Obstructive Sleep Apnea & Cystic Fibrosis (4)

What organization has guidelines for OSA?

American Academy of Sleep Medicine (AASM)

What is OSA?

Sleep disorder characterized by repeated episodes of complete or partial blockage of the airway during sleep

Name 3 conditions worsened by OSA

Decreased Oxygen, increased carbon dioxide

Fragmented sleep

Negative intrathoracic pressure

Name 3 modifiable risk factors for OSA

Obesity

Cigarette smoking

CNS depressants

Name 3 non-modifiable risk factors for OSA

Male

Older age

Down Syndrome

What are 5 symptoms of OSA?

Loud or frequent snoring

Choking/gasping sounds while sleeping

Insomnia

Impotence or decreased sex drive

Daytime fatigue

What causes catecholamines to be released in OSA? Write down the steps involved

Sleep & airway obstruction → fragmented sleep → Increase in the sympathetic nervous system → increase catecholamines → Increase in HR & BP

What is the test used to diagnose OSA called?

Polysomnography (sleep study)

Mild __AHI/hour

more than 5 events per hour

Mild ___passive daytime sleepiness

± daytime sleepiness

Mild___ADL interference

No major ADL interference

Moderate ___AHI/hour

More than 15 events/hour

Moderate ___daytime sleepiness

some daytime sleepiness

Moderate ___ADL interference

No ADL interference

Severe >___ AHI/hour

more than 30 events per hour

Severe ___daytime sleepiness

daytime sleepiness

Severe ___ADL interference

ADL interference

What is the gold standard treatment for OSA?

CPAP

What are the alternative treatments for OSA?

Oral Appliance Therapy

-Mandibular Advancement Device (MAD)

-GLP-1 Agonists (Zepbound)

-UPPP

-Hypoglossal nerve stimulation

What are 4 of the organ systems affected by CF?

Pancreas

Lungs

Skin

Liver

What does CFTR mean?

Cystic Fibrosis Transmembrane Conductance regulator gene

What chromosome contains the CFTR gene?

Chromosome 7

If two CF carries have children what are the odds of the following scenarios:

1. Child is unaffected ___%

2. Child is a CF carrier __%

3. Child has CF __%

1. 25%

2. 50%

3. 25%

Why does CF have that inheritance pattern?

It has an autosomal recessive inheritance pattern because one copy of the CFTR gene produces enough working protein to keep cells healthy

The CFTR Gene encodes for what protein?

chloride channels

What is the most common CFTR mutation?

F508del

How does CF affect the lungs?

Mucus plugging → infection → inflammation → bronchiectasis

How does CF affect the pancreas?

clogged ducts → pancreatic insufficiency → malabsorption, CF-related diabetes

How is CF diagnosed?

Sweat test (iontophoresis) that measures the chloride concentration in the sweat.

Pilocarpine applied topically, an electrode is used to drive chemical into skin

CFTR modulators require what test before initiating?

Genomic testing

What are the two classes of CFTR modulators?

Potentiators

Correctors

What medication is used before any other for airway clearance therapy?

Albuterol

What are the two osmotic agents that can be used in CF?

Hypertonic saline (7% inhaled nebulized)

Mannitol

How does Dornase Alpha work?

selectively hydrolyzes the extracellular DNA in the sputum of CF patients and reduces sputum viscosity

Why is airway clearance treatment used before antibiotics?

Postural drainage

Percussion treatment

What are 3 antibiotics used in CF?

Tobramycin

Aztreonam (Cayston) (Inhaled)

Colistimethate (Coly-Mycin M)

Tobramycin MOA

interferes with bacteria synthesis by binding to 30S ribosome, results in a defective bacteria cell membrane

Aztreonam MOA

Bindas to bacteria cell wall blocking synthesis and causing cell death

Colistimethate MOA

hydrolyzed to colistin, which acts as a cationic detergent and damages bacterial cytoplasmic membrane