Test 3 Ch.15 Cystic Fibrosis Flashcards
(116 cards)
1
Q
The lungs of a pt w/ CF appear normal at birth, but
A
abnormal structures can develop quickly
2
Q
CF is a
A
genetic inheriting disorder
3
Q
Partial obstruction leads to over distention of the ___________.
A
alveoli
4
Q
The anatomic alterations of the lungs associated with CF may result and both….
A
restrictive and obstructive
5
Q
Excessive bronchial secretions, bronchial obstruction, and hyperinflantion of the lungs are features of CF in the…….
A
advance stages
6
Q
The abundance of stagnant mucus in the TB tree serves as a medium for bacteria particularly: (3)
A
- Staphylococcus aureus
- Haemophilus influnzae
- Pseudomonas aeruginosa
7
Q
Some Gram- negative bacteria are also common in CF, such as (2)
A
- Stenotrophomonas maltophilia
- Burkholderia cepacian complex
8
Q
The major respiratory pathologic or structural changes associated w/ CF are: (6)
A
- Excessive production and accumulation of thick tenacious mucus in the TB tree secondary to inadequate hydration of the periciliary fluid layer
- Partial bronchial obstruction (mucus plugging)
- Hyperinflation of the alveoli
- Total bronchial obstruction (mucus plugging)
- Atelectasis
- Bronchiectasis
9
Q
CF is the most common….
A
fatal inherited disorder in childhood
10
Q
CF is an autosomal recessive gene disorder caused by
A
mutations in pair of genes on chromosome 7
11
Q
Both parents must have the gene
A
mutation for child to be diagnosed
12
Q
More than ______ mutations in the gene that encodes for…….
A
1700; Cystic fibrosis transmembrane conductance regulator (CFTR)
13
Q
The abnormal expression of the CFTR results in abnormal transport of ________ and _________ ions across many types of ___________ surfaces including the lining of the bronchial airways ___________, __________, liver ducts and ________ glands.
A
sodium; chloride; epithelial ; intestines; pancreas; sweat
14
Q
As a result of thick viscous, mucus accumulates in the lungs and mucus blocks the passageways of the pancreas prevents enzymes from the pancreas from…….
A
reaching the intestines
15
Q
They are ___ classes of CFTR mutations that can be divided into ___ broad categories affecting either the quantity or function of the _____ protein
A
6;
3;
CFTR
16
Q
CF is a _______ gene disorder
A
recessive
17
Q
The child must inherited ___ copies of the defective CF gene one from…..
A
2
each parent
18
Q
CF gene follows the
A
standard Mendelian pattern
19
Q
According to the pattern there is a ____% chance that each child will have CF; a ___% chance that each child will be completely normal; and a ___% chance that each child will be a carrier
A
25%
25%
50%
20
Q
When both parents carry the CF gene mutation there is a
A
1/4 chance the child will have CF
21
Q
It is estimated that ___ million unknowing carry the gene.
A
10
22
Q
According to the Cystic Fibrosis Foundation, CF affects ___ children and adults in the U.S and about ___ worldwide
A
30,000
70,000
23
Q
The diagnosis of CF is based on the (3)
A
- clinical manifestations associated w/ CF
- family history of CF
- laboratory findings
24
Q
The following two criteria must be met to diagnose CF:
A
- Clinical symptoms consistent w/ CF in at least one organ system
- Clinical evidence of CFTR dysfunction
25
What are the Clinical evidence of CFTR dysfunction to diagnose CF: (3) (test)
- Elevated sweat chloride greater than 60 mEq/L (on two occasions)
- Molecular diagnosis (genetic testing) presence of two disease, causing mutations in CFTR
- Abnormal nasal potential difference
26
Newborn screening for CF has been part of the newborn genetic testing protocol in all 50 states since _____
2011
27
Most infants w/ CF have an elevated blood level of
immunoreactive trypsin
28
What is another name for immunoreactive trypsin
trypsin-like immunoreactive and serum trypsin
29
The sweat test is sometimes called
sweat chloride test
30
The sweat test is the
gold standard diagnostic test for CF
31
The sweat test is a ________ test for the identification of ____% of pts w/ CF
reliable
98%
32
During the sweat test a small amount of colorless, orderless, harmless, sweat- producing called
pilocarpine
33
pilocarpine is applied to the pt's
arm or leg and usually the forearm
34
How many times is the sweat test performed?
2 times
35
An electrode is attached to the chemically area and a mild _______ current is applied to stimulate ______ ___________
electric
sweat production
36
All pts w/ the following characteristics should undergo a sweat test to help confirm CF: (4)
- Infants w/ a positive CF newborn screening
- Infants w/ symptoms of CF
- Older siblings w/ symptoms suggestive of CF
- Members of the pt's family w/ confirmed CF
37
Clinical indicators justifying the initial evaluation for CF, **Pulmonary** (6)
- Wheezing
- Chronic cough
- Sputum production
- Nasal polyps
- Frequent respiratory infections
- Digital clubbing
38
Clinical indicators justifying the initial evaluation for CF **Gastrointestinal Disorders** (5)
- Failure to thrive
- Foul smelling, greasy stools
- Voracious appetite
- Pancreatitis
- Meconium ileus
39
Clinical indicators justifying the initial evaluation for CF, **Nutritional Deficits** (3)
- Fat-soluble vitamin deficiency (A, D, E, K)
- Hypoproteinemia
- Hypochloremia (metabolic alkalosis)
40
Clinical indicators justifying the initial evaluation for CF, **Infertility** (Male)
- Obstructive azoospermia
41
Genetic test is done with a sample of the pt's
blood, or check cells
42
Another name for genetic test (3)
- genotype test
- gene mutation test
- mutation analysis
43
The test can be performed to analyze _____ for the presence of _______ gene mutation
DNA
CFTR
44
Immediate results of sweat chloride test should be further investigated w/ DNA analysis using the
CFTR multimutation method
45
Sweat test interpretations: Infants 6 months or **younger**:
- Normal ( CF unlikely)
- Intermediate (possible CF)
- Abnormal (Diagnosis of CF)
- less than or equal to 29 mmol/L
- 30 to 59 mmol/L
- greater than or equal to 60
46
Sweat test interpretations: Infants **older than 6 months, children and adults**:
- Normal ( CF unlikely)
- Intermediate (possible CF)
- Abnormal (Diagnosis of CF)
- less than or equal to 39 mmol/L
- 40 to 59 mmol/L
- greater than 60 mmol/L
47
The impaired transport of of _______ and _______ across the epithelial cells lining the airways of the pt w/ CF can be measured
sodium (Na+)
chloride (CI+)
48
As the Na+ and CI+ ions move across the epithelia cell membrane they generate what?
Electrical potential difference
49
The amount of energy required to move an electrical charge from one point to another
electrical potential difference
50
In the **nasal** passages this electrical potential difference is called
nasal potential difference (NPD)
51
It is recommended that pregnant females be offered screening for CF mutations using a ____ to ____ mutation panel
32 to 85
52
If females test positive do they have the option to have the father of the fetus tested?
Yes
53
If both parents test positive for a CF mutation, the fetus has a
one in four (25%) chance of having CF
54
This is very important in all cases of prenatal testing for CF to explain this uncertainty or residual risk to prospective parents
Genetic counseling
55
This test measures the amount of fat in the infant’s stool and percentage of dietary fat is not absorbed by the body. What is this test called?
Fecal fat test
56
The fecal fat test is used to evaluate how the ( 4 organs) are functioning
liver, gallbladder, pancreas, and intestines
57
______ __________ is an easier test of the pancreatic function b/c it requires only a ______ amount of stool sample for analysis
Fecal elastance; small
58
Infants w/ CF and pancreatic insufficiency will have a fecal elastance of less than
50 micrograms/ grams (<50) of stool
59
What is normal fecal elastance?
greater than 300 micrograms/grams (>300) of stool
60
The following clinical manifestations results from the pathophysiologic mechanisms caused by (or activated) by (3)
- Atelectasis
- Bronchospasm
- Excessive bronchial secretions
61
The physical examination:
Vitals signs for CF
**Increased RR (Tachypnea)**
- Stimulation of peripheral chemoreceptors (hypoxemia)
- Decreased lung compliance
- Anxiety
- Increased temp
**Increased HR (Pulse) BP**
62
More for physical examination for CF:
- Use of Accessory Muscles During Inspiration and Expiration
- Pursed-Lip breathing
- Increased Anteroposterior Chest Diameter (Barrel Chest)
- Cyanosis
- Digital Clubbing
63
Peripheral Edema and Venous Distention:
B/c polycythemia and cor pulmonale are associated with serve CF, the following may be seen: (3)
- Distended neck veins
- Pitting edema
- Enlarged and tender liver
64
Chest Assessment findings of CF (air 7)
- Decreased or Increased tactile fremitus
- Hyperresonant
- Diminished breath sounds
- Diminished heart sounds
- Bronchial breath sounds (over atelectasis)
- Crackles
- Wheezes
65
Some type of injury to the lung that causes a puncture, and air gets out of the lung into the pleural space
Pneumothorax
66
No obvious injury that should have caused the pneumothorax
Spontaneous pneumothorax
67
SP is commonly seen in pts w/
CF
68
The incidence of a SP is greater than ___% in adults w/ CF
20%
69
When a pt w/ CF has a pneumothorax, there is a.....
50% chance that it will recur
70
Abnormal Lab Test and Procedures: **Hematology (RBC)**
- Increased hematocrit and hg
- Increased WBC count
71
Electrolytes for CF:
- Hypochloremia (chronic vent failure)
- Increased serum bicarbonate (chronic vent failure)
72
Sputum Examination for CF:
- Increased WBC
- Gram- positive bacteria (Staphylococcus aureus, Haemophilus influezae)
- Gram- negative bacteria (Pseudomonas aeruginosa, Stenotrophomonas maltophilia, Burkholderia cepacian complex)
73
Radiologic Findings: Chest Radiograph (7)
- Translucent (dark) lung fields
- Depressed or flattened diaphragms
- Right ventricular enlargement
- Areas of atelectasis and fibrosis
- Tram tracks
- Bronchiectasis
- Pneumothorax
74
____ is the #1 cause of ______________.
CF; Bronchiectasis
75
An obstruction of the small intestine of the newborn that is caused by the impaction of thick and dry, tenacious meconium, usually at or near the ileocecal valve
Meconium ileus
76
Meconium ileus may occur in ___% of infants w/ CF
25%
77
Previously know as Meconium ileus equivalent is an obstruction that occurs in older children and young adults w/ CF (clinical instructor)
Distal Intestinal Obstruction Syndrome (DIOS)
78
About 80% of all pts w/ CF have a ____________ deficiency
vitamin
79
Nasal polyps are seen in (%)
10% and 30% pts w/ CF
80
90% and 100% of pts w/ CF have
sinusitis
81
About ____% of men w/ CF are infertile
99%
82
Women w/ CF who become pregnant may not be able to
carry the infant to full term
83
An infant who is carried to full term may have
CF or be a carrier
84
The primary goals for management of CF are to: (4)
- Prevent pulmonary infections
- Reduce the amount of thick bronchial secretions
- Improve airflow
- Provide adequate nutrition
85
What does Dornase Alpha do in a pt w/ CF?
Breaks down the DNA in CF
86
What are the Respiratory Protocols in CF? (5)
- Oxygen Therapy
- Airway Clearance Therapy
- Lung Expansion Therapy
- Aerosolized Medication
- Mechanical Ventilation
87
Is used to treat hypoxemia, decrease myocardial work in pts w/ CF with advance pulmonary disease or durning acute exacerbations
O2 Therapy Protocol
88
B/c of excessive mucus production and retention of secretions, this is used to mobilize bronchial secretions
Airway Clearance Therapy
89
What are some examples of Airway Clearance (4)
- PEP Therapy
- CPT
- Mechanical percussor and postal drainage
- High- frequency chest wall oscillation (HFCWO)
90
What is the vest therapy called?
High-frequency chest wall oscillation (HFCWO)
91
May be administered to help w/ atelectasis. What protocol is this?
Lung Expansion Therapy
92
A variety of bronchodilators and mucolytic agents are commonly used to induce bronchial smooth muscle relaxation and mucous thinning. What protocol is this?
Aerosolized Medication Protocol
93
In pts w/ CF, Bronchodilators should be given
- Immediately before the pt receives CPT
- Immediately before pt receives inhalation of nebulizer medications
94
What are recommend bronchodilators for CF?
SABA (albuterol) or LABA (salmeterol or formoterol)
95
Also anticholinergic agents....
ipratropium bromide and longer acting tiotropium
96
Mucolytic agents (3)
- Inhaled DNase
- Inhaled hypertonic saline
- Inhaled N- acetylcysteine
97
Brand name for DNase (dornase alpha)
Pulmozyme
98
This may be administered to help hydrate thick mucus in the airways in pts w/ CF
Inhaled hypertonic saline
99
This protocol is justified when acute ventilatory failure is thought to be reversible
Mechanical Ventilation Protocol
100
These are drugs that help mutated CFTR **reach the epithelial cell surface** where the CFTR protein normally functions as a transmembrane regulator of chloride movement out of the cell and sodium transport into the cell
Correctors
101
These are drugs that help mutated CFTR **function more effectively** at the epithelial cell surface transporting chloride out of the cell and inhibiting the movement of sodium into the cells
Potentiators
102
Commonly administered to prevent or combat chronic respiratory tract infections
Antibiotics
103
Inhaled antibiotics widely used to treat P. aeruginosa in Cf includes (2)
- inhaled tobramycin (Bethkis)
- inhaled aztreonam
104
This is used to slow down the destruction of the lungs
Ibuprofen
105
**Questions from the back**
Which of the following organism are commonly found in the TB tree secretions of pts w/ CF (3)
- Staphylococcus
- Haemophilius influzae
- Pseudomonas aeruginosa
106
When 2 carriers of cystic fibrosis produce children, there is a:
- 25% chance the baby will be completely normal
- 25% chance the baby will have CF
107
The cystic fibrosis gene is located on which chromosome?
7
108
In CF the pt commonly demonstrates which of the following? (3)
- Decreased MVV
- Increased RV
- Decreased FEV1/ FVC ratio
109
During the advanced stages of CF the pt generally demonstrates which of the following? (3)
- Bronchial breath sounds
- Diminished breath sounds
- Hyperresonant percussion notes
110
About 80% of all pts w/ CF demonstrates a deficiency in which vitamins?
A, D, E, K
111
What agents targets the underlying cause of CF, the faulty gene G551D, and its defective CFTR protein?
Ivacafor
112
Which of the following are mucoltic agents (3)
- DNase
- Pulmozyme
- Dornase alpha
113
With regard to the secretion of sodium and chloride, the sweat glands of pts w/ CF secrete up to:
4 times the normal amount
114
Which of the following is associated w/ severe CF?
- Increased central venous pressure
- Decreased breath sounds
- Increased pulmonary vascular resistance
115
What are the gram-positive bacteria seen in CF pts (2)
- Staphylococcus aureus
- Haemophilus influezae
116
What are gram- negative bacteria found in CF pts (3)
- Pseudomonas aeruginosa
- Stenotrophomonas maltophilia
- Burkholderia cepacian complex
