Cystic Fibrosis/sleep Disorders

Front 1

CF is most common in what ethnic group?

It has what type of inheritance?

Back 1

Caucasians

autosomal recessive inheritance pattern*

Front 2

CF results from mutations in the _ gene, which leads to _

Back 2

mutations in CFTR gene, leading to defective epithelial cell ion transport

Front 3

What effects does CF have on the GI Tract?

Back 3

-meconium ileus (unable to poop)**
-distal intestinal obstruction*
-biliary cirrhosis/portal HTN*
-obstructive jaundice*
-rectal prolapse*
-pancreatitis*

Front 4

What effects does CF have on nutrition?

Back 4

malabsorption
-steatorrhea
-failure to thrive
-protein deficiency
-fat soluble vitamin deficiency (fat soluble vitamins= ADEK)

Front 5

Patients with CF tend to have _ puberty, _ fertility, _ sodium levels, and what is different about their hands?

Back 5

delayed puberty,
impaired fertility,

hyponatremia

digital clubbing

Front 6

To evaluate for CF, you should look at _

Back 6

sweat chloride,

genetic screening

(also can include chest x rays, spirometries, fecal fat, etc)

Front 7

Bronchiectasis is a _ predisposition to _

Back 7

A genetic predisposition will lead to altered bronchial dynamics which contributes to ineffective mucus clearance, which will lead to chronic or recurrent infection, which leads to bronchial wall inflammation and destruction, which leads to even further altered bronchial dynamics and the cycle repeats itself.

Front 8

Clinical symptoms of bronchiectasis include _

Back 8

-chronic cough
-mucopurulent secretions
-dyspnea
-hemoptysis
-fever
-fatigue
-pleuritic chest pain
-weight loss

Front 9

Early in life, the most common infection associated with CF is _, but as patients get older, it is more common to have _

Back 9

Staph

P aeruginosa
(85% of CF patients have pseudomonas)*

Front 10

What are the goals of treating CF?

Back 10

:* limit irreversible lung damage, limit infectious exacerbations, increase lung function, decrease respiratory symptoms, minimize antibiotics, decrease school/work missed, decreased respiratory symptoms, improve quality of life

Front 11

What are available therapies for CF?

Back 11

bronchodilator therapy (as used with asthma), chest physiotherapy, oral/IV antibiotics, nebulized antibiotics, mucolytics, anti-inflammatory therapies, enzyme/vitamin supplments

Front 12

_ is prolonged partial and/or intermittent complete upper airway obstruction that disrupts normal ventilation and patterns during sleep**

Back 12

Obstructive Sleep Apnea –

Front 13

Obstructive Sleep apnea in adults is cessation of breathing for _, and in children it is _

Back 13

cessation for 10+ sec


Children: 2.5 missed breaths

Front 14

Upper airway collapse caused by _ or _

Back 14

anatomic factors or abnormal neuromuscular tone or both

Front 15

T/F Obstructive Sleep Apnea is worse in Stage II sleep, which makes up 50% of sleep time.

Back 15

False.

Apnea is worse in REM sleep. Patients aren't able to have full REM cycles

Front 16

During Apnea, O2 levels _, and CO2 levels _

Back 16

go down

go up

Front 17

There is a higher occurence of sleep apnea in children 2-6 y/o because of _

Back 17

adenoidal/tonsillar hypertrophy

Front 18

The gold standard to diagnose sleep apnea is _

Back 18

full observed sleep study

Front 19

Surgical treatment of sleep apnea includes _

Back 19

i. Adenotonsillectomy
ii. Uvulopalatopharyngoplasty

Front 20

CPAP and BiPAP are therapies to treat _

Back 20

sleep apnea

Front 21

Sudden infant death syndrome (SIDS) risk factors include _

Back 21

cats and smokers in the house, sleeping on stomach