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Cystic Fibrosis History

 
 
 
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Overview

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  • Diagnosis - age at diagnosis, presenting symptoms, mutation
  • Family history of cystic fibrosis
  • Respiratory - symptoms, bacteria colonised, frequency of admission
  • Pancreatic - steatorrhoea, diet, insulin requirement
  • Gastrointestinal - constipation, reflux, cirrhosis
  • Musculoskeletal - joint pain, osteoporosis
  • Fertility - previous infertility, plans to conceive, genetic counselling
  • Other - symptoms of depression

Complications

  • Complications of Cystic Fibrosis

  • Respiratory

  • Bronchiectasis
  • Respiratory infections
  • Sinusitis
  • Pancreatic

  • Exocrine - fat malabsorption, malnutrition, vitamin A/D/E/K deficiency
  • Endocrine - diabetes mellitus
  • Pancreatitis
  • Gastrointestinal

  • Meconium ileus
  • Distal intestinal obstruction syndrome (DIOS)
  • Gastroesophageal reflux
  • Biliary cirrhosis
  • Malnutrition
  • Musculoskeletal

  • Arthropathy
  • Osteoporosis
  • Reproductive

  • Infertility - congenital absence of vas deferens, thick cervical mucus
  • Risk of transmission to children
  • Other

  • Significant functional impact
  • Depression

Management

  • Management Strategy

  • Pharmacologic Options

  • CFTR potentiator (G511D mutation) - ivacaftor
  • CFTR potentiator / corrector combination (delta F508 mutation) - ivacaftor / lumacaftor
  • Respiratory

  • Sputum clearance - bronchodilators, mucolytics, hypertonic saline, physiotherapy, postural drainage
  • Manage infection - monitor for colonisation, manage exacerbatons
  • Reduce inflammation - using macrolides (azithromycin)
  • Vaccinations - influenza, pneumococcus
  • Lung transplant
  • Pancreas

  • Exocrine - pancreatic enzyme replacement (creon), vitamin supplementation (A, D, E, K)
  • Endocrine - insulin if required
  • Gastrointestinal

  • Nutritional support
  • Constipation - aperients
  • Reflux - proton pump inhibitor
  • Biliary disease - ursodeoxycholic acid
  • Musculoskeletal

  • Arthropathy - NSAIDs
  • Osteoarthritis - calcium, vitamin D, bisphosphonates
  • Reproductive

  • Infertility - assisted pregnancy if required
  • Genetic counselling and testing of partner
  • Other

  • Maximise social supports
  • Treat depression
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