Pulmonary Disease - BPD and CF Flashcards
What is Obstructive Pulmonary Disease
and what could it ultimately affect
Respiratory tract diseases that produce an
obstruction
to air flow
which can ultimately affect the mechanical function and gas exchange of the lungs
Obstructive Pulmonary Disease risk factors (6)
Age: (Prematurity, Elderly) Immunocompromised Environmental Factors Smoking Genetics Restricted Thoracic Movement
The effects of smoking on the lungs
Produces a low level chronic inflammation in the lungs
Increased phagocytes, neutrophils, and alveolar macrophages all contribute to the breakdown of elastin in the alveoli
Destroys the action of cilia
What is Hypoxia
Hypoxia is inadequate oxygenation
Hypoxia can result from
Pulmonary Disease with impaired diffusion
V-Q mismatching: shunting
Inadequate O2 transport
Inadequate tissue oxygenation
Some causes of hypoventilation (5)
Respiratory center depression
Neural conduction interference
Respiratory muscle disease
Restriction of the thorax
Restriction of lung excursion
Some causes of Hyperventilation (8)
Anxiety
Lesion of CNS
Medications/ Hormones
Increased Metabolism
Hypoxia/ CO2 retention (short term)
Acidosis
Hypotension - drop in BP
Pain
BPD stands for
Bronchopulmonary Dysplasia
What is Bronchopulmonary Dysplasia
Chronic lung disease of early infancy
Presentation:
O2 dependency beyond 1 mos., and vent support
Infants at risk for BPD include those with:
Bronchopulmonary Dysplasia
Prematurity
Meconium aspiration
Persistent fetal circulation (PDA and PFO)
BRONCHOPULMONARY DYSPLASIA occurs in some respiratory distress syndrome infants who require (aka)
PROLONGED VENTILATION
AKA Pulmonary Fibroplasia and ventilator lung
BRONCHOPULMONARY DYSPLASIA occurs more in babies who are (2)
LBW (low birth weight)
and who rec’d high flow O2 for 5 d or longer
Effects of BPD on babies
Bronchopulmonary Dysplasia
Many infants recover and improve over time however severe BPD correlates highly with developmental delays
4 stages of BPD pathology
Stage 1: Hyaline membrane disease caused by decreased surfactant (will not have all your surfactant until 32 weeks)
Stage 2: Tissue granulation (breakdown of tissue), obliterating bronchitis
Stage 3: Emphysematous changes (distended terminal airways and open alveoli ->hyperinflation)
Stage 4: Subepithelial fibrosis (scarring), right ventricular hypertrophy (corpulmonaly)
BPD can cause injury by
Barotrauma (injury caused by pressure)
O2 Toxicity (cellular tissue damage occurs from excessive oxygen levels in the blood)
Pulmonary interstitial edema
BPD: Clinical Features
how will infants present?
Infants will present with:
Tachypnea
Cyanosis
Bronchiolitis or Pneumonia
May require a tracheostomy
Why is it important to know if a person has BPD in their medical history
If in persons history - might have decreased reserves and dec. endurance and also correlated with delayed developmental milestones
How does someone get CF
Autosomal recessive trait
Abnormal Gene- affects the transmembrane conductance regulator (CFTR) protein
Normal Pancreatic Function
Pancreas secretes enzymes which aid in the digestion of fats and proteins
Normal mucous bathes and protects internal surfaces, transports chemicals between organs through small ducts
Flows easily, carrying bacteria, dirt and waste
How does CF affect pancreatic function
changes cell permeability to salt causing an electrolyte abnormality
Which tissues have CFTR and what does that mean for CF
CFTR is present in the respiratory tissue, GI tracts, as well as the sweat glands, and reproductive system
CF affects more than the lungs
Which organs most affected with CF
lungs pancreas reproductive system sinuses sweat glands
Accumulation of hyper viscous secretions in pts with CF leads to
progressive obstruction of mucous secreting exocrine glands, preventing delivery to target organs
Cystic Fibrosis Impairments
1) Dysfunctional Cl channel epithelial function causes abnormal concentrations of Na and Cl
2) Blocked exocrine gland function
3) Increased susceptibility to chronic endobronchial colonization
What is endobronchial colonization
infections in the lungs over and over again, pneumonia is a destructive process leads to decrease in function
Pancreatic Dysfunction
and CF
Viscous secretions begin to obstruct pancreas in utero with periductal inflammation and fibrosis
Maldigestion of protein = greasy, bulky, foul
smelling stools
*stool is a confirmatory test
Pulmonary Dysfunction
and CF
Obstruction of small airways
Air trapping and atelectasis
Progressive airway obstruction
Decreased gas exchange
Opportunistic bacteria, inflammation, bronchiectasis, and irreversible airway damage
Patient presentation with CF
Patients are likely to present with: Persistent Cough Recurrent Pneumonia Excessive Appetite Poor Weight Gain Salty Skin/Sweat Bulky Foul Smelling Stools Infertility Nasal Polyps Chronic Wheeze Glucose Intolerance Tachypnea Cyanosis/Digital Clubbing Exertional Dyspnea Pneumothorax Hemoptysis Right Sided Heart Failure (lungs do not work)
Why does a person with CF have poor weight gain
lack of digestion
Associated Medical Complications with CF
↑ Incidence of DM, Intestinal Obstruction, Hepatobiliary involvement, Recurrent Pancreatitis
Obstruction of the vas deferens
(98% infertility in males, ↓ fertility in females)
Sinusitis (Persistent HA)
Pneumothorax 2º rupture of bronchiectatic cysts or subpleural blebs, Clubbing, Anorexia, Malnutrition
*Medical Tests for Diagnosis of CF
Sweat Test
Nasal PD (Potential difference)
Stool Analysis
Can CXR diagnose CF
Findings are negative early on in the disease process
Later, X-rays demonstrate streaky, white bands across the lung fields
Pneumonia, bronchiectasis, atelectasis, pneumothorax
What will a CXR of later stages of CF look like
Streaky
White bands
What problems would a PFT show for a person with CF
Obstructive Components: -Air trapping
Increased RV and FRC
Increased PaCO2
Restrictive Components: Restricted air flow
Decreased TLC and VC
CF chest pt assessment will show
Auscultation - Rales/sonorous wheezes
Increased A/P diameter (Barrel Chest)
Chronic, productive cough
Accessory muscle use
CF chest PT treatment
Postural Drainage (no trendelenburg)
FET (forced expiration technique) (huffing) as effective as 2-3 CPT treatments/day
ACB (Active cycle breathing) uses FET in conjunction with thoracic exp. exercises
AD (Autogenic draining)(uses segmental breathing)
Trunk Mobility/Postural Education
Exercise encouragement
Aduct tools to increase exercise tolerance and secretion clearance in pts with CF
IPV Intrapulmonary Percussive Ventilator
machine that delivers small bursts of air to help clear mucus
PEP (positive expiratory pressure)/flutter
Aerobika- oscillating positive expiratory pressure device
Inflatable therapy vest
Mucolytics (thins secretions)
Any PT program for CF should advocate regular exercise, the benefits of which include:
👍 ↑ sputum clearance
👍 ↑ aerobic fitness
👍 psychological benefits
CF Nutrition
Balanced diet - high calorie
Enzyme supplementation for fat and protein digestion
May require supplemental nutrition at night by NG or IV (hydration essential for adequate expectoration of secretions)
CF Pharmacology
Abx - for recurrent infections
Nebulizers
Mucus thinners
CFTR Modulation Therapies
Steroids to dec. inflammation
Lung transplant and CF
Must be done bilaterally
Severe pulmonary disease with marked hypoxemia
Increase in the frequency of hospitalizations
Increased antibiotic resistance
No other significant vital organ disease
History of medical compliance
Acceptable psychological profile
Complications and things to keep in mind with lung transplants
Complicated by fact many recipients are adolescents
May tend to skip follow up rx in order to fit in better with peers
cystic fibrosis is still all in the body even though lungs do not have gene
Lung Transplant P/O
Immunological suppression post op
Risk of rejection
Pts may be colonized with infectious agents (esp in sinuses) that can infect new lungs
Unique challenges (only organ transplant that comes into contact with the outside world)
