Cystic Fibrosis Flashcards
(45 cards)
What organs does CF affect? (5)
- Lungs
- Pancreas
- Liver
- Intestine
- Reproductive organs
What are the main clinical signs of CF? (4)
- Pulmonary disease
- Recurrent infections
- Production of copious viscous sputum
- Malabsorption due to pancreatic insufficiency
*other complications include hepatobiliary disease, osteoporosis, CF-related diabetes, and distal intestinal obstruction syndrome
What are the aims of treatment in patients with CF? (4)
- Preventing and managing lung infections
- Loosening and removing thick, sticky mucus from the lungs
- Preventing and treating intestinal obstruction
- Providing sufficient nutrition and hydration
________________ is a key predictor of life expectancy in people with cystic fibrosis
Lung function
*optimising lung function is a major aim of care
The importance of ____________ techniques should be discussed with patients with CF and their parents or carers and appropriate training provided
airway clearance
Should patients with CF be advised to exercise regularly?
Patients should be advised that regular exercise improves both lung function and overall fitness.
Specialist physiotherapists should assess patients with cystic fibrosis and provide advice on _____________, ______________, ______________, ______________, and ______________
airway clearance
nebuliser use
musculoskeletal disorders
physical activity
urinary incontinence
Why is urinary incontinence associated with CF?
Urinary and/or fecal incontinence may result from progressive weakness of pelvic floor muscles caused by recurrent coughing episodes and respiratory infections
What is the role of drugs in treating patients with CF? (2)
Prevention of lung infection
maintenance of lung function
(Eg mucolytics, prophylactic abx)
In patients with cystic fibrosis, who have clinical evidence of lung disease, the frequency of routine review should be based on ________________, but adults should be reviewed at least every ______________.
their clinical condition
3 months
What is the first line mucolytic used in patients with CF?
Dornase alfa (cleaves DNA released from neutrophils and reduces mucous viscosity)
If there is an inadequate response to dornase alfa in patients with CF, ______________ and ______________, or _________________ alone should be considered
Dornase alfa
hypertonic sodium chloride
hypertonic sodium chloride
*Mannitol dry powder for inhalation is also recommended as an option when dornase alfa is unsuitable (because of ineligibility, intolerance, or inadequate response), when lung function is rapidly declining, and if other osmotic drugs are not considered appropriate
What is the mechanism of action of inhaled mannitol dry powder in the treatment of CF?
Mannitol is a naturally occurring osmotic agent which works by drawing water into the airways, helping to moisten and thin the sticky mucus found in the lungs of people with CF and making it easier to cough it out and improve lung function
Pulmonary infections are most commonly caused by which agents in patients with CF? (6)
- Staph aureus
- Pseudomonas
- Burkholderia cepacia
- Haemophilus influenzae
- Non-TB mycobacteria
- Aspergillus fumigatus
What is the treatment of choice for a CF patient with a new Staph infection who is clinically well?
Who is clinically unwell?
Oral anti-staph antibacterial
Oral or IV broad spec abx with activity against staph
A ____________ should be considered to suppress chronic Staph. aureus respiratory infections in patients with CF whose pulmonary disease is stable
long-term antibacterial
- In patients with chronic Staph. aureus respiratory infections who become clinically unwell with pulmonary disease, oral or intravenous (depending on infection severity) broad-spectrum antibacterials with activity against Staph. aureus should be given
In CF patients with new evidence of meticillin-resistant Staphylococcus aureus (MRSA) respiratory infection (with or without pulmonary exacerbation), _________________ should be sought
specialist microbiological advice
True or False: Antibacterials should NOT be routinely used to suppress chronic MRSA in patients with stable pulmonary disease
True; If a patient with cystic fibrosis and chronic MRSA respiratory infection becomes unwell with a pulmonary exacerbation or shows a decline in pulmonary function, specialist microbiological advice should be sought
If a patient with cystic fibrosis develops a new Pseudomonas aeruginosa infection, eradication therapy with a course of oral antibacterial should be started (by intravenous injection, if they are clinically unwell), in combination with _______________.
an inhaled antibacterial; An extended course of oral and inhaled antibacterial should follow
If eradication therapy for patients with CF and Pseudomonas is not successful, sustained treatment with an inhaled antibacterial should be offered. Nebulised ________________ should be considered as first-line treatment
colistimethate sodium (an antibiotic)
What is the treatment of CF patients with chronic pseudomonas infection who become clinically unwell with pulmonary exacerbations?
An oral antibacterial or combination of two IV antibacterial drugs of different classes (depending on severity) should be offered
For CF patients being treated for pseudomonas infection, if patients continue to deteriorate despite use of colistimethate sodium, which drugs may be offered? (3)
- Nebulised aztreonam
- Nebulised tobramycin
- Tobramycin dry powder inhalation
Patients who develop a new Burkholderia cepacia complex infection, should be given eradication therapy with _________________ antibacterial drugs
a combination of intravenous
Should CF patients with stable pulmonary status be treated for chronic Burkholderia infection?
No; only if they become clinically unwell with a pulmonary exacerbation
