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What is Cystic Fibrosis

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What is Cystic Fibrosis? Characterised by congenital abnormality of the mucous ... Effects of Cystic Fibrosis. on Other Parts of the Body ... – PowerPoint PPT presentation

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Title: What is Cystic Fibrosis


1
What is Cystic Fibrosis?
  • ? Characterised by congenital abnormality of
    the mucous secreting glands throughout
  • the body
  • ? Lack of pancreatic enzymes
  • ? A risk of 1250 births
  • ? An inherited disorder

2
Glands Effected are-
  • Salivary
  • Sweat
  • Pancreas
  • Tracheal Bronchial Glands
  • Intestinal Tract
  • Bile Ducts
  • Testes
  • Mucus very viscid known as mucoviscidosis

3
Signs and Symptoms
  • Failure to thrive
  • Bulky fatty stools
  • Repeated respiratory tract infections
  • Meconium illeus (intestinal obstruction)
  • Salty to taste
  • Do not respond to antibiotics readily
  • Severe bouts of coughing, causing vomiting
  • Bronchiolitis leading to emphysema

4
Diagnosis
  • BM meconium test showing ? levels of alburnin
    in stool
  • Measurement of chymo-trypsin levels in stool
  • Chest x-rays (repeated chest infections)
  • Abdominal x-ray for diagnosis of meconium illeus
  • Positive sweat test result from 6 wks of age - ?
    sodium chloride

5
TreatmentSurgery
  • - 10 of babies with CF may present with
    intestinal obstruction soon after birth
  • Meconium illeus
  • Marked abdominal distension
  • Bile stained vomiting
  • Diagnosis suggested if family hisotry of CF

6
TreatmentSurgery
  • Gastrografin enema used as diagnostic and
    therapeutic procedure.
  • This being an irritant, hypertonic and draws
    fluid into the lumen of the gut, thus making
    meconium more easily passed
  • If evidence of meconium peritonitis then proceed
    to surgery for illeostomy

7
Chest
  • Regular physiotherapy, varying positions
  • Taught by physiotherapists to parents
  • Mucolytics, bronchiodilators, inhalations,
    postural drainage
  • Possible long term antibiotic therapy
  • (Humidity as neonates)

8
Dietary
  • Pancreatic enzyme supplements
  • E.g. cream
  • As a baby, mix enzyme with a small amount of
    feed, prior to main feed
  • Can breast feed but may need to change to Pepti
    junior with higher salt and protein content.
  • Too much enzyme ?diarrhoea, foul stools, poor
    growth, abdominal pain
  • Too little enzyme ? constipation, abdominal
    pain, sore bottom

9
Vitamin Supplements
  • Not absorbed very well, often needs supplements
    for the rest of their life
  • Vitamins A, D, E, K (fat soluble)
  • Iron and zinc supplements
  • Extra salt, to children crisps, salty snacks

10
Advice
  • Fluoride supplements advice from dentists.
  • From dietician and Cystic Fibrosis Research Trust
  • Contact a family

11
Prognosis
  • Prognosis is improved if diagnosed early and
    treatment commenced
  • Long term psychological and social support

12
Effects of Cystic Fibrosis on Other Parts of the
Body
  • Ears, nose, sinuses, prone to sinusitis and nasal
    polyps which may need nasal sprays, antibiotics
  • Liver becomes involved in 10 of older CF
    patients
  • Gall stones or a type of cirrhosis. LFTs
  • Diabetes mellitis, 10 of CF adults

13
Effects of Cystic Fibrosis on Other Parts of the
Body
  • Joints, may develop a form of arthritis, can be
    improved with treatment
  • Delayed social development and growth girls
    late periods, boys late to develop

14
  • Fertility
  • - Normal in women
  • - Men are sterile
  • Heart
  • In adults whose lungs and heart are severely
    damaged by infection heart/lung transplant
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