cystic dibrosis in adults Flashcards
whats the ratio of people carrying cystic fibrosis?
1 in 25
if a person was a carrier for CF and they met someone thats also a carrier for CF whats the likelyhood of there child having CF?
1 in 4
why has survival of CF got much better in recent years?
CF centres MDT teams Physio Nutrition/Enzymes Antibiotics
what are the challenges for patients with CF?
Transition is a major challenge. New MDT team
Prognosis
Promise of new drugs
Possibility of lung transplant
Other conditons: Diabetes, liver disease, osteoporosis, fertility issues, haemoptysis, mental health issues – can develop anything else not related to CF
Media/Social media pressure and misinformation.
what are common presentations for adults with cystic fibrosis?
infertillity bronchiectasis Mild resp symptoms/recurrent LRTI (40%) Hyperinflation Clubbing
is CF a multisystem disease?
yes
why does pulmonary infection occur?
decreased mucociliary clearance
increased bacterial adherence
decreased endocytosis of bacteria
a person with CF that had a ct scan would show:
tramlines
signet rings
mucous plugging
consolidation
what initiates type 2 diabetes mellitus in CF?
falling lung function, obesity
do you ever see type 1 diabetes in cystic fibrosis patients?
no
what are issues with cystic fibrosis when patients have diabetes?
Compliance with diet a problem
low sugar/high fibre diet not appropriate
OGTT /HBA1C used but not perfect
Insulin of benefit, not so much oral hypoglycaemics
Complication risk same as non CF pts but pulmonary disease gets there first
how is osteoporosis affected with CF?
slower gain, faster loss of bone mass
why does bone mass density decrease with CF patients?
Malnutrition and low BMI Steroids Delayed puberty and hypogonadism Inflam cytokines from sepsis (we know BMD falls in septic pts) Vit D/K defic Lung Tx/drugs
whats the treatment for patients with CF that experiences pneumothorax?
sam treatment as other people,
drain, pleurodesis, surgery
what gender is more likely to get pneumothorax and CF
both as likely as each other
what is HAEMOPTYSIS?
coughing up blood that comes from the lungs or bronchial tubes
what are risk factors of haemoptysis?
Severity, Exacerbations, Fungal, liver disease, Vit K def
what are the bacterial pathogens involved with cystic fibrosis?
pseudomonas aeruginosa
staphylococcus aureus
BURKHOLDERIA CEPACIA
where is pseudomonas aeroginosa acquired from?
Environment (particularly hospitals)
Other CF patients (epidemic strains, antibiotic resistant)
Segregation and disinfection policies
where is BURKHOLDERIA CEPACIA acquired from?
Environment
Other CF patients (epidemic strains more virulent)
Segregation policies
what is burkholderia cepacia’s colonisation associated with?
reduced life expectancy 16 vs 39 years
rapid decline in lung function
Do worse in pregnancy
Some patients ‘cepacia syndrome”
does aspergillus come from environment or people?
environment
how do you manage pulmonary infection?
Treat early and aggressively with antibiotics
Oral antibiotics (eg Staph, Haemophilus, Pneumococcus)
IV antibiotics (eg PA, Stenotrophomonas, Burkholderia)
Two antibiotics, liaise with microbiology
If multiply resistant, test for synergy between antibiotics Large doses (inc volume distribution, increased clearance)
Two week courses
Indwelling subcutaneous ports (vascuports)
Home administration
Side effects, interactions, allergies
are nebulisers or inhaled antibiotics used instead of iv?
no
why might CF patients have airway obstruction?
Asthma/Atopy
BHR in 40% CF pts.
- Mechanical
Bronchial plugging, Inflammation Bronchial wall thickening - Many pts (esp milder disease) - increase in FEV1 despite absence typical asthma symptoms
what are mucolytics used for?
reduce viscocity of phlegm
