CF and Otitis media

Question 1

What is CF?

Answer 1

Cystic Fibrosis (CF) is an inherited autosomal recessive disease that disrupts ion transport in epithelial-lined organs, including pulmonary airways, sweat ducts, pancreatic ducts and intestine

Question 2

Genetics and phenotypes of CF

Answer 2

Mutation of CFTR gene on chromosome 7. Recessive. Can have varying degrees of problems. Common mutation is ∆F508.
Also affected by other genes and environment.
Classic: early age Dx, pancreas ø, high sweat Cl
Mild: older age Dx, pancreas ok, sweat Cl more normal

Question 3

Pathophysiology of CF

Answer 3

Abnormal Cl channels on epithelium leading to changes in electrolytes and hence fluid changes, which results in clogged ducts and thick sticky secretions. This leads to problems in:
Airways: Recurrent atypical organisms, hemoptysis, sinusitis, nasal polyps, pneumothorax
GI tract: Pancreatic disease, Constipation/bloating
Liver and biliary disease
Male infertility (some risk in females)
Salty sweat

Question 4

Common presentations of CF

Answer 4

Recurrent lung infections, often with unusual pathogens (funny bugs)
Obstructive airways disease – cough, thick sticky sputum, wheeze, dyspnea
Low BMI/failure to thrive
Abdominal bloating, constipation/diarrhea

Question 5

Physical exam and other findings

Answer 5

Clubbing, osetoporosis, diabetes symptoms, infertility

Question 6

Investigations for CF

Answer 6

CXR: Bronchiectasis (see thick membranes like train track), Hyperinflation, Fibrosis (white lines = scarring)

Question 7

Diagnosis of CF

Answer 7

Clinical: Positive newborn screen, Clinical symptoms, Positive fam Hx
Tests: Sweat Chloride test*, Genotyping, Nasal potential difference

Question 8

Tx for CF

Answer 8

Improve mucociliary transport, airway clearance: Chest physiotherapy – percussions, vibrations; Postural drainage, Hypertonic saline, Mucolytics 
O2 at home
Antibiotics
Reduce inflammation
Lung transplant (but difficult)
Nasal rinses/Sx
Nutrition supplements
Tx for constipation, bone density, diabetes

Question 9

Define: otitis media with effusion (OME) and acute otitis media

Answer 9

Otitis media is just fluid into the middle ear via eustachian tube; painless, no Tx except tympanostomy tubes maybe. Acute is if this gets infected

Question 10

Potential complications of OME

Answer 10

hearing loss when bilateral; speech and language delay when prolonged in kids; progression to acute otitis media

Question 11

Who is at risk for the development of OME and acute otitis media?

Answer 11

More in children. Bottle feeding = different position than breastfeeding so more likely. Kids exposed to smoke. Kids with facial anomalies (cleft palate). Males at higher risk. Kids at day care more likely.

Question 12

Discuss the natural history of acute otitis media

Answer 12

Acute otitis media usually results in pain for a few hours to days, and then resolves without sequelae, but is always followed by days to weeks of otitis media with effusion before the fluid totally goes away.
Rarely, tympanic membrane rupture, but this normally heals fine
Usually preceded and followed by effusion

Question 13

Classic symptoms and signs of acute otitis media

Answer 13

Usually symptoms of a “cold” (acute otitis media usually starts near the end of a cold)
Intermittent severe earache
Sometimes fever
Hearing loss, but children rarely mention this symptom
Irritable
Pulling on ear (child)

Question 14

Make use of otoscopic images to distinguish between a) a normal tympanic membrane, b) OME and c) acute otitis media

Answer 14

Effusion: amber coloured drum. Sometimes can see bubbles. The drum is usually retracted, does not move normally with air puff.
Acute: Ear drum might not be red in AOM. Puss under pressure behind.

Question 15

Discuss the potential complications of otitis media

Answer 15

Pain, perforation of the tympanic membrane, hearing loss.

Rare: mastoidistis (mastoid bone), meningitis, brain abscess

Question 16

When does a patient with acute otitis media need antibiotics?

Answer 16

Most over 6mo and no fever don’t need antibiotics and will get better w/o them within 48 hours. About 10% do need it.
Antibiotics don’t limit effusion or prevent complications.

Question 17

Which microorganisms most commonly cause acute otitis media?

Answer 17

Streptococcus pneumoniae, Haemophilus influenza, Morraxella catarrhalis, group A and B Streptococcus and Staphlococcus aureus

Question 18

Recall the most appropriate antibiotics to be used in the management of acute otitis media

Answer 18

Amoxicillin usually best, 5 days.

If allergic or recently has had it: TMP/SMX, erythromycin, long list.

Question 19

Discuss the management of a patient who is failing to respond to initial treatment

Answer 19

Could be wrong diagnosis, viral infection, resistance, or compliance. Upgrade to amoxi-clav for 5 days.

Question 20

What do you do with a kid who has recurrent AOM or hearing loss?

Answer 20

Try to change lifestyle/daycare/hygiene, then maybe tympanostomy tubes (drain fluid so nothing to get infected)

Question 21

Discuss primary prevention of OME and acute otitis media

Answer 21

breast feeding (better positioning plus antibodies); avoid cigarette smoke; vaccination

Question 22

Presentation of otitis externa

Answer 22

Presents as pain in the pinna – much worse if you move the pinna
Topical antibiotics +/- topical steroids are treatment of choice (eg. Polysporin or Sofracort ear drops)