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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