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Respirology > Cystic Fibrosis (Raf) > Flashcards

Cystic Fibrosis (Raf) Flashcards

1
Q

How does PEP work?

A

With the PEP device, there is normal inhalation, but there is resistance during exhalation, which results in the creation of back pressure (positive expiratory pressure)

  • Create a PEP of 10-20 cm H20 for 12-15 breaths
  • This results in build up of gas pressure behind mucous through collateral ventilation (pores of Kohn, cannals of Lambert)
  • This positive pressure also stents the airway open, preventing premature closure, enabling movement of mucous
  • Through forced expiratory maneuvers (eg. huffing), the mucous can be moved from peripheral to central airways. Coughing maneuvers enables expectoration of mucous
  • Recent Cochrane Review which compared different methods of airway clearance: neither was superior in terms of FEV1, but PEP was superior for decreased number of exacerbations. CF foundation guideline (which is old, from 2009) does not endorse a specific type of airway clearance.

Side effect: hemoptysis, nausea

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2
Q

Diagnostic criteria for a classic case of ABPA in CF?

A

There are 5 criteria:

  • Acute or subacute clinical deterioration that is not attributable to another etiology
  • Total serum IgE > 1000, unless they are receiving systemic steroids. (Check IgE when patient is off steroids)
  • Positive IgE antibody to A. fumigatus or immediate cutaneous hypersensitivity to aspergillus
  • Precipitating antibody to A. fumigatus or serum IgG to A. fumigatus (this is practically difficult to accomplish)
  • New or recent infiltrate (or mucous plugging) on CXR or CT, which does NOT respond to antibiotics and standard physiotherapy
  • Need to meet all 5 criteria for classic ABPA
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3
Q

Minimal diagnostic criteria for ABPA?

A
  • Acute of subacute clinical deterioration that is not attributable to another etiology
  • Total serum IgE > 500, unless they are receiving systemic steroids. (Check IgE when patient is off steroids)
  • Positive IgE antibody to A. fumigatus or immediate cutaneous hypersensitivity to aspergillus
  • One of:
  • Precipitating antibody to A. fumigatus or serum IgG to A. fumigatus (this is practically difficult to accomplish)
  • New or recent infiltrate (or mucous plugging) on CXR or CT, which does NOT respond to antibiotics and standard physiotherapy
  • So 4 criteria for minimal diagnostic criteria
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4
Q

Difference between classic and minimal diagnostic criteria for ABPA in CF?

A
  • Total IgE threshold of 1000 versus 500
  • For the last 2 criteria (precipitating antibody/IgG A. fumigatus or imaging findings)–>need both for classic ABPA, but only 1 for minimal ABPA
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5
Q

What are the imaging findings of ABPA?

A
  • opacities, which can be fleeting
  • gloved finger shadow from mucous impaction
  • Tram line shadow
  • high attenuation mucous on CT –pathognomonic
  • central upper lobe saccular bronchiectasis
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6
Q

Pre-flight evaluation for patient with CF?

A
  • spirometry
  • hypoxic challenge test if FEV1<50%
  • if saturation <90% on hypoxic challenge, then arrange for supplemental oxygen
  • if they had a pneumothorax, wait for 2 weeks post radiologic resolution, though they are increased risk for pneumo (if no definitive surgical management)
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7
Q

Maximum dose of PERT?

A

10,000 U/kg/day or 2500 U/meal

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8
Q

Diarrhea in spite of adequate PERT?

A
  • chewing enzymes
  • expired enzymes
  • enzymes at end of meal
  • hyperacidity (consider PPI)
  • small bowel bacterial overgrowth, C. dif
  • fibrosing colonopathy
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9
Q

Manifestations of CFRLD?

A
  • Neonatal cholestasis
  • Cirrhosis - which could be focal biliary or multilobular. Interestingly focal biliary cirrhosis often starts in the pediatric years, can be asymptomatic without liver enzyme elevation or hepatomegaly.
  • Steatosis
  • Noncirrhotic portal hypertension
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10
Q

How often are CF patients screened for NTM?

A
  • Annually with a culture based method of sputum

- Oropharyngeal swab should not be sent (less reliable)

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11
Q

If a CF patient on chronic azithro tests positive for NTM, what do you do?

A
  • Stop azithro, while you are working them up for NTM disease
    (you may be partially treating with azithro monotherapy and causing resistance)
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12
Q

How is M. abscessus treated?

A

Intensive phase:
- Oral macrolide (ideally azithro) + 3-12 weeks of IV amikacin and one or more of: IV tigecycline, imipenem or cefoxitin
(ABS needs CIT-ups)

Continuation phase:

  • Oral macrolide (ideally azithro)
  • Inhaled amikacin
  • 2-3 of these oral antibiotics: minocycline, clofazamine, moxifloxacin, linezolid (guided, but not directed by susceptibility testing)
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13
Q

Can monotherapy for NTM?

A

No, similar to TB, it’s pretty intensive to treat NTM

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14
Q

How is M. avium complex pulmonary disease treated?

A

If clarithromycin sensitive, then treat with oral antibiotics:
REA - rifampin, ethambutol, azithromycin

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15
Q

When should you consider IV amikacin for M. avium?

A

If one or more of the following signs of a more severe infection:

  • AFB smear positive respiratory tract samples
  • Radiological evidence of lung cavitation or severe infection
  • Systemic signs of illness
  • Macrolide resistance (mentioned in kendig’s, but not CF guideline)
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16
Q

How long is NTM treated for?

A

For 12 months after culture conversion (3 consecutive negative cultures, with date of conversion being the first culture), as long as there are no positive cultures

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17
Q

How to monitor patients on NTM treatment?

A
  • Expectorated or induced sputum samples every 4-8 weeks
  • Monitor for drug toxicity: hearing loss, vision loss, renal impairment, liver function abnormalities
  • HRCT scan before starting treatment and at end of treatment
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18
Q

What sort of monitoring is required for IV amkacin or streptomycin?

A

These are both aminoglycoside antibiotics so need to monitor serum levels
- risk of ototoxicity and nephrotoxicity

19
Q

Is persistent M. abscessus orM. avium in a patient with CF an absolute contraindication to transplant?

A

No, but ideally want to complete treatment successfully before listing for transplant

20
Q

Does finding NTM in CF patient’s sputum mean they have NTM disease?

A

Probably not.
The more pathogenic bacteria are Pseudomonas aeruginoa and Staph aureus.
In most Cf patients with this finding, the positive culture could be transient or not associated with worsening dissease

21
Q

Which is worse for CF patient with NTM disease, abscessus or MAC?

A

Abscessus is worse since more intense clinical worse, intense therapy and hard to eradicate, may have implications for lung transplant

22
Q

Diagnostic criteria for NTM disease?

A

Need to have all of the following clinical criteria:
- pulmonary symptoms
- Radiology: CXR showing cavities or nodules OR CT showing multifocal bronchiectasis with multiple small nodules. Basically, at minimum, you need to have nodules.
- Exclusion of other diagnoses
Microbiologic criteria: need to have one of the following:
- positive culture from 2 separate sputum samples
- positive culture from 1 BAL or wash
- (last criteria is complicated and refers to biopsy related features along with culture)

23
Q

side effects of:

  • amikacin
  • tigecycline
  • cefoxitin
  • azithromycin
  • ethambutol
  • linezolid
  • rifampin
A

Amikacin: nephrotoxicity, ototoxicity
Tigeclycine: nausea, vomiting, diarrhea, pancreatitis, hepatitis, hypoproteinemia
Cefoxitin: fever, rash, eosinophilia, cytopenia
Azithromycin: nausea, vomiting, diarrhea, prolonged QT, auditory/vestibular toxicity
Ethambutol: optic neuritis, peripheral neuropathy
- Linezolid: optic neuritis, cytopenia, peripheral neuropathy
- rifampin: cytopenia, hepatitis, orange coloration of secretions, renal failure

24
Q

What is the most common co-morbidity in CF?

A

CF related diabetes

25
Q

Bad outcomes of CFRD?

A

Associated with lower survival, poor nutrition and faster lung function decline

26
Q

What is the gold standard screening test for CFRD?

A

2 hour (75 gram) Oral glucose tolerance test

27
Q

At what age does screening for CFRD start?

A

> =10 years of age

28
Q

Is HbA1c recommended as a screening test for CFRD?

A

No

29
Q

When is self monitoring of blood glucose measurement recommended?

A
  • During a pulmonary exacerbation where patients are either admitted for exacerbation, for IV antibiotics or systemic steroids–>for first 48 hours, pre and 2 hour post prandial BG measurement
  • For patients who are chronically on continuous enteral feeds, then do a mid and post glucose measurements. Measurements should be done when feeds are initiated and then monthly
30
Q

Diagnostic criteria for CFRD?

A
  • Same definitions as for diabetes in general.
    In a period of stable health:
  • Fasting glucose >= 7
  • 2 hour oral glucose tolerance>=11.1
  • HbA1C >=6.5 (but note HbA1c is often low in CF and can have normal HbA1c and still have CFRD)

in patient with acute illness:
- persistence of fasting or post-prandial hyperglycemia after first 48 hours of illness

31
Q

Patient with CF who has positive oral glucose tolerance test?

A

CF guideline advises repeating a positive test on a separate day to confirm the diagnosis, unless overt signs of hyperglycemia (polyuria, polydipsia) or random glucose >=11.1

(I’m not sure if this happens in practice)

32
Q

What is preferred management agent for CFRD: insulin or oral hyperglycemic agents?

A
  • Insulin is recommended

- Oral agents are not advised

33
Q

CF patient with diabetes on insulin. How often should they check BG?

A

checking at least 3 times daily

34
Q

In patient with CFRD, how often is HbA1c monitored and what is target?

A

monitored 4x per year, goal is <7%

35
Q

How do you monitor for complications of CF related diabetes?

A
  • BP measurement at every visit
  • start monitoring for microvascular complications (eg. retinopathy, nephropathy, neuropathy, diabetic foot) at 5 years post diagnosis
36
Q

What is an impaired glucose tolerance test?

A

Normal fasting glucose (<7), but 2 hour OGTT: 7.8 to <11.1

37
Q

Side effects of aminoglycosides? How to minimize side effects?

A
  • Ototoxcicity
  • Nephrotoxicity
  • Extended interval dosing (once daily) as opposed to tid dosing–>lower basal level–>this is recommended by CF foundation and cochrane review (2016) showed no difference in FEV1, FVC with less nephrotoxicity in children
38
Q

CF and neutrophils. What are the key cytokines?

A

IL1, IL8, TNF alpha, LeukotrieneB4

39
Q

CF Foundation guideline recommendation re: use of beta agonist for maintenance of lung health?

A

Insufficient evidence to recommend for or against

40
Q

Causes of false positive sweat chloride?

A

Endocrine: hypothyroidism, hypoparathyroidism, adrenal insufficiency, pseudoaldosteronism, nephrogenic DI
Malnutrition
HIV
Skin: eczema, ectodermal dysplasia
Metabolic: G6PD, glycogen storage disease type 1

41
Q

Causes of false negative sweat chloride?

A 
Edema
Malnutrition
Hyponatremia 
Insufficient sweat quantity 
Dilution
42
Q

Timing of sweat chloride in newborn?

A

> 2 kg (Kendig’s and CF guideline)
36 weeks (Cf guideline)
2 weeks (Kendig’s, CF guidelines technically says >10 days)
(do sweat chloride as soon as possible after these criteria are met since there’s risk of hyponatremia dehydration so want to start therapy promptly; that being said can start therapy presumptively without confirmation of diagnosis)

43
Q

Treatment of ABPA in CF?

A

1st = steroids -> Prednisone 0.5-2mg/kg.day 1-2 weeks then taper within 2-3 mosthis is directly from CF guideline
2nd = antifungal: oral Itraconazole 5mg/kg/day x 3-6 mos (therapeutic drug monitoring and LFTs)
add -> BD, inhaled steroids etc only if indicated for asthma

Respirology (28 decks)

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