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

Cystic Fibrosis - Unit 2 Flashcards

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
Q

What is pulmonary hypertension?

A

Increased pressure in pulmonary vessels

26
Q

Cor Pulmonale - what is it?

A

Right ventricular enlargement and failure due to lung disease.

27
Q

What are common organisms found in children with CF?

A

P. aeruginosa, Burkholderia cepacia, S. aureus, H. influenza, E. coli, K. pneumoniae

28
Q

Which organisms cause the most challenges for children with CF?

A

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
Q

What are some respiratory manifestations of CF?

A

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
Q

How do we treat some of the respiratory problems?

A

Preventing infections, exercise, CPT (2 times/day at least)

31
Q

CPT - not time consuming. T/F?

A

FALSE - it is.

32
Q

CPT - includes deep breathing, coughing, huffing, vest clearance system - T/F/

A

True

33
Q

What is Pulmozyme?

A

Aerosolize DNase - it thins mucus.

34
Q

What is huffing?

A

Forced expiration with the glottis partially closed to help move secretions from small airways

35
Q

Vest clearance system - low frequency oscillation helps loosen secretions. T/F?

A

FALSE - it’s HIGH frequency.

36
Q

Bronchodilators (albuterol) are typically used in conjunction with CPT - should they be administered before, during, or after CPT?

A

BEFORE - helps open the bronchi and helps with expectoration.

37
Q

What are some aerosolized antibiotics? When are they given? How do they affect the system? Given where?

A

Given after CPT
Little systemic effects
Tobramycin, ticarcillin, gentamicin
Given at home.

38
Q

Is it okay to give oxygen during acute episodes?

A

Yes - but BE CAREFUL, many kids have a lot of CO2 retention.

39
Q

Monitor for pneumothorax due to bleb rupture. T/F?

A

True

40
Q

Corticosteroids and ibuprofen to counter inflammatory process that leads to lung damage, right?

A

Yes - just be careful with ibuprofen.

41
Q

Lung transplant - used in beginning of the disease?

A

No, advanced disease.

42
Q

How does CF affect the GI tract?

A

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
Q

What nutrients are affected the most when pancreatic enzymes are lacking?

A

Fats (problems with fat-soluble vitamins - ADEK - lack of vitamin K can lead to easy bruising), proteins, carbs (to a lessor extent)

44
Q

What clinical manifestation would indicate the lack of these nutrients?

A

Steatorrhea (fatty stools) - so they have large, loose stools that are EXTREMELY foul smelling, azotorrhea (protein in stools)

45
Q

What are the pancreatic enzymes?

A

Trypsin, Chymotrypsin, Amylase, Lipase

46
Q

What disease process is seen in CF children (usually teens and older) with pancreatic involvement?

A

Type 1 Diabetes

47
Q

If a child with CF has also developed diabetes, he is likely to already have severe pulmonary involvement. T/F?

A

FALSE - there is NO relationship between the progression of lung disease and the development of diabetes.

48
Q

Why can protal hypertension occur in CF?

A

Cirrhosis can result from biliary fibrosis

49
Q

Would the child with CF have a dry or moist mouth?

A

DRY - the salivary glands are also affected - so the dry mouth can increase the susceptibility to infection.

50
Q

What are some other clinical manifestations of CF?

A

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
Q

Meconium ileus at birth - blockage is usually near ___ valve.

A

Ileocecal valve

52
Q

Signs of intestinal obstruction are :

A

abdominal distention, vomiting, dehydration, electrolyte abnormalities, failure to pass stool, intestinal obstruction can also occur in children.

53
Q

Do CF kids have delayed puberty?

A

Yes

54
Q

CF - fertility problems. T/F?

A

TRUE -

Females - thick secretions can block sperm and for males, they are sterile form blockage of vas deferens (but they aren’t impotent)

55
Q

How do we manage the GI issues?

A

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
Q

Pancreatic enzyme replacement - when? how?

A

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
Q

Pancreatic enzymes are adjusted based on what?

A

Stools

58
Q

Stools - the child should have only 3-6 per day. T/F?

A

FALSE - 2-3 per day.

59
Q

CF kids shouldn’t do any exercise. T/F?

A

FALSE - they should. Swimming, running, wheel-barrow races and summersaults, baseball - all good!

60
Q

Why are wheel-barrow races and summersaults great?

A

Assists with postural drainage and mucus clearance, preventing mucus stagnation which leads to pulmonary infection.

61
Q

CF kids should not receive childhood immunizations. T/F?

A

FALSE - they should!

62
Q

When pancreatic enzymes are not taken, the result is stool with a large mount of what?

A

undigested food, fat, and protein.

Peds - Spring 2016 (29 decks)

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