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Respiratory: Chronic Infections > CF clinical > Flashcards

CF clinical Flashcards

1
Q

what are the characteristics of CF patients?

A

bronchiectasis under 40

upper lobe bronchiectasis

colonisation with staph aureus

infertility

low weight

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2
Q

1st patient case

A

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

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3
Q

case 1 - examination

A

BMI 19

crackles in both upper zones

otherwise normal

CXR shows signet ring

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4
Q

case 1 - what she doesnt mention?

A

younger sister has CF

Another sister died age 8 with “A Respiratory Illness

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5
Q

case 1 - tests

A

Del508
Confirms diagnosis of Cystic Fibrosis
Referred on from my normal clinic to the CF service

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6
Q

case 2

A

65 Year old woman
Asthma since childhood
Frequent chest infections

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7
Q

case 2 - examinations

A

Normal pulmonary function
No Allergies
No Wheeze
More frequent chest infections over past 6 months

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8
Q

case 2 - clinical course

A 
HRCT
Sputum Culture
Staph Aureus
Flucloxacillin
Repeat Culture
Staph Aureus
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9
Q

what are the clinical features of CF for neonates?

A

Failure to thrive; meconium ileus; rectal prolapse.

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10
Q

what are the clinical respiratory features of CF for children and young adults ?

A

cough; wheeze; recurrent infections; bron- chiectasis; pneumothorax; haemoptysis; respiratory failure; cor pulmonale.

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11
Q

what are the clinical GI features of CF for children and young adults ?

A

pancreatic insufficiency (diabetes mellitus, steatorrhoea); distal intestinal obstruction syndrome (meconium ileus equivalent); gallstones; cirrhosis.

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12
Q

what are the other clinical features of CF for children and young adults ?

A

male infertility; osteoporosis; arthritis; vasculitis ; nasal polyps; sinusitis; and hypertrophic pulmonary osteoarthropathy (HPOA). Signs: cyanosis; finger clubbing; bilateral coarse crackles.

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13
Q

how is a diagnosis made?

A

sweat test

genetics - screening for known common mutations

faecal elastase is a simple and useful screening test for exocrine pancreatic dysfunction

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14
Q

what are the features of sweat test of CF?

A

sweat sodium and chloride >60mmol/L;

chloride usual- ly > sodium

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15
Q

what things are analysed in blood tests?

A

FBC, U&E, LFT; clotting; vitamin A, D, E levels; annual glucose tolerance test

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16
Q

how is bacteriology assessed?

A

cough swab, sputum culture.

17
Q

what radiological tests are done and what would they show?

A

CXR; hyperinflation; bronchiectasis.

Abdominal ultrasound: fatty liver; cirrhosis; chronic pancreatitis

18
Q

what would spirometry show?

A

obstructive defect

19
Q

how are patients with CF best managed?

A

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.

20
Q

what happens in exocrine failure ?

A

Sludged up ducts
Failure of secretion of lipases, amylase
Digestive failure

21
Q

what is the treatment for this prophylactically

A

CREON

Patients hate taking it
Without it they can’t absorb energy

22
Q

what happens in endocrine failure

A

destruction of pancreatic islet cells

fatty replacement of pancreatic tissue

23
Q

what is the treatment for endocrine failure prophylactically

A

Annual OGTT
CGMS

Usually need insulin as they have insulin production failure

24
Q

what happens to the bowels in CF?

A

Thick mucus blocks up the large and small intestine

Symptoms similar to constipation

25
Q

what is used in the treatment and prevention of these bowel problems

A

Treatment:
Gastrograffin
Laxido
Fluids

Prevention:
Laxido
Hydration
Keep moving

26
Q

what happens to the liver in CF?

A

sludging up of the hepatic ducts
EITHER INTRA HEPATIC OR EXTRA HEPATIC

portal hypertension

27
Q

what does portal hypertension cause?

A

Porto-systemic anastamoses
Variceal Bleeding
Hepatic encephalopathy

28
Q

what is the treatment for liver problems

A

TIPSS

29
Q

what does TIPSS do?

A

Reduces anastamoses
Reduction in bleeding risk
Can increase encephalopathy risk

30
Q

how are exacerbations managed?

A

antibiotics

physiotherapy

adequate hydration

increased dietary input

31
Q

what is the physiotherapy treatment?

A

Autogenic Drainage
ACBT
With and without a physiotherapist

32
Q

why are two antibiotics always given together?

A

for resistance reduction

33
Q

what oral antiBs are given?

A 
Augmentin
Fluclox
Minocycline
Septrin
Fusidin
Ciprofloxacin
34
Q

what IV antiBs are given for pseuomonas?

A 
Tazocin
Ceftazidime
Tobramicin
Meropenem
Colistin
35
Q

what IV antiBs are given for staph aureus?

A

Flucloxacillin

Tigecycline

36
Q

what IV antiBs are given for cepacia ?

A

Temocillin

37
Q

what is the treatment for G551D/

A

ivacaftor

38
Q

what does ivacaftor do?

A

CFTR Potentiator

Improves Chloride Flow through the CFTR

$194,000 per year

Tablet, twice a day

Beware Grapefruit
No other ‘entry’ requirements

39
Q

what is a new treatment for F508 mutation?

A

lumacaftor
Phase II trials
8.5% improvement in FEV1 at 56 Days

Respiratory: Chronic Infections (6 decks)

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