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