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Peds - Spring 2016 > Cystic Fibrosis - Unit 2 > Flashcards

Cystic Fibrosis - Unit 2 Flashcards

(62 cards)

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1
Q

Cystic Fibrosis - defective gene? If so, where is it?

A

Inherited defective gene, located on chromosome 7.

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2
Q

Cystic fibrosis - leads out to defective chloride ion transport. T/F?

A

True

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3
Q

Both parents cannot be carriers. T/F?

A

FALSE - they ARE.

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4
Q

If both parents are carriers, there is a ____ % chance they will have an affected child, ___ % chance the child will be a carrier, and a ___% chance the child will not be a carrier or have the disease.

A

25%
50%
25%

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5
Q

Is there universal screening for cystic fibrosis?

A

No - just DNA analysis.

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6
Q

Cystic fibrosis - dysfunction of the exocrine glands that are the ___ producing glands.

A

Mucus

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7
Q

CF - more prevalent in blacks. T/F?

A

FALSE - usually whites. 95% are white!

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8
Q

__ in ___ caucasians are symptom free carriers.

A

1 in 29

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9
Q

CF mostly affects epithelia cells in the airway and pancreas. T/F?

A

True

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10
Q

CF - no abnormal transport of sodium and chloride. T/F?

A

FALSE

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11
Q

What’s the life expectancy? How many are 18 and older?

A

1966 - 7.5 years.
1996 - 31 years.

40% are 18 years and older

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12
Q

How has life expectancy increased?

A

improved therapies, CPT, gene therapy, aerosolized antibiotics, improved nutrition.

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13
Q

Increased viscosity of mucus secretions - - what does that do?

A

Obstructs small passageways of bronchioles and pancreas.

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14
Q

Increases sodium and chloride in saliva and sweat - T/F?

A

True

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15
Q

Sodium and chloride in sweat is 2-5 times greater than children without CF. T/F?

A

True

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16
Q

Kids with CF can taste salty when kissed. T/F?

A

True

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17
Q

Do CF kids have autonomic nervous system abnormalities?

A

Yes

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18
Q

What is the sweat chloride test?

A

Pilocarpine iontophoresis - 2 separate samples. Sweat production is stimulated with electric current and then that sweat is collected on filter paper.

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19
Q

Sweat test - easy on newborns. T/F?

A

FALSE - they don’t really sweat!

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20
Q

What’s the normal sweat chloride test?

A

60 meq/L

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21
Q

No correlation between severity of disease and amount of electrolytes found in sweat. T/F?

A

True

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22
Q

Cilia in the lung have difficulty moving the thick mucus - T/F?

What happens then?

A

True - so they do not effectively cough and remove secretions.

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23
Q

Since they can’t cough well and remove secretions, what happens? hint, think of leaving shit in there….

A

Infection - mucus serves as a medium for bacterial growth!

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24
Q

In addition to hypoxia, hypercarbia, and acidosis, what else might occur in the child with severe lung involvement secondary to CF?

A

Pulmonary Hypertension and Cor Pulmonale

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25
What is pulmonary hypertension?
Increased pressure in pulmonary vessels
26
Cor Pulmonale - what is it?
Right ventricular enlargement and failure due to lung disease.
27
What are common organisms found in children with CF?
P. aeruginosa, Burkholderia cepacia, S. aureus, H. influenza, E. coli, K. pneumoniae
28
Which organisms cause the most challenges for children with CF?
Burkholderia cepacia (can rapidly lead to sepsis and rapid pulmonary function deterioration) and P. aeruginosa (pseudomonas, difficult for the macrophages to destroy and develops resistance to antibiotics)
29
What are some respiratory manifestations of CF?
Wheezing, coughing (chronic), a cough that can begin as dry and non productive, respiratory infections, increased dyspnea, hyperinflation and areas of atelectasis, barrel shaped chest, cyanosis, finger and toe clubbing
30
How do we treat some of the respiratory problems?
Preventing infections, exercise, CPT (2 times/day at least)
31
CPT - not time consuming. T/F?
FALSE - it is.
32
CPT - includes deep breathing, coughing, huffing, vest clearance system - T/F/
True
33
What is Pulmozyme?
Aerosolize DNase - it thins mucus.
34
What is huffing?
Forced expiration with the glottis partially closed to help move secretions from small airways
35
Vest clearance system - low frequency oscillation helps loosen secretions. T/F?
FALSE - it's HIGH frequency.
36
Bronchodilators (albuterol) are typically used in conjunction with CPT - should they be administered before, during, or after CPT?
BEFORE - helps open the bronchi and helps with expectoration.
37
What are some aerosolized antibiotics? When are they given? How do they affect the system? Given where?
Given after CPT Little systemic effects Tobramycin, ticarcillin, gentamicin Given at home.
38
Is it okay to give oxygen during acute episodes?
Yes - but BE CAREFUL, many kids have a lot of CO2 retention.
39
Monitor for pneumothorax due to bleb rupture. T/F?
True
40
Corticosteroids and ibuprofen to counter inflammatory process that leads to lung damage, right?
Yes - just be careful with ibuprofen.
41
Lung transplant - used in beginning of the disease?
No, advanced disease.
42
How does CF affect the GI tract?
Varies..secretions can block the ducts and lead to fibrosis of the small glands, which PREVENTS pancreatic enzymes from reaching the duodenum (which leads to impaired digestion and absorption)
43
What nutrients are affected the most when pancreatic enzymes are lacking?
Fats (problems with fat-soluble vitamins - ADEK - lack of vitamin K can lead to easy bruising), proteins, carbs (to a lessor extent)
44
What clinical manifestation would indicate the lack of these nutrients?
Steatorrhea (fatty stools) - so they have large, loose stools that are EXTREMELY foul smelling, azotorrhea (protein in stools)
45
What are the pancreatic enzymes?
Trypsin, Chymotrypsin, Amylase, Lipase
46
What disease process is seen in CF children (usually teens and older) with pancreatic involvement?
Type 1 Diabetes
47
If a child with CF has also developed diabetes, he is likely to already have severe pulmonary involvement. T/F?
FALSE - there is NO relationship between the progression of lung disease and the development of diabetes.
48
Why can protal hypertension occur in CF?
Cirrhosis can result from biliary fibrosis
49
Would the child with CF have a dry or moist mouth?
DRY - the salivary glands are also affected - so the dry mouth can increase the susceptibility to infection.
50
What are some other clinical manifestations of CF?
Failure to thrive, weight loss despite increase appetite, rectal prolapse from large stools and increase abdominal pressure from chronic cough, meconium ileus at birth
51
Meconium ileus at birth - blockage is usually near ___ valve.
Ileocecal valve
52
Signs of intestinal obstruction are :
abdominal distention, vomiting, dehydration, electrolyte abnormalities, failure to pass stool, intestinal obstruction can also occur in children.
53
Do CF kids have delayed puberty?
Yes
54
CF - fertility problems. T/F?
TRUE - Females - thick secretions can block sperm and for males, they are sterile form blockage of vas deferens (but they aren't impotent)
55
How do we manage the GI issues?
Pancreatic enzyme replacement, high calorie/high protein diet, may use tube feedings at night, multi-vitamins as well as ADEK, additional salt to diet.
56
Pancreatic enzyme replacement - when? how?
At the beginning of meals and snacks, can be swallowed or taken apart and sprinkled over small amount of food, extra for fatty foods!!!!!
57
Pancreatic enzymes are adjusted based on what?
Stools
58
Stools - the child should have only 3-6 per day. T/F?
FALSE - 2-3 per day.
59
CF kids shouldn't do any exercise. T/F?
FALSE - they should. Swimming, running, wheel-barrow races and summersaults, baseball - all good!
60
Why are wheel-barrow races and summersaults great?
Assists with postural drainage and mucus clearance, preventing mucus stagnation which leads to pulmonary infection.
61
CF kids should not receive childhood immunizations. T/F?
FALSE - they should!
62
When pancreatic enzymes are not taken, the result is stool with a large mount of what?
undigested food, fat, and protein.

Peds - Spring 2016 (29 decks)

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