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Cystic Fibrosis

Last modified: 05 November 2022, 2:49:13 PM AEDT

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incidence 1:2,000
gene carried by 5% population
most common genetic disease in Caucasians

Prognosis1 / 8

median life expectancy 40 yo
80% reach adulthood
causes of death\n
- 1 = lung disease
- 2 = TX complications
- 3 = CFLD
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inherited disorder = autosomal recessive

Pathophysiology2 / 8

CFTR gene = chromosome 7
encodes chloride ion channel\n
- important in making sweat, digestive juices, mucus
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copious viscous secretions and its consequences\n
- mucus plugging
- repeated infections / chronic pneumonia / bronchiectasis / bullous disease
- progressive MIXED restrictive / obstructive pattern
- hypoxia, hypercarbia
- severe VQMM even in seemingly well patients
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extra-pulmonary\n
- B = ENT\n
  - Sinusitis
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- C = Cardiovascular\n
  - PHTN
  - RVF = cor pulmonale
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- E = Endocrine\n
  - endocrine = destruction of islet cells
  - CFRD = CF related diabetes, unique form DM, with similarities of type 1 and 2 DM
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- G = Gastrointestinal\n
  - GORD
  - exocrine failure 85%, destruction acinar cells\n
    - requiring PERT = Pancreatic Enzyme Replacement Therapy
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  - CFLD = Cystic Fibrosis Liver Disease\n
    - affects 30% patients
    - abnormal CFTR protein in biliary system = impaired secretion and deposition of thick viscous bile
    - hepatotoxic effects of bile and obstruction
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- N = Nutritional\n
  - chronic malnutrition, cachexia, deconditioning
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Complications3 / 8

High risk periop pulmonary complications
PTX

Symptoms / History4 / 8

RS\n
- thick sputum and its consequences
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GIT\n
- pancreatic insufficiency with malabsorption
- vitamin K deficiency
- low albumin
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sweat glands

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Management5 / 8

common operations\n
- venous access
- bronchoscopy
- nasal polypectomy
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Medications\n
- O2
- bronchodilators
- mucolytics\n
  - Dornase = purified recombinant human deoxyribonuclease\n
    - inhaled
    - hydrolyses DNA in sputum / mucus
    - produced in Chinese hamster ovary cells
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- antibiotics
- steroids
- insulin
- pancreatic enzymes
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Tune-ups\n
- mucolytics
- bronchoD
- intensive chest physiotherapy
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Anaesthetic considerations6 / 8

Pre-op

RFTs
optimisation\n
- physiotherapy
- sputum culture + AB where appropriate
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Intra-op

avoid GA if feasible
induction\n
- prolonged induction
- rapid hypoxia
- bronchial hyperactivity
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maintenance
bronchospasm\n
- bronchodilation
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secretions\n
- aggressive pulmonary toilet
- tracheal suction as required
- adequate hydration
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lung protective ventilation\n
- bullae may rupture + PTX
- vigilance for PTX
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avoid exacerbation PHTN
multimodal analgesia, limit sedatives

Post-op

postop high acuity setting
aggressive respiratory therapy / physiotherapy
humidified O2
RA where possible

Common questions / related topics7 / 8

Pregnancy\n
- very high risk patient
- ⬆risk LGA and preterm delivery
- Vaginal delivery\n
  - ensure monitored setting
  - EDB = reduce hyperventilation / stress, titrate to T10 to prevent respiratory weakness
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- CS\n
  - EDB + titration
  - GA acceptable but\n
    - prevent bronchospasm
    - frequent pulmonary toilet
    - lung protective ventilation
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Links / References8 / 8

https://www.anesthesiaconsiderations.com/cystic-fibrosis-considerations

Pancreatic insufficiency in Cystic Fibrosis 2017

Liver disease in cystic fibrosis, 2014

https://cystic-fibrosis.com/liver-disease

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