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Respiratory System > Cystic Fibrosis in Adults > Flashcards

Cystic Fibrosis in Adults Flashcards

1
Q

How common is cystic fibrosis?

A
  • Most common autosomal recessive disorder in caucasians
  • 1 in 2000/2500 caucasian live births
  • 1 in 25 carriage rate
  • Respiratory failure is the cause of death in 90% of people with CF
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2
Q

Why has the survival for CF improved?

A
  • CF centres
  • MDT teams
  • Physio
  • Nutrition/enzymes
  • Antibiotics
  • Aggressive approaches
  • Annual flu/pneumococcal
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3
Q

What is the Cystic Fibrosis protein?

A

Cystic fibrosis transmembrane conductance regulator (CFTR) is a membrane protein and chloride channel in vertebrates that is encoded by the CFTR gene.

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4
Q

What occurs due to the mutation of the CFTR gene?

A

Abnormal transport of chloride and sodium across epithelium

  • Reduced chloride secretion from epithelium
  • Reduced Na absorption from lumen

= thick secretions
= Impaired bacterial killing via neutrophils as normal chloride required

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5
Q

What is the advantage in mice being heterozygous for the CFTR gene?

A

Increased resistance to salmonella, cholera toxin

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6
Q

What are the 5 classes of disease: pancreatic insufficiency?

A

Class 1: no synthesis G542X

Class 2: Increased degradation DF508

Class 3: Defective reg G551Db

Class 4: Abnormal conduction R117H

Class 5: Reduced synthesis/trafficking A455E

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7
Q

What are challenges for patients in CF for adults?

A
  • Transition is a major challenge, new MDT team
  • Prognosis
  • Promise of new drugs
  • Possibility of lung transplant
  • Other conditions: diabetes, liver disease, osteoporosis, fertility issues, haemoptysis etc
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8
Q

What are commonest presentations of CF in newborns?

A
  • Meconium Ileus (15%)
  • Prolonged conj jaundice
  • Newborn screening
  • Screening due to FH
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9
Q

What are commonest presentations of CF in infancy and children?

A
  • FTT (29%)
  • Abnormal stools (24%)
  • Recurrent LRTI/symptoms (40%)
  • Rectal prolapse 20%
  • Nasal polyps (10-40%)
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10
Q

What are commonest presentations of CF in adults?

A
  • Infertility (esp in men)
  • Bronchiectasis
  • Clubbing
  • Mild resp symptoms/recurrent LRTI (40%)
  • Hyperinflation
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11
Q

Why does pulmonary infection occur in CF?

A

CFTR abnormality

  • Decreased mucociliary clearance
  • Increased bacterial adherence
  • Decreased endocytosis of bacteria
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12
Q

Features of progressive airflow obstruction

A
  • Survival related to FEV1
  • Increasing exertional dyspnoea
  • Brochodilators (inhaled nebulised)
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13
Q

Features of respiratory failure

A

Type 1 = decreased PaO2

Type 2 = Decreased PaO2, Increased PaCO2

Oxygen, ambulatory oxygen
Nocturnal NIV, symptomtic relief, bridge to LTx

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14
Q

What does a cystic fibrosus CT scan usually show?

A
  • Tramlines
  • Signet rings
  • Mucous plugging
  • Consolidation
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15
Q

Features of Type 2 Diabetes Mellitus in CF

A
  • Often preceded for years by falling lung function, BMI
  • Almost never get DKA, type 1 DM only rarely seen
  • Complication risk same as non CF pts but pulmonary disease gets there first
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16
Q

CF issues in type 2 diabetes mellitus

A
  • Compliance with diet a problem
  • Low sugar/high fibre diet not appropriate
  • OGTT/HBA1C used but not perfect
  • Insulin of benefit, not so much oral hypoglycaemics
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17
Q

Features of Osteoporosis in CF

A
  • Significant BMD (bone mineral density) decrease in 1/3 adults patients with CF
  • Slower gain, faster loss, worse the sicker you are
  • May exclude lung transplant
18
Q

What does osteoporosis in CF occur due to?

A
  • Malnutrition and low BMI
  • Steroids
  • Delayed puberty and hypogonadism
  • Inflam cytokines from sepsis (we know BMD falls in septic pts)
  • Vitamin D/K deficiency
  • Lung Tx/drugs
19
Q

What are predictors of low BMD (bone mineral density)?

A
  • Low FEV1
  • Frequent a/b courses
  • Steroids
  • Low BMI
  • Low exercise
  • Age
  • Male
  • Diabetes
  • Vit D
  • Delayed puberty
20
Q

Features of Pneumothorax in CF

A
  • 3-4% Cf patients during lifetime
  • Especially if older, more severe obstructive lung disease
  • 16-20% > 18
  • Males=Females

Treatment same as other patients = drain, pleurodesis, surgery

21
Q

Features of Haemoptysis in CF

A
  • Bronchial wall destruction
  • Minor (60%) = blood streaking common, no special treatment
  • Massive in 1% patients each year, may need embolisation
22
Q

Risk factors of Haemoptysis

A
  • Severity
  • Exacerbations
  • Fungal
  • Liver disease
  • Vitamin K deficiency
23
Q

Microbiology: features of Pseudomonas Aeroginosa

A
  • Colonisation increases with age
  • Acquired from enviroment, other CF patients, segregation and disinfection policies

Colonisation associated with: reduced life expectancy, rapid decline in lung function

First isolation: attempt eradication - 3 months of treatment

24
Q

Microbiology: features of Burkholderia Cepacia

A
  • Environmental organism (causes onion rot)
  • Acquired from: environment, other CF patients (epidemic strains more virulent), segregation policies
  • Colonisation associated with: reduced life expectancy 16 vs 39 years, rapid decline in lung function, do worse in pregnancy
  • Innate resistance to most antibiotics
  • Genomovar III contraindication for transplantations
25
Q

Features of Sternotrophonas Maltophilia

A
  • Increasing frequent colonisation
  • Usually after pseudomonas, but can occur as first Gram negative infection
  • Multiple antibiotic resistance
  • Unsure if detrimental to prognosis
26
Q

Features of Aspergillus

A
  • Comes from environment, not person to person
  • Can cause spectrum disease
  • E.g. coloniser, infection, allergy
27
Q

Features of Non TB Mycobacteria in CF (NTM)

A

Isolated in up to 13% CF patients

  • MAI = 75% of these
  • M. abcessus grows rapidly, comprises 16% of NTM patients with CF

May not need treatment:
- Treat if clinical/lung function/CXR deterioration

28
Q

Features of Mycobacterium Abscessus

A
  • Currently rapid increase in number of isolates
  • Highly resistant
  • Contraindication for transplantation
  • Eradication: 1 month in hospital then maintenance treatment for 1 year- side effects, failure
29
Q

Treatment for pulmonary infection

A
  • Treat early and aggressively with antibiotics
  • Oral antibiotics (e.g. Staph, Haemophilus, Pneumococcus)
  • IV antibiotics (e.g. PA, Stenotrophomonas, Burkholderia)
  • Two antibiotics, liaise with microbiology
  • If multiply resistant, test for synergy between antibiotics
  • Large doses (include volume distribution, increased clearance)
  • Two week courses
  • Indwelling suncutaneous ports (vascuports)
30
Q

Is there evidence for antibiotics in acute exacerbations?

A
  • Not much evidence for regular ivs but anecdotally seem to work in some patients
  • Concerns re-resistance
31
Q

Antibiotics: Can resistance it vitro still work in combination in vivo?

A

Yes it may still work
- CF organisms often grow poorly on standard media
- Rise in FEV1 in iv tobra/ceftaz trial not related to whether ps was sensitive to a/b or not
- Never use 1 iv: avoids resistance.
Some evidence that 2 a/b use reduces readmiss rates

  • Synergy testing may help
32
Q

Features of Nebulised or Inhaled antibiotics

A
  • Not used instead of intravenous antibiotics
  • Colobreathe inhaler or ocassionally colomycin nebuliser
  • TOBI inhaler or occasionally Tobramycin nebuliser: it treats Pseudomonas aeruginosa
33
Q

Why would bronchodilators be used in CF?

A

CF patients may have airway obstruction due to:

  • Asthma/Atopy: BHR in 40% CF patients
  • Mechanical: bronchial plugging, inflammation > bronchial wall thickening
  • Many patients (especially milder disease): increase in FEV1 despite absence typical asthma symptoms
34
Q

Features of airway clearance: Chest Physio

A
  • Autogenic drainage, active cycle of breathing, huffing
  • Airway oscillating devices (PEP device), percussion vests
  • All patients with sputum should do physio: compliance issues
  • Expensive devices not routinely recommended as lack of data
35
Q

Features of Mucolytics

A
  • Reduce viscosity of phlegm

- Making it easier to expectorate

36
Q

Examples of Mucolytics

A
  • Pulmozyme
  • Hypertonic saline
  • Carbocysteine
  • Bronchitol
37
Q

What is the suggested regime to optimise drug delivery?

A
  • Bronchodilator MDI
  • Hypertonic saline
  • Chest physio
  • DNAse
  • (Neb antibiotics)
38
Q

Features of anti-inflammatory therapy: Azithromycin

A
  • Antibacterial and anti-inflammatory effects
  • Reduces biofilms
  • Increased lung function, decreased decline, decreased exacerbation rate by 40%
39
Q

What are new drugs - Potentiators and Correctors in CF?

A
  • Ivacaftor
  • Ivacaftor/Lumacaftor (Orkambi)
  • Tezacaftor/Ivacaftor (Syndeco)
  • Triple therapies in development

Small benefit in lung function.
More significant benefit is fall in pulmonary exacerbations and weight gain

40
Q

What are indications for a double lung transplant?

A
  • Rapidly deteriorating lung function
  • FEV1 <30% predicted
  • Life threatening exacerbations
  • Estimated survival <2 years
41
Q

Survival features for a double lung transplant

A
  • 70-80% at 5 years
  • 50% at 10 years
  • Gradual attrition due to bronchiolitis obliterans
  • Viewed as a measure to prolong survival and improve quality of life
42
Q

What are other things to consider to improve quality of life?

A
  • Oxygen and NIV
  • Exercise
  • Support
  • Advanced care planning
  • DNAR

Respiratory System (28 decks)

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