CF clinical Flashcards
what are the characteristics of CF patients?
bronchiectasis under 40
upper lobe bronchiectasis
colonisation with staph aureus
infertility
low weight
1st patient case
has persistent cough
frequent chest infections
frequent courses of antibiotics
occasional chest pains
moves around a lot so doesn’t keep the same GP for a very long time
case 1 - examination
BMI 19
crackles in both upper zones
otherwise normal
CXR shows signet ring
case 1 - what she doesnt mention?
younger sister has CF
Another sister died age 8 with “A Respiratory Illness
case 1 - tests
Del508
Confirms diagnosis of Cystic Fibrosis
Referred on from my normal clinic to the CF service
case 2
65 Year old woman
Asthma since childhood
Frequent chest infections
case 2 - examinations
Normal pulmonary function
No Allergies
No Wheeze
More frequent chest infections over past 6 months
case 2 - clinical course
HRCT Sputum Culture Staph Aureus Flucloxacillin Repeat Culture Staph Aureus
what are the clinical features of CF for neonates?
Failure to thrive; meconium ileus; rectal prolapse.
what are the clinical respiratory features of CF for children and young adults ?
cough; wheeze; recurrent infections; bron- chiectasis; pneumothorax; haemoptysis; respiratory failure; cor pulmonale.
what are the clinical GI features of CF for children and young adults ?
pancreatic insufficiency (diabetes mellitus, steatorrhoea); distal intestinal obstruction syndrome (meconium ileus equivalent); gallstones; cirrhosis.
what are the other clinical features of CF for children and young adults ?
male infertility; osteoporosis; arthritis; vasculitis ; nasal polyps; sinusitis; and hypertrophic pulmonary osteoarthropathy (HPOA). Signs: cyanosis; finger clubbing; bilateral coarse crackles.
how is a diagnosis made?
sweat test
genetics - screening for known common mutations
faecal elastase is a simple and useful screening test for exocrine pancreatic dysfunction
what are the features of sweat test of CF?
sweat sodium and chloride >60mmol/L;
chloride usual- ly > sodium
what things are analysed in blood tests?
FBC, U&E, LFT; clotting; vitamin A, D, E levels; annual glucose tolerance test
how is bacteriology assessed?
cough swab, sputum culture.
what radiological tests are done and what would they show?
CXR; hyperinflation; bronchiectasis.
Abdominal ultrasound: fatty liver; cirrhosis; chronic pancreatitis
what would spirometry show?
obstructive defect
how are patients with CF best managed?
Patients with cystic fibrosis are best managed by a multidisciplinary team, eg physician, GP, physiotherapist, specialist nurse, and dietician, with attention to psy- chosocial as well as physical wellbeing. Gene therapy (transfer of CFTR gene using liposome or adenovirus vectors) is not yet possible.
what happens in exocrine failure ?
Sludged up ducts
Failure of secretion of lipases, amylase
Digestive failure
what is the treatment for this prophylactically
CREON
Patients hate taking it
Without it they can’t absorb energy
what happens in endocrine failure
destruction of pancreatic islet cells
fatty replacement of pancreatic tissue
what is the treatment for endocrine failure prophylactically
Annual OGTT
CGMS
Usually need insulin as they have insulin production failure
what happens to the bowels in CF?
Thick mucus blocks up the large and small intestine
Symptoms similar to constipation
what is used in the treatment and prevention of these bowel problems
Treatment:
Gastrograffin
Laxido
Fluids
Prevention:
Laxido
Hydration
Keep moving
what happens to the liver in CF?
sludging up of the hepatic ducts
EITHER INTRA HEPATIC OR EXTRA HEPATIC
portal hypertension
what does portal hypertension cause?
Porto-systemic anastamoses
Variceal Bleeding
Hepatic encephalopathy
what is the treatment for liver problems
TIPSS
what does TIPSS do?
Reduces anastamoses
Reduction in bleeding risk
Can increase encephalopathy risk
how are exacerbations managed?
antibiotics
physiotherapy
adequate hydration
increased dietary input
what is the physiotherapy treatment?
Autogenic Drainage
ACBT
With and without a physiotherapist
why are two antibiotics always given together?
for resistance reduction
what oral antiBs are given?
Augmentin Fluclox Minocycline Septrin Fusidin Ciprofloxacin
what IV antiBs are given for pseuomonas?
Tazocin Ceftazidime Tobramicin Meropenem Colistin
what IV antiBs are given for staph aureus?
Flucloxacillin
Tigecycline
what IV antiBs are given for cepacia ?
Temocillin
what is the treatment for G551D/
ivacaftor
what does ivacaftor do?
CFTR Potentiator
Improves Chloride Flow through the CFTR
$194,000 per year
Tablet, twice a day
Beware Grapefruit
No other ‘entry’ requirements
what is a new treatment for F508 mutation?
lumacaftor
Phase II trials
8.5% improvement in FEV1 at 56 Days
