Cystic Fibrosis COPY Flashcards
Which organs does CF particularly affect?
Pancreas
Lungs
Intestines
Reproductive System
How many babies are born with CF?
1 in 2500
How many people in the UK have CF?
9,000
What is CF characterised by?
The abnormal transport of Cl- ions across the epithelium leading to thick, sticky, viscous secretion
What gene is mutated in CF?
F508
What protein is affected in CF?
CFTR Protein
What does the CFTR protein do in physiology?
Transports Cl- in to the lumen dragging water with it
Hydrating secretions
In CF what happens to the CFTR protein?
It doesn’t work
Water isn’t dragged into the lumen
This makes secretions thick
What does thick secretions lead to?
Pathogenesis in various organs and systems
What is Type 1 respiratory failure?
Decreased PaO2 only
What is Type 2 respiratory failure?
Decreased PaO2
Increased PaCO2
What are the early stage CF symptoms?
Incessant coughing
Large volume of phlegm production
Shortness of breath
What are the later stage CF symptoms?
Haemoptysis
Pneumothorax
Why is the thick mucous in CF ineffectively cleared?
Because their is a poor mucocilliary beating
What does poor clearing of mucous lead to?
Bacterial infection
Recurrent inflammation
Infection causing structural changes to the lung
Patients with CF get alot of bacterial infections what does the type of infection they acquire depend on?
Age
What does pseudomonas aeruginosa reduce life expectancy to?
From 39 to 28
Where is pseudomonas aeruginosa acquired from?
The environment
Other CF patients
How does pseudomonas aeruginosa affect the lungs?
Causes a rapid decline in lung function
Once colonized what does pseudomonas aeruginosa do?
Undergoes mucoid change
Forms a biofilm, which protects the bacteria from the hosts defense system
Why does pseudomonas aeruginosa form a film?
To protect itself from the hosts immune system/response
Is staphylococcus aureus a common bacteria that affects CF patients?
Yes
How is CF diagnosed?
By a sweat test
What is type 1 respiratory failure?
When there is decreased PaO2 ONLY
What is type 2 respiratory failure?
Decreased PaO2 and increased PaCO2
What are the early stage symptoms of CF?
Incessant coughing
Large volume of phlegm production
Shortness of breath (dypsnoea)
What are the later stage symptoms of CF?
Haemoptysis - this is commonly associated with infections of the respiratory tract
Pneumothorax
What is the last resort treatment for CF?
Double lung transplant
When is double lung transplant called upon for CF?
When: FEV1 < 30% predicted Significant weight loss Hypoxia even at rest Recurrent worsening sepsis Worsening quality of life If without surgery their prognosis is <2 years
What % of CF patients die whilst on the waiting list for transplant?
30-40%
On what chromosome if the F508 gene located on?
Chromosome 7
When is lung transplant considered for a CF patient?
When FEV1<30% predicted Significant weight loss There is hypoxia even at rest Recurrent worsening sepsis Worsening quality of life If their prognosis without surgery is <2 years to live
Under what circumstances does a CF patient not qualify for transplantation surgery?
Other organ failure e.g liver, kidney
Malignancy
Drug, alcohol smoking dependency
Active systemic infection M.Abscessus - terrible prognosis but cannot have surgery
What other organs are affected by CF?
Pancreas Liver Fertility Bones Endocrine
What does the pancreas produce?
Strong destructive enzymes
How does CF affect the pancreas?
The thickening of secretions can lead the pancreatic duct to becoming blocked
What do persistent respiratory infections cause?
A negative feedback cycle
What does poor clearance of mucous lead to?
Increased bacterial infection
What does the colonization of pseudomonas aeruginosa increase with?
Age
Over 80% colonized over 26 years of age
What is the treatment or recurrent respiratory tract infection in CF patients?
Treat early and aggressively with antibiotics
Which bacterial infections should be given oral antibiotics?
Staph
Haemophilus
Pneumococcus
Which bacterial infections should be given IV antibiotics?
Pseudomonas
Stenotrophomonas
Burkholderia
What 3 questions are asked at a multidisciplinary meeting prior to a patient being put on the transplant waiting list?
Is the condition bad enough?
Is the rest of the body good enough?
Does the patient actually want this?
What does the blocking of the pancreatic duct lead to?
Pancreatitis Pancreatic cysts Scarring Malabsorption Malnutrition
How does CF affect the intestines?
Poor intestinal secretion can lead to harm
Immovable faeces which causes constipation and distal intestinal obstruction syndrome
What duct can CF block in the liver?
Bile duct
What does the blockage of the bile duct lead to?
Lead to liver scarring
Cirrhosis
What is the 3rd most common death associated with CF?
Liver disease
Which cells produce insulin?
Islets of Langerhans
What does Islets of Langerhan cells produce?
Insulin
What can CF do to the pancreas?
Damage the cells that produce insulin
Leading to a unique type of diabetes known as cystic fibrosis-related diabetes (CFDR)
What % of CF males are infertile?
95%
How does CF cause infertility in males?
Blocks the ducts that connect the testes to the ejaculatory ducts
What is ICSI?
Intra-cytoplasmic sperm injection
Describe the process of ICSI?
Sperm is injected directly into the egg
3 attempts are given on the NHS
2-3 year waiting list
What is the success rate of ICSI?
25% success rate per cycle
What should be taken into consideration when CF patients are wanting children?
The partner should be screened as to if a carrier
That the mother is at optimum health
That the father will still be alive to raise the child
Be aware of the danger of prolonging life rather than extending death
Their can be a fine line
Give examples of the disciplines involved in the care of CF patients
Consultants CF nurses Dietician Physiotherapists Pharmacist Micro-biologist Ward staff Phycologist Diabetic team GI team X-ray staff GP Obstetricians Ect...
