Pulmonary Disease - BPD and CF

Question 1

What is Obstructive Pulmonary Disease

and what could it ultimately affect

Answer 1

Respiratory tract diseases that produce an
obstruction
to air flow

which can ultimately affect the mechanical function and gas exchange of the lungs

Question 2

Obstructive Pulmonary Disease risk factors (6)

Answer 2

Age: (Prematurity, Elderly)
Immunocompromised
Environmental Factors
Smoking
Genetics
Restricted Thoracic Movement

Question 3

The effects of smoking on the lungs

Answer 3

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

Question 4

What is Hypoxia

Answer 4

Hypoxia is inadequate oxygenation

Question 5

Hypoxia can result from

Answer 5

Pulmonary Disease with impaired diffusion
V-Q mismatching: shunting
Inadequate O2 transport
Inadequate tissue oxygenation

Question 6

Some causes of hypoventilation (5)

Answer 6

Respiratory center depression

Neural conduction interference

Respiratory muscle disease

Restriction of the thorax

Restriction of lung excursion

Question 7

Some causes of Hyperventilation (8)

Answer 7

Anxiety

Lesion of CNS

Medications/ Hormones

Increased Metabolism

Hypoxia/ CO2 retention (short term)

Acidosis

Hypotension - drop in BP

Pain

Question 8

BPD stands for

Answer 8

Bronchopulmonary Dysplasia

Question 9

What is Bronchopulmonary Dysplasia

Answer 9

Chronic lung disease of early infancy

Presentation:
O2 dependency beyond 1 mos., and vent support

Question 10

Infants at risk for BPD include those with:

Answer 10

Bronchopulmonary Dysplasia

Prematurity
Meconium aspiration
Persistent fetal circulation (PDA and PFO)

Question 11

BRONCHOPULMONARY DYSPLASIA occurs in some respiratory distress syndrome infants who require (aka)

Answer 11

PROLONGED VENTILATION

AKA Pulmonary Fibroplasia and ventilator lung

Question 12

BRONCHOPULMONARY DYSPLASIA occurs more in babies who are (2)

Answer 12

LBW (low birth weight)

and who rec’d high flow O2 for 5 d or longer

Question 13

Effects of BPD on babies

Answer 13

Bronchopulmonary Dysplasia

Many infants recover and improve over time however severe BPD correlates highly with developmental delays

Question 14

4 stages of BPD pathology

Answer 14

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)

Question 15

BPD can cause injury by

Answer 15

Barotrauma (injury caused by pressure)

O2 Toxicity (cellular tissue damage occurs from excessive oxygen levels in the blood)

Pulmonary interstitial edema

Question 16

BPD: Clinical Features

how will infants present?

Answer 16

Infants will present with:
Tachypnea
Cyanosis
Bronchiolitis or Pneumonia

May require a tracheostomy

Question 17

Why is it important to know if a person has BPD in their medical history

Answer 17

If in persons history - might have decreased reserves and dec. endurance and also correlated with delayed developmental milestones

Question 18

How does someone get CF

Answer 18

Autosomal recessive trait

Abnormal Gene- affects the transmembrane conductance regulator (CFTR) protein

Question 19

Normal Pancreatic Function

Answer 19

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

Question 20

How does CF affect pancreatic function

Answer 20

changes cell permeability to salt causing an electrolyte abnormality

Question 21

Which tissues have CFTR and what does that mean for CF

Answer 21

CFTR is present in the respiratory tissue, GI tracts, as well as the sweat glands, and reproductive system

CF affects more than the lungs

Question 22

Which organs most affected with CF

Answer 22

lungs
pancreas
reproductive system
sinuses
sweat  glands

Question 23

Accumulation of hyper viscous secretions in pts with CF leads to

Answer 23

progressive obstruction of mucous secreting exocrine glands, preventing delivery to target organs

Question 24

Cystic Fibrosis Impairments

Answer 24

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

Question 25

What is endobronchial colonization

Answer 25

infections in the lungs over and over again, pneumonia is a destructive process leads to decrease in function

Question 26

Pancreatic Dysfunction | and CF

Answer 26

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

Question 27

Pulmonary Dysfunction | and CF

Answer 27

Obstruction of small airways Air trapping and atelectasis Progressive airway obstruction Decreased gas exchange Opportunistic bacteria, inflammation, bronchiectasis, and irreversible airway damage

Question 28

Patient presentation with CF

Answer 28

``` 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) ```

Question 29

Why does a person with CF have poor weight gain

Answer 29

lack of digestion

Question 30

Associated Medical Complications with CF

Answer 30

↑ 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

Question 31

*Medical Tests for Diagnosis of CF

Answer 31

Sweat Test Nasal PD (Potential difference) Stool Analysis

Question 32

Can CXR diagnose CF

Answer 32

Findings are negative early on in the disease process Later, X-rays demonstrate streaky, white bands across the lung fields Pneumonia, bronchiectasis, atelectasis, pneumothorax

Question 33

What will a CXR of later stages of CF look like

Answer 33

Streaky | White bands

Question 34

What problems would a PFT show for a person with CF

Answer 34

Obstructive Components: -Air trapping Increased RV and FRC Increased PaCO2 Restrictive Components: Restricted air flow Decreased TLC and VC

Question 35

CF chest pt assessment will show

Answer 35

Auscultation - Rales/sonorous wheezes Increased A/P diameter (Barrel Chest) Chronic, productive cough Accessory muscle use

Question 36

CF chest PT treatment

Answer 36

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

Question 37

Aduct tools to increase exercise tolerance and secretion clearance in pts with CF

Answer 37

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)

Question 38

Any PT program for CF should advocate regular exercise, the benefits of which include:

Answer 38

👍 ↑ sputum clearance 👍 ↑ aerobic fitness 👍 psychological benefits

Question 39

CF Nutrition

Answer 39

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)

Question 40

CF Pharmacology

Answer 40

Abx - for recurrent infections Nebulizers Mucus thinners CFTR Modulation Therapies Steroids to dec. inflammation

Question 41

Lung transplant and CF

Answer 41

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

Question 42

Complications and things to keep in mind with lung transplants

Answer 42

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

Question 43

Lung Transplant P/O

Answer 43

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)