Therapeutics - Cystic Fibrosis in Children Flashcards by amanda kastel

in CF patients, many different organs are affected

however, which are the only one(s) that result in mortality of the patient

just the lungs

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2nd most affected organ in CF patients

pancreas

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name the problem in class 1-6 CF mutation

class 1 - no CFTR protein is synthesized

class 2 - most common. CFTR protein is synthesized, but it misfolds and thus cant move to the cell surface

class 3 - CFTR protein is synthesized and moves to surface BUT channel gate doesn’t open properly

4 - function of channel is faulty

5 - insufficient quantities. but it’s synthesized and gets to the surface and everything

6 - reaches membrane and everything but has a fast turnover

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4 nonpharm therapies to help with the pulmonary system in CF patients

exercise
chest physiotherapy and drainage
chest/vest therapy
nutrition!

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what is the pharmacotherapy to help the pancreas in CF patients

pancreatic enzymes - to help them digest fat

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during pulmonary EXACERBATIONS in CF patients, what pharmacotherapy is given

systemic antibiotics

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4 potential maintenance therapy to help the pulmonary system in CF patients

INHALED antibiotics
dornase alpha
mucolytics/bronchodilators
corticosteroids

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what class is the disease modulators in CF patients

CFTR modulators

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why is nutrition so important in CF patients

important for lung function. proper nutrition really does increase the life expectancy of a CF patient.

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specific nutrition recommendations for CF patients

give calories and protein at 120-200% (3000-4000 cals)

give a high FAT and high PROTEIN diet

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role of appetitie stimulants in CF pts

name 2

can help them to eat and get proper nutrition - lot of them dont rly have appetite

cyproheptadine
dronabinol

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vitamin recommendations for CF patients

give 2 multivitamins/day

CF patients have issues absorbing fat-soluble things. therefore, give WATER SOLUBLE FORMS of vitamins A D E K

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can a CF patient ever be given the FAT SOLUBLE FORMS of vitamins A D E and K

yes - but only if we increase dose

this may have to be done if the pt cant afford the water soluble forms (more $$$$)

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what is meconium ileus

obstruction in the bb passing 1st stool. if this happens - test for CF

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treatment for meconium ileus

hyperosmolar enemas (gastrograffin or acetylcysteine)

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treatment of distal intestinal obstruction syndrome (after newborn) in CF patients

oral rehydration and osmotic laxatives (PEG, oral gastrogreffin or acetylcysteine)

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true or false

MANY CF patients have an issue with not releasing pancreatic enzymes

true - 85%

due to very thick secretions

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creon is a pancreatic enzyme preparation.

explain a VERY IMPORTANT COUNSELING POINT for all pancreatic enzyme preparations

MUST TAKE WITH A MEAL

prone to breakdown by gastric acid

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can a patient chew or open pancreatic enzyme preparations

DO NOT CHEW - ulceration

however, can be opened onto applesauce or jelly

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true or false

it is necessary to stay on the same brand of pancreatic enzymes

true - ensures the same efficacy

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how are pancreatic enzyme preparations available and why? how are they dosed?

microencapsulated tabs/capsules to avoid breakdown

dosed according to lipase (fat) content

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true or false

pancreatic enzymes can be taken with OJ

FALSE - cannot. the acidity destroys the enzyme

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the starting doses of the pancreatic enzymes are according to lipase content, then are titrated according to….

effect

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when the dose of pancreatic enzymes is over _____ - what to do?

what about over 6000u/k/meal lipase

over 2500 - investigate issue

over 6000 - figure out what’s wrong. colonic structure issues, etc

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true or false if the patient is having NON FATTY FOODS, they still need to take the pancreatic enyzmes

FALSE - may not need it. may just be able to eat the non fat food

true or false the dissolution/absorption of pancreatic enzymes is better in ALKALINE PH

true dont drink acidic juice!

the substitution of pancreatic enzyme preparations is only appropriate between___ and ____

creon and pancreaze

if a patient is still having diarrhea after taking a 2500u/kg/meal of pancreatic enzymes, what may be needed?

may need an adjuncitve H2 antagonist or PPI to increase absorption

****how to monitor the efficacy of pancreatic enyzmes

WATCH THE HEIGHT AND WEIGHT!! also # of stools

issue with some people not adhering to creon

some pts may not want to take bc it will make them gain weight

2 toxicity concerns of pancreatic enzymes

mouth ulcerations and perianal irritation

additional immunizations for CF patients

PPSV23 (pneumococcal polysaccharide) COVID vaccination advisable

true or false the pulmonary system is the most common organ affected in CF patients

true

pulmonary complications in CF patients

accumulation of mucus and impaired host defense airway gets plugged with mucus - prone to bacterial infection and inflammation

how to tell when a CF patient is having a pulmonary exacerbation

the sputum will be thicker than normal - it's something that the patient will obviously notice also hypoxia, dyspnea on exertion, reduced resp rate (sob)

postural drainage is typically done how often? what about during a pulmonary exacerbation?

typically - 1-2x a day during exacerbation - 5-6x a day may inhale normal saline beforehand!

true or false B2 agonists are always indicated for CF patients in order to enhance airway clearance

false - ONLY indicated if the patient has asthma

name 3 mucolytics for CF

dornase alpha hypertonic saline manntol (not rly used)

can both dornase alpha AND hypertonic saline be used to enhance airway clearance?

YES work by diff mechanisms dornase degrades DNA from dead and dying neutrophils and hypertonic saline draws water out of the airway by making an osmotic gradients

where is dornase alpha stored counseling points?

fridge takes 2-3 weeks to start working, may take up to 3 months AE is voice alterations, rash, conjunctivitis also, a specific nebulizer is needed to use it (jet or vibrating mesh nebulizer)

hypertonic saline is a....

mucolytic

how is hypertonic saline helpful in CF

improves lung function and reduces pulmonary exacerbations doesnt just improve symptoms - actually helps!

name some AE of hypertonic saline and how these might be avoided

irritation, wheeze, bronchospasm may use a bronchodilator beforehand to limit these effects (or decrease dose)

true or false for CF patients, dornase alpha and hypertonic saline can either be used together or separate

true

what CF patients are eligible for inhaled antibiotics as maintenance therapy?

mod-severe lung disease (FEV1 25-75%) AND have bacteria in the lungs

if the patient has pseudomonas in the lungs, what inhaled maintenance antibiotic is given?

pseudomonas - TOBI/azithromycin/aztreonam

if a CF patient is getting inhaled tobramycin for p. aeuroginosa colonization, explain how it is given

nebulized BID 28 days on and 28 days off

if pt is getting inhaled meds + an inhaled antibiotic, what order are they given

given inhaled antibiotic LAST to make sure it really enters the lungs

AE of tobi

voice alterations and irritation

alternative to TOBI

aztreonam also given 28 days on and off

can inhaled aztreonam be combined with TOBI?

yes, or used in "off months"

role of azithromycin in CF patients

has immunomodulatory and anti inflammatory effects!!!! reduces biofilm of pseudomonas

indication for azithromycin in CF

chronic P. aeruginosa infection (better evidence than non pseudomonas)

before a CF patient is started on azithromycin, what should they be screened for

screen for non tuberculosis mycobacterium before starting AND at 6-12 month intervals bc macrolides block the killing of mycobacteria in macrophages - will cause a chronic non-tb mycobacterium infection

****how often is azithromycin administered for CF patients as maintenance therapy

3 DAYS A WEEK

role of ibuprofen in CF maintenance

has role as an anti inflammatory not used too often recommended for FEV1 60% or less. slows progression of lung disease in kids

true or false we want to stop CF patients from coughing

FALSE - we want them to cough

differentiate between the type of antibiotic used for maintenance vs exacerbation in CF patients

maintenance - inhaled exacerbation - systemic DO NOT GIVE BOTH AT THE SAME TIME

what is the goal in treating pulmonary exacarbations

treat the infection and improve oxygenation

concern with biofilm in CF patients

the antibiotics wont be able to get thru

3 most common bacteria in CF patients in lungs

early in life - staph h. influenzae later on - p. aeruginosa

general approach when treating pulmonary exacarbations with antibiotics

follow susceptibilities but start broad typicall will use more than 1 antibiotic follow clinical improvement

**LENGTH OF THERAPY for antibiotics in pulmonary exacarbations

10-14 days!!!!! (up to 4 weeks (rare)) according to CLINICAL IMPROVEMENT - not bacteriologic

**2 pharmacokinetic alterations in CF patients and what this means for dosing and interval of the antibiotics we give

increased Vd and enhanced clearance need to increase the dose and give at a shorter interva;

in general what do the CFTR modulators do in CF patients

correct the malfunctioning protein and improve respiratory function

Therapeutics - Cystic Fibrosis in Children Flashcards

(66 cards)