Cystic Fibrosis
Patient (condition)
 
Aliases:
CF

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Cystic fibrosis (CF) is a multisystem disorder characterised by abnormally viscous mucous secretions predisposing patients to recurrent respiratory infections and pancreatic exocrine dysfunction. The underlying pathology is a mutation in the chloride ion transporter gene and reduced mucous water content.
Anesthetic considerations revolve predominantly around assessment of pulmonary and gastrointestinal disease, avoiding pulmonary complications, and minimising the psychological effects of repeated hospital admissions. refl.in/l8

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Diabetes mellitus is a known complication of cystic fibrosis. Aetiology appears to be a combination of pancreatic beta islet cell dysfunction and insulin resistance. Enquire about presence of insulin dependent diabetes and related comorbidities. refl.in/la. PDF - refl.in/l9

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May present with bronchiectasis/chronic obstructive respiratory dysfunction. Consider lung function tests in the patient presenting with functional impairment for major surgery .

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Vitamin K deficiency (due to reduced absorption of fat soluble vitamins) may result in a coagulopathy. Check INR if indicated and replace Vit K intravenously.

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Clearance of accumulated overnight respiratory secretions may take several hours after waking in some patients, and aiming to perform general anesthesia later in the morning list may reduce respiratory complications in those patients. refl.in/l8

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If evidence of liver disease and/or portal hypertension, patients may have oesophageal varices. Caution must be taken with insertion of nasogastric tubes or TOE probes to avoid varocele rupture. #safety

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Avoid general anesthesia where possible to minimise the risk of pulmonary complications. Consider regional or local anesthesia techniques. refl.in/l8

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Coordinate with respiratory physicians to ensure perioperative chest physiotherapy is performed where appropriate.

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Pre- and postoperative care must be directed towards optimal clearance of viscous respiratory secretions. refl.in/l7

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Relevant investigations that may be considered taking the extent of disease and nature of surgery into account include full blood count, urea, creatinine & electrolytes, liver function tests, coagulation profile, chest Xray, ECG, spirometry, echocardiogram (pulmonary hypertension).

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#improve split into separate investigations with justifications
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