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Pharmacology II > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

What is the most common lethal genetic disorder in the caucasian population?

A

Cystic fibrosis

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2
Q

What is the average life expectancy for cystic fibrosis?

A

27.4 years

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3
Q

How is cystic fibrosis passed on?

A

Autosomal recessive genetic disease

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4
Q

What glands does cystic fibrosis mainly involve?

A

Exocrine glands

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5
Q

What is the pathophysiology of the cystic fibrosis transmembrane regulator (CFTR) protein?

A

Cystic fibrosis transmembrane regulator (CFTR) protein
Chloride channel in the epithelia of most of the lumens of the body
Transport chloride with accompanying sodium and water
Significant contributor to sodium and water balance
CF is caused by mutations that lead to dysfunction of CFTR protein
More than 1500 mutations

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6
Q

What is the pathophysiology of the CFTR protein dysfunction?

A

Defective electron transport
Decreased chloride secretion and increased sodium absorption

Increase in viscosity of secretions

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7
Q

Is cystic fibrosis the result of a genetic mutation?

A

Yes involves a 3 base pair deletion

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8
Q

What are the organ systems involved in cystic fibrosis?

A 
Gastrointestinal tract
Pulmonary system
Hepatic system
Reproductive system
Bone and joint system
Sweat glands
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9
Q

How does cystic fibrosis present?

A 
Failure to thrive 
Recurrent pneumonia
Meconium ileus
Nasal Polyps 
Uncontrolled asthma
Chronic sinusitis 
Salty taste to skin
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10
Q

How is a diagnosis made for cystic fibrosis?

A 
One or more characteristic signs/symptoms, history of CF in sibling, or a positive newborn screening test
AND
Evidence of CFTR abnormality
Elevated sweat chloride test X 2
Known CFTR mutations
> 70% diagnosed by age 2 years
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11
Q

What is the sweat test consist of?

A

Sample of sweat is collected and concentration of chloride is determined
Positive test is > 60 mmol/L in children and adults

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12
Q

What does the newborn screening measure?

A

Measures immunoreactive cationic trypsinogen (pancreatic enzyme precursor)

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13
Q

What is the goal of therapy?

A

Lead a normal, healthy, active life

CF foundations guidelines require quarterly visits

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14
Q

What should be done at quarterly visits?

A

Vital signs, history, physical exam
Review of therapies (pharmacologic/nonpharmacologic)
Detailed dietary history (stooling/appetite)
Spirometry
Lab work

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15
Q

What complications arise in the GI tract from cystic fibrosis?

A

Deficient secretion of digestive enzymes- Maldigestion of ingested nutrients

Fat-soluble vitamin deficiency

Insulin deficiency

Intestinal obstruction- Meconium ileus, Distal intestinal obstruction syndrome (aka meconium ileus equivalent)

Reflux

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16
Q

What is the presentation for maldigestion of nutritients?

A

Steatorrhea and malnutrition

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17
Q

What is steatorrhea?

A

Stools are foul smelling, bulky, greasy, abnormally high number/day

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18
Q

What is the presentation of malnutrition?

A

Below age-related norms for both height and weight

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19
Q

What are the sx of pancreatic insuffiency caused by cystic fibrosis?

A

steatorrhea, frequent loose stools, flatulence, cramping, bloating, voracious appetite

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20
Q

How is pancreatic insufficiency diagnosed in cystic fibrosis?

A

72 hour fecal fat collection

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21
Q

What is enzyme deficiency caused by in cystic fibrosis?

A

Due to mucous plugging and damage to the pancrease

Amylase, protease and lipase are not available to small intestine

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22
Q

What is the therapy for enzyme deficiency in CF?

A

Pancreatic enzyme replacement
Creon®, Zenpep®, Pancreaze®
—Contain lipase, protease, amylase

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23
Q

When should enzyme replacement be taken?

A

Taken with each meal or snack due to this is when the body normally releases these enzymes and if they arent able to then you need to take them at this time.

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24
Q

Pancreatic enzyme replacement- ADRs

A

Nausea, abdominal cramps, constipation, diarrhea, greasy stools, flatulence
Reports of fibrosing colonopathy reported at high doses

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25
Q

Pancreatic enzyme replacement- clinical issues

A

Inadequate enteric coating and poor acid resistance: denaturing of enzymes
Case reports of poor patient outcomes when switched between brand and generic enzymes

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26
Q

Should you fill out the no substitute line when filling out a prescription for pancreatic enzyme replacement?

A

YES ALWAYS

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27
Q

Do pancreatic enzyme replacements now have to be approved by the FDA?

A

Yes or they are pulled from the market

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28
Q

What is the chronic therapy for GI sx associated with CF?

A
  • -High caloric diet- 120-150% nutritional supplements, often require nutritional supplements
  • -Fat soluble vitamin replacement - ADEK
  • -May require extra salt
29
Q

Can CF patients develop diabetes? And if so what do you treat it with?

A

Yes and you treat with insulin

30
Q

What is the complication with GI obstruction in CF patients?

A

Manifested as distal intestinal obstruction syndrome (DIOS_

31
Q

What are the sx and therapy for distal intestinal obstruction syndrome (DIOS)?

A

Vomiting, abdominal distention, pain

Therapy- electrolyte lavage solutions (Endpoint is passage of stool, sx resolution)

32
Q

What is the cause of GERD and the treatment for patients with CF?

A

May be due to recurrent/chronic cough, hyperinflation

Treatment
Antacids
H2 blockers
Proton pump inhibitors

33
Q

What are the liver complications associated with CF?

A

Biliary fibrosis, bile duct proliferation, chronic inflammatory infiltration

34
Q

How do you detect liver damage in patients with CF?

A

↑ GGT may reflect liver damage even if other liver enzymes are normal

35
Q

Ursodeoxycholate

A

Decreases liver inflammation and bile duct proliferation

36
Q

What is the system most affected by CF?

A

Pulmonary system with pulmonary disease

37
Q

What are the manifestations in the pulmonary system a result of?

A

accumulation of viscous mucus in the small airways

38
Q

What are the consequences to the accumulation of viscous mucus in the small airways of the pulmonary system for CF?

A

Obstruction
Infection
Inflammation
Chronic rhinitis, sinusitis, nasal polyps

39
Q

What is the goal for the pulmonary system in CF patients?

A

decrease the long-term rate of lung function decline

40
Q

What is used to decrease the long-term rate of lung function decline in CF patients?

A 
Airway clearance techniques (ACT)- important and will be part of the daily regimen
Anti-inflammatory agents
Chronic antibiotics
Mucolytics
Bronchodilators
41
Q

ACT- Airway clearance techniques

A 
Improve ventilation, reduce accumulation of secretions
Done at least BID
Includes:
Chest/back percussions followed by vigorous coughing
Flutter valve
Exercise
Mechanical vest
Bronchodilator pre-treatment optional
42
Q

Oral corticosteroids

A

Undesirable side effects

43
Q

NSAID agents- ibuprofen

A

Decreased rate of decline of FEV1
5-12 years with mild lung disease (FEV1> 60%)
Peak plasma concentrations of 50-100 mg/L

44
Q

Anti-inflammatory therapy- azithromycin

A 
Given 3X/week
Slows decline in FEV1 in CF patients with Pseudomonas
Improve FEV1 ~6%
Decreased pulmonary exacerbation
Well tolerated
45
Q

What are the mucolytic agents?

A

Pulmozyme and mucomyst

46
Q

Mucolytic agent- Pulmozyme

A 
Aerosolized recombinant dornase alfa (DNase)
Decreases viscosity of sputum
Clinical trials show modest improvement
6-12% FEV1 improvement
Decreased pulmonary exacerbation
2.5 mg nebulized once daily or BID
47
Q

Mucolytic agent- Pulmozyme- ADRs

A

AEs: hoarseness, voice alteration and pharyngitis

48
Q

Mucolytic agent- Mucomyst

A

Not generally used

Irritating, bronchoconstriction

49
Q

Nebulized hypertonic saline

A

Draws water into airways, increases ability to cough out mucus
7% nebulized daily-BID

Associated with increased cough

50
Q

Nebulized hypertonic saline- ADRs

A

Associated with increased cough

May cause bronchospasm (reaction to the hypertonic saline itself)

51
Q

When are inhaled B2 agnosists/inhaled corticosteroids useful for CF?

A

If the patient also has asthma

52
Q

What is the major pathogen in the first year of life for pulmonary complications in patients with CF?

A

Staphylococcus Aureus

53
Q

What is the major pathogen by age 3 for pulmonary complications in patients with CF?

A

Haemophilus Influenzae

54
Q

What is the major pathogen by the age of 5 for pulmonary complications in patients with CF?

A

Psuedomonas Aeruginosa

55
Q

What do chronic antibiotics do when used for chronic therapy for pulmonary complications in CF?

A

Prolong time b/w acute exacerbations

56
Q

Tobi (Nebulized, powder for inhalation tobramycin)

A

P. aeruginosa colonization

Given BID; 28 days on treatment, 28 days off

57
Q

Crayston (nebulized aztreonam)

A

P. aeruginosa colonization

Give TID; 28 days on treatment, 28 days off

58
Q

What are the immunizations recommended for chronic therapy for pulmonary complications in CF?

A

Flu vaccine

Streptococcus pneumoniae- Grive b/w 19-64

59
Q

What are the sweat gland complications associated with CF?

A

Abnormally high concentrations of sodium and chloride in sweat
Excessive sweating may lead to salt depletion

60
Q

What are the reproductive system complications associated with CF?

A

99% of males have congenital bilateral absence of the vas deferens
Females may have less than normal fertility
Delayed onset of puberty in both sexes

61
Q

What are the bone and joint complications associated with CF?

A

Low bone mineral density
Decreased osteoblasts (make bones) and increased osteoclasts (destroys bones)
Calcium and vitamin D supplementation

Arthritis
May be due to immune complexes formed in response to chronic pulmonary infections
Short courses of nonsteroidal and steroidal anti-inflammatories

62
Q

What are the signs and sx of acute pulmonary exacerbation?

A 
Increased cough frequency and duration
Increased chest congestion
Increased sputum production
Decreased exercise tolerance
Decreased PFTs
Decreased weight
Decreased oxygen saturation
Use of accessory muscles for breathing
\+/- Fever
63
Q

What are the goals of therapy for acute pulmonary exacerbation?

A

Decrease pulmonary signs and symptoms

Eradication of P. aeuroginosa is unlikely

64
Q

What is the pharmacotherapy for acute pulmonary exacerbation in CF?

A

Antibiotics, oral or iv
S. aureus, H. influenzae
P. aeruginosa cover based on sensitivities
Generally aminoglycosides in combination with an antipseudomonal penicillin (for adults)
Dose at upper end of range patients with CF have increased Vd (volume distribution) and Cl (clearance) (scary for aminoglycosides)
Increased nutrition
Increased ACT

65
Q

What is the survival rate post-lung transplant for a patient with cystic fibrosis?

A

5 years

Usually aren’t available till >10 yrs

66
Q

What is in the future for CF?

A

Gene Therapy
CFTR modulation
Correct the function of CFTR protein
VX-770

67
Q

What is the increased survival rate for patients with CF accredited too?

A

Improved survival is due to improved nutrition, pancreatic enzymes, ACT, early treatment of exacerbations

68
Q

What do the majority of CF patients die from?

A

Respiratory failure

69
Q

What does the typical patients daily medications consist of?

A
  • -Albuterol nebs BID
  • -Pulmozyme daily
  • -TOBI BID
  • -Hypertonic saline nebs BID
  • -Airway clearance- 20 mins. vest BID, Exercise
  • -Azithromycin 500 mg 3X/week
  • -ADEK vitamin daily
  • -Multivitamin daily
  • -Ursodeoxycholic acid 300 mg BID
  • -Ranitidine 150mg BID
  • -Creon 12: 2 with every meal 1 with snack

Pharmacology II (23 decks)

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