Cystic Fibrosis Flashcards
What are the 3 key points in the day-to-day management of CF?
- Infection control
- Physiotherapy
- Nutritional management
What are the main aims of these combined treatment strategies?
To ensure infants and children with CF do not have respiratory symptoms or symptoms of malnutrition
What do many CF specialists recommend in order to reduce the risk of respiratory tract infection patients with CF?
Prophylactic antibiotic treatment
What is usually recommended for prophylactic antibiotic treatment in patients with CF?
Flucloxacillin
What is recommended if there is an increase in respiratory symptoms or decline in lung function?
Rescue antibiotics
What is an indicator in CF for IV antibiotics?
Persisting signs or symptoms despite oral antibiotics
Why is vigorous IV antibiotic therapy often required?
In order to prevent lung damage
For how long are IV antibiotics given in more severe CF related infections?
14 days
How are IV antibiotics usually administered in CF?
Via a PIC line
What type of infection is more commonly associated with rapid decline in lung function?
Pseudomonas infection
What is used to treat pseudomonas infection?
Specific anti- pseudomonal antibiotics
What do more severe cases of CF often require?
Regular IV antibiotic therapy
How can regular IV antibiotic therapy best be administered?
Via a central venous catheter with a SC access port
What is the problem with using a central venous catheter with a SC access port?
They require monthly flushing and complications may develop
When and how often should children with CF begin engaging with physiotherapy techniques?
From diagnosis at least twice a day
What is the aim of physiotherapy techniques in CF?
To clear the airways of secretions
What physiotherapy techniques are advised for young children?
Chest percussion and postural drainage performed by parents
What physiotherapy techniques are advised for older children?
Controlled deep breathing exercises
What else may be useful in the physiotherapy management of CF?
Physiotherapy devices
What else is encouraged in CF?
Exercise
What should be assessed regularly in CF patients?
Dietary status
How is pancreatic insufficiency treated?
Oral enteric-coated pancreatic replacement therapy
When should pancreatic replacement therapy be taken?
With every meal and snacks
How is the dose of pancreatic replacement therapy determined?
Based on clinical response
What is essential in a CF diet?
High calories
How high must the calorie count be in a CF diet?
Around 150% of normal
How may a high calorie diet be achieved?
Over night feeding via gastrostomy
What else may CF patients require to supplement their diet?
Fat soluble vitamin tablets
What is the only therapeutic treatment that can be considered in end stage CF lung disease?
Bilateral sequential lung transplant
What is the current 10 year survival rate post Bilateral sequential lung transplant?
50%
What must be considered before deciding to perform a lung transplant?
- Co-morbidities
- Microbiology
- Psychological preparation
- Optimal timing
- Post-transplant care
How has the average life-expectancy of a CF patient improved?
From a few years to mid 30’s
What is the current predicted life expectancy of a baby born with CF?
Into the 40’s
What do 95% of CF patients ultimately die of?
Respiratory failure
What are the other common complications of CF?
- Meconium ileus
- Distal intestinal obstruction syndrome (DIOS)
- Infection
- Liver disease
- Respiratory complications
- Infertility
- Psychological impact
How many infants with CF are affected by meconium ileus?
10-20%
What does meconium ileus cause?
Intestinal obstruction
What are the typical symptoms of meconium ileus?
- Vomiting
- Abdominal distension
- Failure to pass meconium
What is usually needed to treat meconium ileus?
Surgical treatment
What can sometimes be useful in relieving the obsstuction caused by meonium ileus?
Gastrografin enema
What is a DIOS?
It is the later equivalent of a meconium ileus by where the mucofaeculent material obstructs the bowel
How is DIOS treated?
Combination of oral laxatives
What can chronic infection in CF lead to?
- Damage to bronchial wall
- Bronchiectasis
- Abscess formation
What organisms often cause infection in CF?
- Staph aureus
- Haemophilus influenzae
- Pseudomonas aeruginosa
- Burkholderia
Which organisms tend to cause rapid decline of lung function in CF?
Pseudomonas and Burkholderia
What are patients often advised to do as a result of high risk infection with specific organisms?
Avoid socialising with other CF patients
How many adolescent CF patients will have evidence of liver disease?
1/3
What evidence can be seen suggesting liver disease?
- Hepatomegaly
- Abnormal LFTs
- Abnormal USS
What may be beneficial to improve bile flow in patients with liver disease in CF?
Ursodeoxycholic acid
What does CF associated liver disease rarely progress to?
- Cirrhosis
- Portal hypertension
- Liver failure
What treatment is generally very successful in CF patients with severe liver disease?
Transplant
What respiratory complications becoming increasingly more likely as CF progresses?
- Chest infections
- Pneumothorax
- Life-threatening haemoptysis
Which gender are affected by infertility in CF?
Male
Why do males almost always experience infertility in CF?
They do not have a vas deferens
How may males with CF father children?
Through intracytoplasmic sperm injection
Are females affected by infertility in CF?
No, they tolerate pregnancy well unless they have severe lung disease
Why do patients with CF require psychological support?
They have to cope with a chronic and ultimately life-limiting disease
Which age group require particularly special psychological consideration?
Adolescents
