cystic fibrosis Flashcards
what is it and what organs does it affect
Cystic fibrosis is a genetic disorder affecting the lungs, pancreas, liver, intestine, and reproductive organs
main clinical signs (3)
pulmonary disease, with recurrent infections
the production of copious viscous sputum
malabsorption due to pancreatic insufficiency
name 4 complications
hepatobiliary disease, osteoporosis, cystic fibrosis-related diabetes, and distal intestinal obstruction syndrome.
aims of treatment
preventing and managing lung infections
loosening and removing thick, sticky mucus from the lungs
preventing or treating intestinal obstruction
providing sufficient nutrition and hydration.
what is a key predictor of life expectancy in people with CF
Lung function is a key predictor of life expectancy in people with cystic fibrosis and optimising lung function is a major aim of care.
treatment for CF lung disease is based on …
prevention of lung infection and the maintenance of lung function
In patients with cystic fibrosis, who have clinical evidence of lung disease, the frequency of routine review should be based on their clinical condition, but adults should be reviewed at least
3 monthly
when to offer mucolytic
Patients with cystic fibrosis who have evidence of lung disease should be offered a mucolytic.
1st choice mucolytic, and alternatives
Dornase alfa is the first choice mucolytic.
If there is an inadequate response, dornase alfa and hypertonic sodium chloride, or hypertonic sodium chloride alone should be considered.
When can mannitol dry powder for inhalation be used as an option for mucolytics
(dornase alfa is first line)
use 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
offer long term treatment with azithromycin unlicensed at an immunomodulatory dose should be offered to pt with …
deteriorating lung function or repeated pulmonary exacerbations
Long-term treatment with azithromycin [unlicensed indication], at an immunomodulatory dose, should be offered to patients with deteriorating lung function or repeated pulmonary exacerbations.
What should you do in those patients with continued deterioration in lung function or continuing pulmonary exacerbations
long-term azithromycin should be discontinued and the use of an oral corticosteroid considered.
Offer the following to pt with exocrine pancreatic insufficiency
Pancreatin
Adjust dose as needed to minimise any symptoms or signs of malabsorption
True or false
An acid-suppressing drug, such as an H2 receptor antagonist or a proton pump inhibitor [unlicensed indications] can be considered for patients who have persistent symptoms or signs of malabsorption.
True
If LFTs are abnormal in pt with CF, this can be given unlicensed until liver function is restores
ursodeoxycholic acid
