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CYSTIC FIBROSIS (MANAGEMENT (ALLERGIES (Documentation (Nature of allergy,…

- - - - Mutation
        
        Chromosome 7
        
        Deletion of phenylalanine #
        
        Position 508
        
        Codes for ABC transporter
        
        Acts as anion channel
        
        Transports Cl- & HCO3-
        
        Alteration in salt balance
      - Codes for
        
        CF transmembrane conductance regulating protein (CFTRP)
        
        Found in epithelial cells
        
        Lungs
        
        Pancreas
        
        Intestine
        
        Gall bladder
        
        Salivary & sweat glands
        
        Testes
        
        Uterus
- - - - Biliary stasis
        
        Bile acids are toxic
        
        to Hepatocytes
        
        Untreated
        
        End stage liver disease
        
        Stoppage or suppression of bile
        
        TREATMENT
        
        Ursodeoxycholic acid
        
        Vitamin K
  - - - Thickened mucus in the cervix
    - - Development of vas deferens and epididymis failure
  - - - Lead to bronchiectasis
    - - Pseudomonas aeruginosa
      - Staphylococcus aureus
      - Haemophilus influenzae
      - Stenotrophomonas maltophilia
      - Burkholderia cepacia
      - Non-tuberculous mycobacterium
      - MRSA
      - Aspergillus fumigatus
      - Pathogen varies with patient's age
- - - - REDUCED volume pancreatic secretion
        
        Secretion is more viscous
        
        Lead to obstruction of pancreatic ducts
        
        Secondary pancreatic damage
      - TREATMENT: Pancreatic enzymes
        
        Adjust dose according to requirements
        
        Creon
        
        Take with meals and snacks
    - - Exacerbated by pancreatic bicarbonate insufficiency
      - Characterised by steatorrhoea
        
        Fat in faeces
    - - Loss of endocrine function
      - TREATMENT: Insulin
  - - - Patients have
        
        Malabsorption
        
        Fat maldigestion
        
        Chronic chest infection
      - Poor appetite
      - Resting energy expenditure INCREASED
      - Lung disease INCREASES energy requirements
    - - Oral supplements
      - Naso-gastric , gastrostomy/
        jejunostomy feeding
      - Fat soluble vitamins
        
        Vitamins A, D, E
        
        Vitamin K
        
        Menadiol
        
        Water soluble
  - - - Meconium ileus
        
        Material found in intestines
    - - Partial / complete intestinal obstruction
      - TREATMENT
        
        Lactulose
        
        Oral acetylcysteine
        
        Gastrografin
    - - TREATMENT: Laxatives
    - - INCREASED Gastro-oesophageal reflux
        
        TREATMENT
        
        PPIs
        
        H2 Antagonists
        
        Prokinetics
      - Peptic ulcer
      - Rectal prolapse
      - GI malignancy
      - Intestinal mucosal transport abnormalities
      - INCREASED small intestine motility
- - - - Desensitisation
    - - Nature of allergy
      - List of allergies
    - - Administer antibiotics at home
    - - Familiar with signs of allergic reaction
    - - TREATMENT: Oral corticosteroids
        
        Prednisolone
      - Caused by fungus
        
        Aspergillus fumigatus
  - - - Cleaves extra-cellular DNA
      - FVC > 40% of predicted
      - Used with jet nebuliser
    - - Immediately before physiotherapy
    - - G551D mutation in CFTR gene
    - - Improved mucus clearance
      - Add-on therapy
  - - - Clinical psychologist support
    - - Aid mucus clearance from lungs
  - - - Anti-inflammatory
        
        Reduce airway inflammation
      - Interferes with
        
        Adherence of P. aeruginosa
        
        To epithelial cells
        
        Biofilm mode of growth