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Cystic Fibrosis - Coggle Diagram
Cystic Fibrosis
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Diagnosis
- Sweat test - CL > 60mmol/L = confirmed CF (NR 10-20)
- New born screening - Immuno Reactive Trypsin as part of the Guthrie test.
Population screening?? - Cost, ethical and religious issues
- if CF postive, what would you do to the fetus? - very unethical
Historically - failure to thrive, chronic respiratory infections and underweight. Meconium ileus (GIT obstruction) at birth, male infertility, pancreatitis, liver disease.
Child: Coughing, hot, ill and distressed - potential CF?
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Treatment stratgies
Bacteria in children: Haemophilus and Staphyloccous
Later in life: Pseudomonas Aeruginosa in almost all pateints.
- PA Non-mucoid: essentially no biofilm yet thus can be treated effectively. Recommended to treat aggressively to prevent Mucoid stage.
- PA Mucoid: Very difficult to treat and drugs have reduced effectiveness
Aggressive treatment of lung disease
- Specialised CF clinic
- Optismising lung function + Physiotherapy
- Optimise nutrition
- Aggressive antibiotics (oral or inhaled, IV if required)
- Review and monitor
We can clear the intial infection but eventually regrows and and cycle continues, constantly damaging lung function
Pseudomonas Colonisation: poorer PFTs and prognosis
- Can be delyaed with first growth treatment but eventually colonised and unlikely to eradicate.
- Detection of growth via broncoscopy or given saline to cough and discharge is sent for culture.
- Eradication protocols are different based on hospital with > 80% success rate but regrows in 1-2 years.
Acute treatment
May initially try oral Ciprofloxiacin + inhaled tobramycin (or colistin) for 2 weeks - to avoid hospital
2 anti-PAs IV + inhaled anti-PA with intensive physiotherapy to push mucous out, at hospital or home, major advantage in hosp. is physio therapy
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Anti-PAs = Ciprofloxacin, Tobramycin, Colistin
Classification
Most common life-shortening genetic disorder in Caucasians.
Genetic mutation in gene encoding CFTR protein - functions as a Cl channel
- Moves Cl out of cells into airways
- Na follows thus water follows
= Keeps the moist and functional
Reduction/No CFTR function = less moist and less functional airways
- viscous secretions sit in airways causing infection, and inflammation, causing lung damage
- Also impairs HCO3 transport, decreasing GIT pH
- and reduced hydration to other organs
Other issues due to reduced CFTR function
- Reduced pancreas function, reducing fat absorption, thus reduced fat soluble vitamins and minerals absorption.
- GIT pH changes, can cause absorption issues.
- Excessive loss of NaCI in sweat, causing dehydration
- Lung inflammation.
Goals of therapy
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- Maintain lung function and reduce the frequency of exacerbations. If more exacerbations occur, more problems will occur down the track
- Give and maintain adequate nutrition to maintain the lung function. Low BMI is a predictor of mortality and high BMI is associated with better lung function.
Symptoms
Increased sputum, coughing, weight loss, fall in PFTs
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Role of Pharmacists
- Provide education and counselling
- Drug-dose advice: altered PK and polypharmacy
- Family and patient advice + support
- Adherence and monitoring/HMRs