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MS-PA: Respiratory Diseases > Cystic Fibrosis & Bronchiectasis > Flashcards

Cystic Fibrosis & Bronchiectasis Flashcards

1
Q

What is Bronchiectasis?

A

A chronic obstructive lung disease Characterised by the abnormal, localized irreversible dilatation of the bronchi/bronchial tree. It is divided into CF and Non-CF bronchiectasis.

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2
Q

What are the causes of Bronchiectasis?

A
  • old age
    *Recurrent aspiration pneumonia
  • Tuberculosis, Measles and Pertussis
  • Immunoglobulin deficiency
  • Idiopathic
  • RA and ABPA
  • Alpha-1 antitrypsin deficiency
  • Genetic causes:Primary ciliary dyskinesia, Kartageners syndrome, Youngs syndrome.
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3
Q

What are the signs and symptoms of Bronchiectasis?

A

*Chronic productive cough
*Coarse crepitations in affected regions
* ± Wheeze and Clubbing
* Recurrent lower respiratory tract
infections
*Intermittent Haemoptysis

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4
Q

What are the imaging studies in Bronchiectasis ?

A
  • CXR to rule out Kartageners syndrome or cavitary lesions of TB
  • HRCT may show abnormally widened and thickened airway with an irregular wall.
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5
Q

What are the blood works in Bronchiectasis ?

A

*IgE and RAST to rule out ABPA
* Immunoglobulins: to look for < IgG, IgA and IgM.
* Mannose binding lectin level to rule out immune deficiency.
* Pertussis and Measils serology
* Sputum culture to detect MRSA and other Drug resistant organisms.
*Mantoux test for TB

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6
Q

What are the principles of bronchiectasis management ?

A

*Treat symptoms
* Treat infections early and aggressively & prevent colonisation of difficult pathogens
* Optimise lung function
* Optimise weight & nutrition
* Smoking cessation
* Vaccination against COVID-19, influenza and pneumococcus

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7
Q

What is the management of acute bronchiectasis exacerbation?

A
  • Respiratory support: O2, NIV, ICU referral may be needed.
  • Antimicrobial therapy after sensitivity testing for 10 to 14 days with gram negative and positive cover.
  • Airway clearance: PT, SABA, and Mucolytics: DNAase (pulmozyme), hypertonic saline (7%).
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8
Q

What is the abx for Pseudomonas aeruginosa and Burkholderia cepacia?

A

ceftazidime or Tazocin

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9
Q

What is the abx for MRSA cover ?

A

Linazolid and vancomycin.
In severe resistant cases: aztreonam or meropenem

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10
Q

What is the long term management of bronchiectasis ?

A
  • Portable oxygen if desaturating on exertion
  • +/- Non-invasive ventilation at night
  • Physiotherapy
  • Short-acting bronchodilators
  • Mucolytics: DNAase (pulmozyme), hypertonic saline (7%)
  • Airway clearance devices
  • Inhaled antibiotics for Pseudomonas colonisation e.g. Tobramycin, Colomycin, Aztreonam, Levofloxacin
  • Oral macrolides used as anti-inflammatory to minimise exacerbations e.g. Azithromycin
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11
Q

what are the Complications of Bronchiectasis?

A
  • Recurrent infections with resistant organisms
  • Hypoxia
  • Respiratory failure
  • Massive haemoptysis
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12
Q

What is cystic fibrosis ?

A

The most common life-shortening autosomal recessive disease in Western Caucasian populations due to to a mutation in the CF Transmembrane Conductance Regulator (CFTR) gene, causing dysfunction of a complex chloride channel found in all exocrine tissues.This chloride channel defect results in reduced volume and hyperviscosity of mucosal
secretions leading to end –organ damage causing muti-system disfunction.

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13
Q

What is the CF gene mutation frequency and epidemiology ?

A
  • 1:35 Continental Europe
  • 1:25 U.K. Anglo Saxon descent
  • 1:19 Irish/ Celtic descent are “carriers” of a mutated CFTR gene.
    Ireland has the highest incidence of the disease, with 1 in 19 people being carriers of a mutated CFTR gene.
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14
Q

What is the most common CF mutation in Ireland ?

A

Delta F508 in the homozygous state [ 2 copies of this mutation ] which accounts for 56% of Irish people living with CF.
* Since 2011 neonatal heel-prick screens for CF [newborn screening programme]

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15
Q

What is the pathophysiology of cystic fibrosis ?

A

CFTR gene mutation on chromosome 7 causes chloride channel dysfunction in exocrine tissues. This causes abnormal sodium and chloride transport leading to thick mucus in bronchi, the biliary tree, pancreas, intestines and the reproductive tract. This result in Defective mucociliary clearance, Bacterial colonization, Neutrophilic inflammation, and end organ damage for instance Panbronchiectasis.

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16
Q

What are the most common CFTR mutations in Irish population ?

A

> 1000 CFTR gene mutations
Irish Population:
delta F508 (92%)
G551D (15.5%)
R117H (5.9%)
All other mutations < 4% of CF population

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17
Q

What is the clinical presentation of CF at birth or infancy ?

A
  • Failure to thrive
  • Meconium ileus/ intestinal obstruction
  • Testing – sibling with CF
  • Newborn screening (2012 – onwards) – Diagnosis will predate
    symptoms
18
Q

What is the clinical presentation of CF in Childhood?

A
  • Failure to reach growth milestones
  • Recurrent chest infections
  • Abdominal cramps/ diarrhoea
19
Q

What is the clinical presentation of CF in Adulthood?

A

Less common
* Long-standing history of bronchiectasis and never tested
* Atypical disease
* Milder disease/mutations

20
Q

What are the respiratory manifestations of CF ?

A
  • Decreased mucociliary clearance
  • Recurrent pulmonary exacerbations
  • Early = Upper and mid zone bronchiectasis
  • Later = Diffuse Pan-bronchiectasis
  • Mucus plugging
  • Obstructive airways disease
  • Respiratory failure
21
Q

What are the CF CF Respiratory exacerbation symptoms?

A
  • > Productive cough
  • > Sputum - > volume, >purulence, > viscidity
  • > SOB
  • > Wheeze
  • > Haemoptysis
  • Nasal/ sinus symptoms
  • Duration of onset – few days- couple of weeks
    (Absence of febrile symptoms – common)
22
Q

What is the clinical presentation of pancreatic CF ?

A

Pancreatic insufficiency is present in majority of CF patients.
* Fatty infiltration of pancreas from birth can be seen on ultrasound.
* Diarrhoea, fatty stools, Abdominal cramps
* Malabsorption causing Vit deficiencies, low BMI, failure to gain weight.
Recurrent Pancreatitis is only seen in PWCF with mild CF mutations who are pancreatic sufficient.

23
Q

What are the GI manifestations of CF ?

A

Large bowel
* Constipation – very common – slower transit
* Increased risk of colonic carcinoma (in older patients  30 years)
Small bowel
* Distal intestinal obstruction syndrome (DIOS) – recurrent abdo pain, no
definitive clinical findings, abdominal CT diagnosis
Upper GI
* Vomiting, GORD – common with coughing
* Advanced disease – delayed gastric emptying – early satiety and vomiting

24
Q

What is the CF Liver disease presentation ?

A

focal biliary cirrhosis, fatty infiltration, can lead to cirrhosis with splenomegaly, varices.

25
Q

How does CF cause Male infertility?

A

Obstructive azospermia – functional sterility in males (due to CBAVD)

26
Q

What is the CF renal presentation?

A

distinct entity, compounded by aminoglycoside usage, and diabetes

27
Q

What is the diagnostic criteria in CF?

A

Clinical features or CF Hx in siblings or + new born screening + Positive sweat test or positive nasal PD or Two identifiable CFTR mutations.

28
Q

What are the treatment goals in CF ?

A
  • Slow disease progression
  • Treat infections early and aggressively & prevent colonisation with
    difficult pathogens
  • Optimise lung function
  • Optimise weight & nutrition
  • Ensure transplant eligibility
29
Q

What is the acute Tx of CF exacerbations?

A
  • IV antibiotics – 2 weeks/ 2 agents
  • Intensive airway clearance/chest physio
30
Q

What are the antibiotic choice in CF Respiratory exacerbations?

A
  • Anti-pseudomonal cover– Piptazobactam, colomycin, tobramycin,
    meropenem
  • Staph Aureus – Flucloxacillin
  • MRSA – Vancomycin
  • Stenotrophomonas – Cotrimoxazole (‘Septrin’)
  • Aspergillus colonization – Itraconozole (oral) x 6 weeks
31
Q

What should be the CF Maintenance therapy?

A

Nebulized/Inhaled therapies
* Nebulized anti-pseudomonal antibiotics – Tobramycin, Colistin, Aztreonam
* Nebulized DNAase – Dornase Alpha (‘Pulmozyme’)
* Nebulized 7% Hypertonic Saline
* Inhaled/ Nebulized bronchodilators – Salbutamol, Ipratropium
* Concurrent asthma – ‘Seretide’ (Salmeterol/Fluticasone) or ‘Symbicort’
(Formoterol/Budesonide)
Anti-inflammatories
* Azithromycin (orally, alternate days)

32
Q

What is the Nutritional Support in CF Maintenance therapy?

A
  • Oral nutritional supplementation
  • Calcium + Vit D supplementation for bone protection
  • Proton-pump inhibitor [PPI]
  • PEG insertion with nocturnal feeding should be considered
  • Manage constipation
33
Q

What is the CF Maintenance therapy in Pancreatic Insufficiency?

A
  • Creon- pancreatic enzyme replacement
  • Fat soluble vitamin replacement (ADEK)
  • Insulin if DM present
34
Q

What is the CF Maintenance therapy in Liver/Biliary disease?

A

Bile acid binding salts: Ursodeoxycholic acid [Ursofalk]

35
Q

What are CFTR Modulators ?

A

CFTR Modulators are a class of drugs that act by improving production, intracellular processing, and/or
function of the defective CFTR protein.

36
Q

What are two types of CFTR drugs?

A
  1. Potentiators - hold the gate to the CFTR channel open so chloride can flow through the cell membrane.
  2. Correctors - help the CFTR protein to form the right 3-D shape so that it is able to move to the cell surface.
37
Q

What is Kaftrio and its indication ?

A

Triple combination therapy which contains ivacaftor, tezacaftor
and elexacaftor. It is indicated for patients over 6 have two copies of the F508del mutation or one copy of
F508del and any other mutation.

38
Q

What are the benefits of Keftrio ?

A

It is a ”Miracle drug” – approved for use since October 2020. It Improves lung function [FEV1 increased by 10 -14 % from baseline] and Reduced exacerbations and Increased BMI.

39
Q

What are the elements of CF ABLE score ?

A

A - Age
B - BMI
L – Lung Function (FEV 1)
E – Exacerbations (no. in past 3 months)

40
Q

What is the interpretation of CF ABLE score ?

A
  • Helps determine prognosis and outcomes in CF in the next 4 years and is calculated every clinic visit.
  • Scores between 0-7.
  • Low scores indicate low risk of poor outcomes.
  • A score >5 gives a 26% risk of poor outcome.
41
Q

What are the indications for referral for lung transplant in CF ?

A

Rapid clinical decline of FEV1 and high ABLE score

42
Q

What are the contraindications for lung transplant in CF ?

A
  • Colonization with Burkholderia Cepacia or Complex colonization
  • BMI < 17
  • Liver failure
  • Psychological concerns or non compliance

MS-PA: Respiratory Diseases (15 decks)

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