of symptoms appearance constipation vs intestinal obstruction

A

obstruction symptoms are very soon to show

constipation symptoms are slower to show and the condition is not as severe

22
Q

Symptoms of DIOS

A

Symptoms include acute onset of severe abdominal pain, nausea, vomiting (including bilious material) and inability to pass gas or stool.

23
Q

Treatment for DIOS

A

Incomplete DIOS:
 Oral rehydration with stool softeners and laxatives (ex. PegLyte)

Complete DIOS:
 IV rehydration
 Nasogastric aspiration
 Enemas, laxatives
 Surgery may be required

patient may be NPO during obstrucutin
have to refeed slowly-> clear liquid to start

24
Q

Precautions after DIOS

A

 Patient may have anxiety towards certain foods or eating in general
 Changes may need to be made on enzyme dosage
 Patient may need to keep track of their water or fiber intake
 Patients may have to add laxatives to their usual routine

25
Q

What can help detect worsening of disease

A

Research is suggesting that the 1 hr post reading in an OGTT may better help detect worsening of disease

26
Q

What are two symptoms that will help the team determine action plan for CFRD.?

A

Inexplicable decline in breathing function and inability to gain weight (or weight loss)

27
Q

CF assessment tests

A

 Breathing test at each visit
 Weight monitoring at each visit- rapid weight loss can occur-> loss of lean body mass
 X-ray of lungs or abdomen- phenumonia, mucus plugging
 Blood tests yearly and/or during exacerbations
 Dexa scan- done yearly to monitor for osteoporosis
 Ultrasound
 ECG
 OGTT yearly
 Colonoscopy if IBD present

28
Q

What are the possible nutrient abnormalities in CF

A

Vitamin A, D, E,K deficiencies due to Malabsorption
Hgb, Hct, Fe, B12
Zinc deficiency due to malabsorption
Malabsorption
Increased AST, Alt, Alkaline phosphatase, GGT due to CF related liver disease
Increased Glucose due to CF related diabetes, glucose intolerance

29
Q

Why is malnutrition so common in CF

A
  • Energy requirement is said to be 110%-200% of that of a healthy individual of same age and gender
  • Energy requirements are higher due to many factors like higher work of breathing/coughing, the presence of inflammation, malabsorption, complications, etc.
  • Pancreatic insufficiency-> poorer digestion
30
Q

Research suggests a __ association between BMI and lung function.

A

Research suggests a positive association between BMI and lung function.

31
Q

WHat are the recommended BMI for CF patients?

A

BMI >50% for child

BMI 20 of females and BMI of 25 for males due to increased need for energy storage to cope wiht abnormalitities

32
Q

Factors that might influence eating In CF: What does it lead to?

A

 Presence of Dyspnea- shortness of breath
 Sinus problems
 Medications (steroids)
 Presence of GI problems
 Slow intestinal motility- result in fuller feeling for longer
pro-motility agent can be given
 History of DIOS

all this contributes to difficulty to supply sufficient calories

33
Q

Why does infection lead to weight loss

A

1) Decreased appetite

2) Increased calorie needs

34
Q

Meal Plan for CF

A

 High protein, High Energy Diet
 Teach patients/parents ways to increase energy/protein in each recipes (ex: adding milk powder to shakes, adding cream in soups)
 Supplements added to diet as snacks (Scandishake, Ensure plus, Boost, etc.) !!not as a meal replacement!!
 Enteral nutrition may be necessary

35
Q

is increased sodium intake advised to CF patients

A

only after strenuous exercise

36
Q

What are the causes of death in CF

A

pulmonary exacerbation and malnutrition are the 2 main factors that result in death

37
Q

A CF patient developed diabetes probably due to:

a) Being overweight
b) Being inactive
c) Mucus causing damage to the pancreas
d) Having a stressful job

A

c) Mucus causing damage to the pancreas

38
Q

A CF patient keeps complaining of steatorrhea and weight loss. As a dietitian, you should:

a) Change pt’s diet to a low fat diet
b) Change number of pancreatic enzymes pt consumes before anything
c) Ask pt about their compliance first and if necessary adjust enzymes
d) Decrease number of meals per day that pt consumes

A

c) Ask pt about their compliance first and if necessary adjust enzymes

NUTR 344 (22 decks)

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