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CF
An inherited, autosomal recessive genetic disorder that causes altered transport of sodium and chloride ions in and out of epithelial cells and airway obstruction due to changes in exocrine glandular secretions, resulting in increased mucus production
lungs, pancreas, biliary tract, reproductive organs
what does CF primarily affecet
6-8 months old
what is the median age of diagnosis for CF
Decreased pancreatic functioning causes decreased absorption of nutrients leading to steatorrhea (large fatty BMs)
Pancreatic issues can result in DM
Presence of meconium ileus in newborns
Persistent respiratory symptoms
Failure to thrive
Malnutrition
Bronchiectasis
Thick, sticky mucus
Barrel chest
Decreased absorption of vitamins and ezymes
Abdominal distension
Rectal prolapse
Adult S/S may include new onset DM, infertility issues, frequent cough, URI, clubbing, weight loss, increased cough, decreased lung function
what are the clinical manifestations of CF
Sweat Chloride Test
The gold standard test for CF in which sweat is collected and analyzed; values >60 mmol/L are positive for CF
relieving airway obstruction
loosen mucus with CPT, percussion, vibrations
controlling infection
use aerosol and nebulizer to promote drainage
Antibiotics to prevent complications from lung infections
adequate home support and resources such as IV antibiotic therapy at home, independently
use oral agents for mild exacerbations
pancreatic insufficiency management
aggressive pulmonary training (chest PT, IS, pursed lip breathing, breathing exercises, O2) to maintain airway clearance
what does interprofessional care for CF include
hemoptysis (blood sputum), respiratory failure
why are CF patients admitted to the ICU (SATA)
a. hemoptysis
b. respiratory failure
c. decreased oxygenation
d. clogged bile ducts
e. delayed growth
asthma
bronchial hyperreactivity with reversible expiratory airflow limitations (spontaneously or with treatment) that effects millions of adults each year and causes millions of ED visits
inflammatory factors
respiratory infections
allergens
work
irritants
temperature changes
exercise
cold air
stress and emotions
strong odors
medications
tobacco
gastric reflux
air pollutants
food additives
what are different asthma triggers
asthma
exposure to triggers triggers inflammatory mediators such as edema of airway mucosa, muscle spasm, accumulation of secretions leading to vasodilation of BVs, itching, bronchospasms, and airway narrowing, and mucus production leading to problems on expiration
labored breathing, cough, wheezing, tightness in chest, troubles with airflow, sleep problems, feeling tired, allergies, common cold
what are the clinical manifestations of asthma
mild asthma
what severity of asthma:
dyspnea occurs with activity and patient may feel that he or she “can’t get enough air”
Usually treated at home with an inhaler or a short course of oral corticosteroids
moderate asthma
what severity of asthma:
Dyspnea interferes with or limits usual activities
requires office/ED visit
Frequent inhale SABA and oral systemic corticosteroids
severe asthma
what severity of asthma:
dyspnea at rest, speaks in words not sentences because of difficulty breathing, feeling of suffocation
requires ED visit and usually hospitalization, partial relief from SABA, oral systemic corticosteroids in combination with IV magnesium and ipratropium
life threatening asthma
what severity of asthma:
too dyspneic to speak, may be drowsy/confused, perspiring
requires ED/hospitalization and maybe ICU, minimal/no relief from SABA, IV corticosteroids with ipratropium, and IV mag
airways remodeling, respiratory related issues, fatigue, HA, lack of activity, pneumonia, influenza, acute renal failure, status asthmaticus
what are the complications of athma
Peak expiratory flow rate (PEFR)
Incentive spirometry to see lung volumes and capacities
Fraction of exhaled Nitric Oxide (FENO)
Serum eosinophils and IgE can suggest allergens
Chest X-ray and S&C to rule out other disorders
what are the diagnostic studies used to diagnose asthma
peak flow expiratory rate (PFER)
A test of lung function that measures the maximum rate of airflow after forceful exhalation, which helps predict asthma attacks and monitor the severity of the disease
fraction of exhaled nitric oxide (FENO)
diagnostic study that helps to determine asthma and can gauge loss of asthma control and attacks and patient adherence to therapy
if symptoms worsen step up medications, if symptoms are controlled step down medications
what is the stepwise approach/medication guidlnes based on steps for asthma
step 1 medications for asthma
which step of asthma treatment:
no preferred controller med
consider low dose ICS
Use SABA PRN for relief
step 2 medications for asthma
which step of asthma treatment:
preferred controller med: low dose ICS
alternative: LTRA, low dose theophyine
SABA PRN for relief
step 3 medications for asthma
which step of asthma treatment:
preferred controller med: low dose ICS/LABA
alternative: med/high dose ICS, or low dose ICS + LTRA or theophylline
SABA or low-dose ICS/formoterol PRN for relief
step 4 medications for asthma
which step of asthma treatment:
preferred controller med: med/high ICS/LABA
alternative: add tiotropium + med/high dose ICS + LTRA or theophyline
SABA or low dose ICS/formoterol PRN for relief
step 5 medications for asthma
which step of asthma treatment:
preferred controller med: hgih ICS/MBA + add on (tiotropium, anti-IgI, anti-IL5, etc.)
alternative: add low dose OCS
SABA or low-dose ICS/formoterol PRN for relief
teach patient to identify/avoid known triggers and irritants
wash clothes in hot or cold water with detergent and bleach
use scarves/masks for cold air
avoid aspirin and NSAIDs
consider desensitization therapy
promptly report URIs and sinusitis
evaluate job environment
weight loss/exercise
what are the nursing interventions for asthma
COPD
a progressive lung disease with persistent airflow limitation with an enhanced chronic inflammatory response in airway and lungs that is characterized by chronic inflammation of airways, lung parenchyma, and pulmonary BVs
cigarette smoking and noxious particles/gases
what are the main causes of COPD
cigarette smoking, infection, asthma, air pollution, occupational chemicals/dusts, aging, genetics
what are the RF of COPD
COPD
Airflow limitation is not fully reversible during forced exhalation due to loss of elastic recoil of the lungs and airflow obstruction from mucous hypersecretion, mucosal edema, and bronchospasm, Disease progression is marked by worsening abnormalities in airflow limitation, air trapping, and gas exchange which when severe can cause pulmonary HTN and systemic manifestations
hypoxemia (low O2) and hypercapnia (high CO2)
how does COPD affecet O2 and CO2 levelshy
hypoxemia
PaO2 < 60 mmHg and SaO2 < 88% on RA
hypercapnia
PaCO2 > 45 mmHg
decreased elastic recoil and decreased chest wall compliance
decreased functional alveoli and SA for gas exchange
PaO2 decreased with age (normal for an older adult is 70 mmHg)
Respiratory defense mechanisms are less effective
increased burden of disease due to reduced lean body mass, decreased respiratory muscle strength, increased dyspnea, and lower exercise intolerane
smoking cessation may be hard
handling/coughing out secretions may be more difficult
how does COPD affect the aging adult
chronic cough, dyspnea that occurs with exertion and becomes progressive, wheezing/chest tightness, chest heaviness (can’t take a deep breath, gasping, increased effort to breathe, air hunger), digital clubbing, use of accessory muscles. muscles to breath, easily fatigued, barrel chest, tripod position, pursed-lip breathing, peripheral edema (ankles) → right HF, decreased breath sounds, weight loss/anorexia, pulmonary HTN, Cor Pulmonale, Acute exacerbations, acute renal failure (ARF)
what are the clinical manifestations of COPD
pulmonary HTN, cor pulmonale, acute exacerbations, ARF
what are the primary complications of COPD
spirometry to confirm diagnosis if FEV1/FVC ration is < 70%
The lower the FEV1 the more severe the COPD
chest X-ray
serum a1-antitrypsin levels
6-minute walk test to test for exercise-induced hypoxemia
Qualify for O2 therapy if Pulse Ox at rest is < 88%
ABGs
ECG: normal or signs of right HF
Echo, MUGA scan to evaluate heart function
COPD assessment test/ questionnaire
what are teh diagnostic studies for diagnosis COPD
< 88% at rest
what does a patient’s pulse ox have to be to qualify for O2 therapy
oxygen therapy
what is the only treatment linked to improved survival for COPD patients
keep SaO2 > 90% during rest, sleep, and exertion, keep PaO2 > 60 mmHg, or levels as appropriate in moderate-severe COPD, too much O2 can decrease the drive to breathe so MUST be carefully monitored
what is the goal of oxygen therapy in COPD patients
pursed-lip breathing
breathing retraining exercise that prolongs expiration to reduce bronchial collapse and air trapping
diaphragmatic breathing
breathing retraining exercise that promotes the use of diaphragm instead of accessory muscles to achieve max inhalation and slow respiratory rate, in some patients may increase WOB and dyspnea
ACTs
airway clearance techniques that is often used with other treatments (bronchodilator) to loosen mucus to clear with coughing; ie. effective/huff coughing
effective/Huff coughing
airway clearance technique that conserves energy, reduces fatigue, and facilitates the removal of secretions
nothing specific, but a well-balanced diet, high in protein, moderate in carbohydrate, moderate to high in fat, 5-6 small meals per day, nutrition supplements between meals, dietician consult, increased protein and caloric needs
What is nutrition therapy for COPD
quit smoking, use good hand hygiene, take drugs as prescribed, early treatment of respiratory tract infections/exacerbation, vaccinations, avoid/control exposure to pollutants and irritants
what does health promotion look like for a COPD
Exercise training leads to energy to energy conservation
modify ADLs to conserve energy
schedule daily/weekly activities, including plenty of rest periods
regular exercise that is appropriate, safe, and easy to perform
what are activity considerations for those with COPD