Cystic Fibrosis

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  • Cystic Fibrosis
    • Symptoms
      • babies born with jaundice
      • persistent coughing, wheezing, SOB
      • recurring chest and lung infections
      • large, smelly stools
      • hard to gain weight
      • malnutrition
      • diabetes
      • infertility
      • liver disease
      • salty sweat
      • incontinence
      • sinusitis
    • Causes
      • GENETIC
        • faulty gene (CFTR gene) inherited from both mother and father
          • the defective gene leads to a build up of thick, sticky mucus
          • the gene controls the movement of salt and water in and out of the cells
    • Diagnosis
      • SCREENING
        • Newborn Testing
          • heel prick sample of blood, to identify infection and check the functioning of organs
        • Antenatal Testing
          • offered to mums at high risk of having child with CF
          • a fine needle is passed into the womb and a small piece of the placenta is taken to examine the chromosomes
        • Sweat Testing
          • measures the amount of salt in the sweat
          • a weak, painless electric current causes the area of skin to sweat, then a sample is taken
      • GENETIC TESTING
        • analyzing either a blood sample or a saliva sample
      • CLINICAL OBSERVATION
        • of temperature, respirations and blood pressure
      • CHEST XRAY
        • shows changes in lung structure due to dysfunction and shows infection
      • SPIROMETRY
        • measure volume of air that is breathed in and out; readings are compared with normal measurements to show any obstructions in airways
    • Treatment
      • there is NO CURE, but can ease the symptoms
      • MEDICATION
        • Steroids
          • reduces the inflammation and mucus production in airways
        • Antibiotics
          • treats infections in the lungs by inhibiting the growth of microorganisms
      • HYPER-TONIC SALINE
        • strong, salt solution that's inhaled to help thin the mucus
      • BRONCHODILATORS
        • inhaled medication used to help the person to breathe more easily
      • vitamin supplements and a diet high in calories and fat
      • annual flu jabs
      • PHYSIOTHERAPY
        • focused on airway clearance; loosens the mucus from the lungs; reduces infection and lung damage
    • Lifestyle Effects
      • long periods of time off work/school
      • embarrassment of symptoms
      • low self-esteem, frustration, depression, anxiety
      • strain on social relationships

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