Respiratory therapeutics 4 & 5 Flashcards
What is cytstic fibrosis
- A genetic autosomal recessive systematic and multiorgan disease
- Caused by a mutation in a gene that encodes cystic fibrosis
- Imbalance of fluid and electrolytes causes thick, sticky mucus in different organs
CF clinical manifestation/symptoms/signs
CFTR mutations cause …
- [CFTR is a protein found in many places like lung epithelium, liver, sweat glands]
- Reduce transciption or translation
- Affect traffinking to surface
- Accelerate protein turnover
- Loss of protein function
Most common type of CFTR mutation
- F508del
- This removes a single amino acid from CFTR protein which means it cant stay in the same shape
- The cell no longer recognises this protein
How do we test for CF
- Blood test: for newborns, test for most common gene
- Sweat test: child/adults with clinical features of the disease, measures amount of salt in sweat
Overview of treatment for pulmonary disease
What is a mucoactive?
- Increase the ability to expectorate sputum (cough up) and decrease mucus hypersecretion
Name three example of mucoactivate drugs
- Inhaled recombinant human DNase I -rhDNase: rhDNase is an enzymethats degrades DNA in mucus, airways clearance
- Hypertonic saline (7%): Inhaler through nebuliser, draws water into airways which helps thin mucus, easier to cough
- Mannitol: Dry powder inhalation, acts as osmotic agent, draws water into airways, makes it less viscous and easier to cough up
What do CFTR corrector treatment do?
- They prevent misfold of CFTR protein
- Enables F508del mutation to fold into a better shape
- Therefor increases trafficking of mature proteins to the cell surfac
Name four examples of CFTR corrector drugs
- Lumacaftor
- Elexacaftor
- Tezacaftor
- Ivacaftor
Chest physiotherapy
- Physical techniques to help loosen and clear thick mucus from lungs
- Postural drainage: patient is put into a specific position to help gravity drain mucus
- Percussion: Therapist clap the chest using cupped hands to loosen the mucus, to cough up
Management of other clinical manifestations
- Increase calorie intake
- Oral nutritional supplements
- Pancreatic enzymes
- Regular excercise
- Antibiotic prophylaxis
What is TB
- Persistant and contagious airborne infectious disease caused by bacteria
- Curable and preventable
What type of bacteria causes TB
- Mycobacterium turberculosis complex
- Different to gram positive or negative
- Walls made of mycolic acids
Who are at risk of developing TB?
Active pulmonary TB clinical signs
- Sputum/phlegm under microscope: acid fast staining
- Chest radiography: non specifc, advanced TB, white/shadowing shows lung infiltration
Initial phase treatment for pulmonary TB
- If clinical signs and symptoms are consistent with a diagnosis of TB start treatment without waiting for culture results!
- Isoniazid
- Rifampicin
- Pyrazinamide
- Ethambutol
Isoniazid for TB: mechanism, pharmokinetics and adverse effects
Rifampicin: mechanism, side effects, adverse, drug interactions
Ethambutol mechanism, pharmokinetics, effects, adverse effects
Pyrazinamide mechanism, effects, pharmokinetics, adverse effects
Diagnosis latent TB
- TB skin testing
- Mantoux test - read 48-72 hours later
- Erythma forms - measure it
Two ways to classify a cough
Management of cough in the uk
