T5 - IE4 - Pulmonology - Munjy - Cystic Fibrosis

Background of CF: ______________ _________ disease

- autosomal recessive (disease)

Background of CF: mutation of ________ gene

- CFTR (gene)

Background of CF - mutation of CFTR gene

regulates intracellular Cl- ions

electrochemical equilibrium is disturbed

adaptive responses lead to increased absorption of Na+ and this leads to dehydration of epithelial surface

Mucociliarly clearance declines

Background of CF - mutation of CFTR gene: regulates ______________ ____ ions

- intracellular Cl- (ions)

electrochemical equilibrium is disturbed

Background of CF - mutation of CFTR gene: _______________ ______ is disturbed, due to dysregulation of intracellular Cl-

- electrochemical equilibrium (is disturbed)

Adaptive response leads to increased absorption of Na+ and this leads to dehydration of epithelial surface

Background of CF - mutation of CFTR gene: adaptive response from dysregulation of intracellular Cl- ions leads to ____________ absorption of _____ and this leads to ___________ of epithelial surface

- increased (absorption)

- Na+

- dehydration of (epithelial surface)

mucociliary clearance declines

Background of CF - mutation of CFTR gene: adaptive response leads to increased absorption of Na+ and this leads to dehydration of epithelial surface

_____________ __________ declines

- mucocilliary clearance (declines)

Background of CF - median predicted survival is ______ years

- 39.3 (years)

Occurs in 1:3200 caucasians, 1:10,000 hispanics, 1:15,000 African-Americans, 1:30,000 Asian Americans

Diagnostics of CF

elevated immunoreactive trypsinogen (IRT) level in serum at birth

increased sweat chloride concentration

Diagnostics of CF: increased sweat chloride concentration

< 29 mmol/L - normal

30-59 mol/L: POSSIBLE CF (confirm with DNA testing and/or subsequent sweat test)

> 60 mmol/L: abnormal

- considered diagnostic if confirmed by second test + symptoms of CF present

Clinical features of CF

respiratory features

gastrointestinal features

musculoskeletal disorders

CF

cystic fibrosis

Clinical features of CF - respiratory features

infections

recurrent, productive cough, wheezing, crackles

hemoptysis, pneumothorax in later stage disease

Clinical features of CF - gastrointestinal features

pancreatic insufficiency

nutritional deficiency

inability to absorb nutrients

inability to digest food

Respiratory management of CF: the leading cause of morbidity and mortality is due to _____________ complications

- respiratory (complications)

Respiratory management of CF: CFTR modulators

requires CFTR genotyping to determine efficacy

Respiratory management of CF: bronchodilators

short-acting β-2 receptor agonist

Dosed 2 puffs prior to therapy 2-4 times per day

Respiratory management of CF - bronchodilators: short-acting β-2 receptor agonist dosing

Dosed 2 puffs prior to therapy 2-4 times per day

Respiratory management of CF - hypertonic saline

improves mucocillary function

improves FEV1, FVC and SOB

Respiratory management of CF - Dornase alpha

enzyme to cleave intracellular DNA and decrease viscosity of mucous

contraindicated in patients with Chinese Hamster Ovary products

Respiratory management of CF - Dornase alpha: enzyme to ______ intracellular DNA and decrease _________ of mucus

- cleave (intracellular DNA)

- (decrease) viscosity (of mucus)

Respiratory management of CF - Dornase alpha: contraindications

patients with hypersensitivity to Chinese hamster ovary products

Respiratory management of CF - chest percussion

percussion vest or manual with cupped hand

- airway clearance therapy

- chest percussion and/or percussion vest

Respiratory management of CF - chest percussion: _________ vest or manual with ________ hand

- percussion (vest)

- (manual with) cupped (hand)

Hypertonic saline

HyperSal, PulmoSal

Hypertonic saline (HYPERSAL, PULMOSAL): sodium ________ water into airways and ______ mucus in respiratory tract

- attracts (water into airways)

- thins (mucus in respiratory tract)

Hypertonic saline (HYPERSAL, PULMOSAL): ________ attracts water into airways and thins mucus in _________ _______

- sodium (attracts water)

- (thins mucus in) respiratory tract

Hypertonic saline (HYPERSAL, PULMOSAL) availability

3%, 3.5% and 7% solutions

Given BID via nebulizer (or as directed by CF care team)

Hypertonic saline (HYPERSAL, PULMOSAL) should be given right before ___________ __________ __________ techniques

- conducting airway clearance (techniques)

Dornase alfa

PULMOZYME

Dornase alfa (PULMOZYME) dosing

2.5 mg 1-2 times per day via nebulizer

Dornase alfa (PULMOZYME): is approved in ages ____ and ________

- (ages) 5

- older

Dornase Alfa supports use in younger age

Dornase alfa (PULMOZYME): keep __________ and protect from _______ until ready to use

- (keep) refrigerated

- (protect from) light

Dornase alfa (PULMOZYME) side effects

change in or loss of voice

throat discomfort

red, watery eyes

rash

dizziness

fever

runny nose

Steps to respiratory therapy of CF

1) bronchodilators

2) hypertonic saline

3) dornase alpha

4) chest percussion

5) inhaled antibiotics

What steps occur next for respiratory therapy of CF:

1) bronchodilators

2) hypertonic saline

Helps mobilize mucus

What steps occur next for respiratory therapy of CF:

1) bronchodilators

2) hypertonic saline...

3) dornase alfa

Breaks down mucus

What steps occur next for respiratory therapy of CF:

First step

1) bronchodilators

To open airways

What steps occur next for respiratory therapy of CF:

1) bronchodilators

2) hypertonic saline

3) dornase alpha...

4) chest percussion

moves mucus out

What steps occur next for respiratory therapy of CF:

1) bronchodilators

2) hypertonic saline

3) dornase alpha

4) chest percussion...

5) inhaled antibiotics

treats infection

Respiratory therapy function - bronchodilators

opens airways

Respiratory therapy function - hypertonic saline

helps mobilize mucus

Respiratory therapy function - dornase alpha

breaks down mucus

Respiratory therapy function - chest percussion

moves mucus out

Respiratory therapy function - inhaled antibiotics

treat infections in the lungs

Nutrition support: CF patients require significant __________ _________

- (significant) nutrition support

Pancreatic enzyme supplementation with meals

Nutrition support: _____________ enzyme supplementation with meals

- pancreatic (enzyme supplementation)

Insulin for CF associated DM

Nutrition support: ___________ for CF associated diabetes mellitus

- Insulin (for CF associated diabetes mellitus)

Nutrition support: insulin for CF associated _______________ ________

- (CF associated) diabetes mellitus

Vitamin supplements are also needed, specifically fat-soluble vitamins (A, D, E, K)

Nutrition support for CF: vitamin supplements are also needed, specifically _______-____________ ___________ (____________)

- fat-soluble vitamins (A, D, E, K)

Nutrition support for CF: increased rate of metabolism requires __________ ______ content for food

- higher fat (content for food)

Nutrition support for CF: _____ for GERD symptoms and for absorption of pancreatic enzymes

- PPI (for GERD symptoms)

Nutrition support for CF: PPI for _______ symptoms and for absorption of ______________ _______

- GERD (symptoms)

- (absorption of) pancreatic enzymes

Maintenance of BMI: improved nutritional status leads to _____________ health outcomes

- improved (health outcomes)

May require 110-200% of normal energy requirements

Maintenance of BMI: may require _______-_______% of normal energy requirements

- 110-200(% of normal energy requirements)

Maintenance of BMI - BMI goals

2 - 20 years: BMI > 50th percentile

> 20 years: BMI > 22 for females and > 23 for males

GERD: ____-____% of patients with cystic fibrosis may experience

- 40-90(% of patients with CF)

Possibly due to:

- decreased esophageal sphincter tone

- delayed gastric emptying

- increased intraabdominal pressure

GERD in CF patients are possibly due to...

decreased esophageal sphincter tone

delayed gastric emptying

increased intra-abdominal pressure

GERD may contribute to __________, _______ bronchospasm, and increased _________ ____________

- microaspiration

- reflex (bronchospasm)

- (increased) airway inflammation

GERD in CF: manage with ______ with __________ GERD treatment strategies

- PPI

- standard (GERD treatment strategies)

Pancreatic insufficiency: affects approximately ____% of CF patients

- 85(% of CF patients)

In CF patients, pancreatic insufficiency affects fat absorption and ability to break down food

In CF patients, ___________ ___________ affects fat absorption and ability to break down food

- pancreatic insufficiency (affects fat absorption)

In CF patients, pancreatic insuffiency affects ____ _________ and ability to _______ _______ food

- fat absorption

- break down (food)

Pancreatic insuffiency in CF patients, supplementation with ___________ _________ usually necesary

- pancreatic enzymes (usually necessary)

Pancreatic enzyme supplementation drugs

CREON

PANCREAZE

ULTRESSA

VIOKASE

ZENPEP

Pancreatic Enzyme Supplementation (CREON, PANCREAZE, ULTRESSA, VIOKASE, ZENPEP) contains ________, _________, and _________

- (contains) lipase

- protease

- amylase

Pancreatic Enzyme Supplementation dosing based on __________ units

- lipase (units)

2500 units / kg / meal

10,000 units / kg /day

Pancreatic Enzyme Supplementation formulated as _______-_______ and ________-coated

- delayed-release

- enteric(-coated)

Enzymes inactivated by acidic environment so may require H2RAs or PPIs

Pancreatic Enzyme Supplementation: enzymes inactivated by __________ environment thus may require ___________ or _______

- (inactivated by) acidic (environment)

- H2RAs

- PPIs

Pancreatic Enzyme Supplementation take _________ to snacks or meals

- prior (to snacks or meals)

Pancreatic Enzyme Supplementation: can be ________ and _________ in fluid or on soft foods but do NOT crush or chew

- opened

- sprinkled (in fluid or on soft foods)

Pancreatic Enzyme Supplementation: can be opened and sprinkled in fluid or on soft food but do ______ _______ nor _______

- NOT crush

- (nor) chew

Cystic fibrosis related diabetes mellitus: relative _________ ________ in CF patients due to destruction of islet cells

- insulin deficiency (in CF patients)

Cystic fibrosis related diabetes mellitus: relative insulin deficiency in CF patients due to destruction of _______ ______

- (destruction of) islet cells

Risk increases with age

Cystic fibrosis related diabetes mellitus: risk increases with _______

- (increases with) age

Requires insulin treatment

Cystic fibrosis related diabetes mellitus: requires _________ ___________

- (requires) insulin treatment

Dose adjustments and management highly dependent on patient nutritional intake and individual needs

Monitor closely to prevent hypoglycemia

Cystic fibrosis related diabetes mellitus: Dose adjustments and management highly dependent on patient _____________ _______ and individual _____

- (dependent on patient) nutritional intake

- (and individual) needs

Monitor closely to prevent hypoglycemia

Cystic fibrosis related diabetes mellitus: Monitor closely to __________ _____________

- (closely to) prevent hypoglycemia

Cystic fibrosis related diabetes mellitus: consider time to ______ absorption of insulin vs. _________ of food

- peak (absorption)

- absorption (of food)

Delayed gastric emptying and absorption of nutrients

Maintain bone density in CF: decreased absorption of ______________ ____

- (decreased absorption of) vitamin D

poor nutritional status

Maintain bone density in CF: CF patients have decreased bone density due to _______ nutritional status and _________ exercise

- poor (nutritional status)

- reduced (exercise)

Manage bone density with appropriate supplementation

Maintain bone density in CF: manage bone density with _____________ _______________

- appropriate supplementation

Encourage exercise / activity

Maintain bone density in CF: encourage _________ / __________

- (encourage) exercise / activity

Assess vitamin D and minerals frequently

Maintain bone density in CF: assess _________ ___ and _________ frequently

- Vitamin D

- minerals (frequently)

Maintain bone density in CF: __________ and _______ risk

- fracture

- fall (risk)

Infection in CF: chronic ______________ __________ in airways occur in most CF patients

- (chronic) bacterial infection

Infection in CF: ____________ bacterial infection in _______ occur in most CF patients

- chronic (bacterial infection)

- airways (occur)

Infection in CF: common pathogens

Pseudomonas aeruginosa

Staphylococcus aureus

Methicillin-resistant Staphylococcus aureus

Infection in CF: other pathogens in CF

aspergillus species

non-tuberculosis mycobacteria

Infection in CF is associated with increased _________ and __________

- (increased) morbidity

- mortality

Treated with ID specialist and CF team

Inhaled antibiotics: chronic infection with pathogens such as ______ ______________ can occur

- P. aeruginosa (can occur)

Inhaled antibiotics: inhaled use of antibiotics may be ____________

- (may be) warranted

Inhaled antibiotics

inhaled tobramycin (first-line)

aztreonam lysine (first-line)

colistin (second-line)

First-line inhaled antibiotics

inhaled tobramycin

aztreonam

Second-line inhaled antibiotics

colistin

Inhaled antibiotics: may consider ______________ regimens periodically

- alternating (regimens periodically)

Antibiotics: may consider _______ __________ for anti-inflammatory effects

- oral azithromycin (for anti-inflammatory effects)

Will not eradicate P. aeruginosa

Antibiotics: long-term use of other oral antibiotics (other than oral azithromycin) not routinely recommended due to risk of _________________

- (due to risk of) resistance