Cystic Fibrosis Flashcards
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
True or False: Haemophilus influenzae infection in the absence of clinical evidence of pulmonary infection SHOULD be treated with an appropriate oral antibacterial drug in patients with CF
True; unlike other infections which only require treatment when unstable, H.influenza should be treated even if asymptomatic
- In those who are unwell with clinical evidence of pulmonary infection, an appropriate antibacterial should be given by mouth or intravenously depending on the severity of the illness
Treatment with an antifungal drug should only be considered to suppress chronic Aspergillus fumigatus complex respiratory infection in patients with _______________
declining pulmonary status
Treatment with an ______________ drug should only be considered to suppress chronic Aspergillus fumigatus complex respiratory infection in patients with declining pulmonary status
antifungal
An oral or intravenous (depending on the exacerbation severity) ______________ antibacterial should be used for CF patients who have a pulmonary disease exacerbation and no clear cause
broad-spectrum
- If a causative pathogen is identified, an appropriate treatment should be selected
Long-term treatment with azithromycin [unlicensed indication], at an ________________ dose, should be offered to patients with CF and deteriorating lung function or repeated pulmonary exacerbations
immunomodulatory
Long-term treatment with ______________ [unlicensed indication], at an immunomodulatory dose, should be offered to patients with CF and deteriorating lung function or repeated pulmonary exacerbations
azithromycin
In those CF patients with continued deterioration in lung function or continuing pulmonary exacerbations, long-term azithromycin should be discontinued and the use of an _______________ considered instead
oral corticosteroid
_____________ should be offered to CF patients with exocrine pancreatic insufficiency
Pancreatin (given to compensate for reduced or absent exocrine secretion; they assist in digestion of starch, fat, and protein)
*Dose should be adjusted as needed to minimise any symptoms or signs of malabsorption
An _______________ [unlicensed indications] can be considered for patients with CF who have persistent symptoms or signs of malabsorption
acid-suppressing drug, such as an H2 receptor antagonist or a proton pump inhibitor
What is distal intestinal obstruction syndrome?
A condition where the small intestine gets blocked by thickened stool; occurs due to poor nutrient absorption and reduced flow through the intestines
What this the management of distal intestinal obstruction syndrome? (3)
- Oral or IV fluids to ensure adequate hydration
PLUS
- First line: Meglumine amidotrizoate with sodium amidotrizoate solution (orally or via an enteral tube)
Second line: iso-osmotic polyethylene glycol and electrolyte solution
OR
- Surgery is last resort
If liver function blood tests are abnormal in patients with cystic fibrosis, ________________ [unlicensed indication] can be given until liver function is restored
ursodeoxycholic acid
Patients should be monitored for cystic fibrosis-related _________________ and _______________
low bone mineral density
Diabetes
What are the side effects of dornase alfa? (8)
(Frequency unknown)
- Chest pain
- Conjunctivitis
- Dyspepsia
- Dysphonia
- Dyspnea
- Fever
- Increased risk of infection
- Skin reactions
Is dornase alfa safe to use in pregnancy and breastfeeding?
Probably safe, use with caution only if benefits outweigh risks
Expert sources advise that dornase alfa should usually be used __________ daily at least 1 hour before __________
once
physiotherapy
How is dornase alfa administered?
Undiluted by inhalation using a jet nebulizer
Colistimethate sodium is a _______________ antibiotic
Polymixin
The polymyxin antibiotic, colistimethate sodium (colistin sulfomethate sodium), is active against Gram- ____________organisms including … (3)
negative
Pseudomonas aeruginosa, Acinetobacter baumanii, and Klebsiella pneumoniae
How is colistimethate sodium administered?
IV; not absorbed by mouth
What is the contraindication to using colistimethate sodium?
Myasthenia gravis
