lecture 12 Flashcards
- the role of physiotherapy - the role of antimicrobials - managing pancreatic insufficiency - future therapies: pharmacological, gene therapy
1
Q
What are the goals of treatment in CF?
A
- maintaining lung function:
- treat infections
- airway clearance
- adequate growth
- diet
- supplementation
- managing complications e.g.
- CFRDM
- Liver disease
2
Q
Who is in ‘The CF Team’?
A
- multidisciplinary care
- physicians
- nurses
- physiotherapist
- dietician
- geneticist
- psychologist
- centres of excellence: centres where they have a minimum of 50 patients with CF attending the centre, have access to multidisciplinary team, outcome for patients in terms of morbidity and mortality are significantly better
3
Q
What are some of the physiotherapeutic treatments of CF?
A
Techniques to improve airway clearing/mucus drainage.
In the first year or two of life the form of physiotherapy used are manual techniques i.e. techniques of percussion
- gravity assisted drainage and manual techniques
- modified 5 position technique in infants
- active cycle of breathing techniques (ACBT)
- introduced as blowing games
- autogenic drainage (AD)
- Assisted AD
- Positibe Expiratory Pressure (PEP)
- mask
- mouthpiece (MoPEP)
- combination
- bubble
- activity and exercise lead to bronchodilation and assist in airway clearance, e.g. trampolining
4
Q
How are antimicrobials used in the treatment of CF?
A
- prophylaxis
- exacerbations
5
Q
What are the routes by which an antibiotic can be taken?
A
- oral
- intravenous
- home
- hospital
- nebulised
6
Q
How do we chose what antibiotics to use? What are important things to take into consideration when choosing antibiotics?
A
- knowledge about what are the common pathogens involved in CF lung disease
- access to sputen, cough swabs, and bronchiolalveolar lavage samples (washing samples from lower airways taken via a brinchioscope - a camera with a suction channel passed down into the airways)
- based on the results of these sputen etc can determine what the pathogen is and test against a range of antibiotics to determine what is the most appropriate to deal with that infection
- in a person who is on antibiotics long term there is a risk of developing resistance: seen particularly in older CF patients; start to have airways infected by bacteria that are resistant to a wide range of antibiotics
- tend to give patients (particularly when they are there for a tune up) a minimum of two different antibiotics from different classes to minimise development of resistance
- also important to consider that patients with CF have different pharmacokinetics (i.e. clear the antibiotic differently): tend to require higher doses e.g. aminoglycosides significantly higher doses.
7
Q
Is there a general correlation between age of patient and pathogens they are most likely to acquire?
A
Yes
- staphylococcus aureaus is the most common organism in the younger age group
- Pseudomonas aeruginosa is unusual in the young childhood years but as the child moves through adolescence into adulthood it becomes much more common
- acquisition of pathogens occurs age-wise
- majority eventually acquire Pseudomonas
8
Q
What are some infections commonly spread from patient to patient in CF?
A
Patient to patient spread of infection
- pseudomonas aeruginosa
- Burkholderia cepacia
- MRSA (more of a problem in the US than australia)
9
Q
How can we prevent the spread of infection among CF patients?
A
Segregation of patients
- in-patient: don’t allow patients with CF to interact, have different rooms
- out-patient: when they come into clinic they go into a clinic room and the whole team rotates around them. Rooms are cleaned and disinfected between patients
- social activities: strongly recommend to families that they don’t mix CF children with other CF children. Means they lose peer support. Encourage parents to interact without the child present. Internet contact - link up young people with CF.
10
Q
What are mucolytics?
A
Therapies that work to think the mucus lining the lungs
- recombinant human deoxyribonuclease-1 (Pulmozyme)
- hypertonic saline
- mannitol (nebulise it, lines the lungs, is a sugar so draws water to itself)
11
Q
What some anti-inflammatory agents used in the treatment of CF?
A
- Azithromycin: initially developed as an antibiotic, but discovered to have anti-inflammatory properties, been shown to improve lung function and reduce exacerbations, particularly in those infected with Pseudomonas
- Prednisolone: corticosteroid, strong important anti-inflammatory agent, reduce inflammation and improve lung function but at a huge cost: all corticosteroids have very significant side-effects so have to limit their use in CF
- Inhaled steroids: lower dose, but not particularly effective in improving outcomes in patients with CF
- Ibuprofen: non-steroid, benefit in terms of improving lung function but with significant side effects especially in nephrotoxicity
12
Q
What is the role of nutrition in treating CF?
A
- close monitoring
- specialist dietician
- energy requirements 120-150% normal
- wide variation
- increased expenditure
- increased losses
- reduced intake/impaired appetite
- high fat (35-40%) high protein diet, high salt (i.e. maccas)
- supplemental feeds
- oral
- nasogastric
- gastrostomy (PEG)
- improved growth improves lung function
- replacement of fat soluble vitamins: A, D, E and K
- measure vitamin levels at least once a year and adjust supplements accordingly
- salt replacement
13
Q
How do we treat pancreatic insufficiency?
A
- enteric-coated pancreatic enzyme microspheres e.g. Creon
- quite big so hard to teach patient to swallow (that’s what he said)
- derived from porcine extract
- taken with all fat, protein and complex carbohydrate containing foods
- contain lipase, amylase, protease
- 500-2000 units lipase/kg/meal
- maximum 10,000 units lipase/kg/day
14
Q
What are some future therapies for CF?
A
- novel inhaled antibacterials
- anti-inflammatory agents
- correction of the underlying gene effect
- protein rescue therapy
(high throughput screening and combinatorial chemistry has allowed for the development of some of these treatments)
15
Q
What are the different phases of clinical trials?
A
(usually animal testing prior to human testing)
Phase 1:
- safety and pharmocology
- start with low doses and increase
- healthy volunteers
Phase 2: examine effectiveness - dose - delivery method - dosing interval confirm safety - 100-300 patients
Phase 3 Confirm previous findings Demonstrate safety and efficacy Determine best dosage - 1000+ patients
