Cystic fibrosis Flashcards
Cystic fibrosis is a genetic disorder affecting the lungs, pancreas, liver, intestine, and reproductive organs. List the main clinical signs of cystic fibrosis (3)
1) Pulmonary disease, with recurrent infections
2) Production of copious viscous sputum
3) Malabsorption due to pancreatic insufficiency
Outline the aims of cystic fibrosis treatment (4)
1) Preventing and managing lung infections
2) Loosening and removing thick mucus from the lungs
3) Preventing or treating intestinal obstruction
4) Providing sufficient nutrition and hydration
What is the key predictor of life expectancy in people with cystic fibrosis?
Lung function - optimising lung function is a major aim of care
1) Outline the non-pharmacological management for cystic fibrosis
2) And the non-pharmacological advice forpatients with this condition
1) Specialist physiotherapists provide advice on airway clearance, nebulisers, musculoskeletal disorders, physical activity, urinary incontinence and airway clearance techniques
2) Regular exercise improves both lung function and overall fitness
How often should a review be conducted in patients with cystic fibrosis who have clinical evidence of lung disease?
At least every 3 months
Patients with cystic fibrosis who have evidence of lung disease should be offered a mucolytic. State which mucolytics are used to manage CF
1) Dornase alfa - first choice mucolytic
2) If inadequate response: Dornase alfa + hypertonic sodium chloride (or hypertonic sodium chloride alone)
3) Mannitol dry powder for inhalation if intolerant to Dornase alfa
Pulmonary infections are common in cystic fibrosis. List the causative organisms that are involved (6)
1) Staphylococcus aureus (+ve)
2) Pseudomonas aeruginosa (-ve)
3) Burkholderia cepacia complex (-ve)
4) Haemophilus influenzae (-ve)
5) Non-tuberculous mycobacteria
6) Aspergillus fumigatus complex
Outline the management of a pulmonary infection caused by Staphylococcus aureus
1) If no prophylaxis, new infection & clinically unwell = oral anti-Staph. aureus agent e.g. Flucloxacillin
↳Clinically unwell + pulmonary disease: oral or IV broad spec antibiotic
2) Do not routinely use antibiotics to suppress chronic MRSA in people with stable pulmonary disease
If a person with cystic fibrosis develops a new pseudomonas aeruginosa infection how should they be treated?
1) Eradication therapy with oral + inhaled antibacterial
2) Then extended course of oral + inhaled antibiotics
3) If not successful - sustained treatment with nebulised colistimethate sodium
How should patients with cystic fibrosis who develop a new Burkholderia cepacia complex infection be treated?
Regardless if clinically well or not, give antibiotic eradication therapy using a combination of IV antibiotics
How should patients with cystic fibrosis who develop a Haemophilus influenzae infection be treated?
1) If no pulmonary infection - treat with oral antibacterial
2) Unwell with pulmonary infection - oral or IV antibacterial, depending on the severity
When should treatment with an antifungal drug be considered to suppress chronic Aspergillus fumigatus complex respiratory infections?
In patients with declining pulmonary status
Long-term treatment with azithromycin is offered to patients with deteriorating lung function or repeated pulmonary exacerbations. What can be considered if this fails?
Discontinue oral azithromycin and start an oral corticosteroid
1) Pancreatic insufficiency is the most common GI complication of cystic fibrosis and can lead to malabsorption. What drug should be offered to those with exocrine pancreatic insufficiency?
2) What drug can also be considered for patients who have persistent signs of malabsorption despite treatment and why?
1) Pancreatin - assists digestion of starch, fat, and protein
2) An acid-suppressing drug: H2 receptor antagonist or a PPI [unlicensed]
↳ Acid suppression may improve the effectiveness of pancreatin
Distal intestinal obstruction syndrome occurs in about 20% of individuals with cystic fibrosis. How should this be managed?
1) Oral or IV fluid to ensure adequate hydration
2) First line: Meglumine amidotrizoate with sodium amidotrizoate solution
3) Second line: Macrogols
What drug is used for abnormal LFTs in cystic fibrosis?
Ursodeoxycholic acid [unlicensed] can be given until liver function is restored
What should be monitored in those with cystic fibrosis? (3)
1) Cystic fibrosis-related low bone mineral density
2) Cystic fibrosis-related diabetes
3) Liver disease
Which parts of the body does cystic fibrosis affect? (5)
1) Lungs
2) Pancreas
3) Liver
4) Intestine
5) Reproductive organs
What is the most common GI complication of cystic fibrosis and what can it lead to?
Pancreatic insufficiency
Can lead to malabsorption
How does pancreatin help those with CF?
Pancreatin assists digestion of starch, fat, and protein
To prevent malabsorption due to pancreatic insufficiency
What can be done to improve the effectiveness of pancreatin?
Acid suppression - PPI [unlicensed] or H2 antagonist
Limited evidence
If still signs of malabsorption despite using pancreatin
What causes cystic fibrosis?
Mutated CFTR gene
Recessive disease - must have mutation in both copies of CFTR gene to have CF
What makes someone a carrier for CF rather than have CF themselves?
CF is a recessive disease - must have 2 copies of mutated CFTR gene to ave CF
Carrier = only has 1 copy of CFTR gene with mutation, and 1 normal CFTR gene
What is the role of the CFTR gene?
The CFTR gene codes for the CFTR protein
CFTR protein = Transmembrane protein for cells that produce mucus, sweat, saliva, tears and digestive enzymes
Acts as a channel for Cl- ions in and out of cells
This helps to control water movement in tissues - for thin, freely-flowing mucus
This lubricates and protects the lining of the airways, digestive system and reproductive systems
What is long term azithromycin used for in CF?
Patients with deteriorating lung function or repeated pulmonary exacerbations
Stop and start oral corticosteroid if ineffective
When are oral corticosteroids used in CF?
Started if long term azithromycin fails to help patients with deteriorating lung function or repeated pulmonary exacerbations
