PULMONARY DISEASE MANAGEMENT

CHAPTER TITLE
CHARISA ANTONETTE S. HUELVA
September 17, 2021

I. ANATOMIC ALTERATIONS OF THE LUNGS B. ETIOLOGY AND EPIDEMIOLOGY

• Bronchiectasis is characterized by chronic dilation and • Bronchial obstruction and infection
distortion of one or more bronchi as a result of extensive o Most causes of bronchiectasis
inflammation and destruction of the bronchial wall cartilage, • Cystic fibrosis
blood vessels, elastic tissue, and smooth muscle o Most common cause of bronchiectasis in developed
components. countries
• One or both lungs may be involved. ▪ Prevalence of NCFB in developed countries is
• Commonly limited to a lobe or segment and is frequently relatively low.
found in the lower lobes ▪ Low incidence of NCFB in developed countries is
o The smaller bronchi, with less supporting cartilage, are most often attributed to early medical management
predominantly affected (e.g., antibiotic therapy)
• Normal mucociliary clearance is impaired because of • Pulmonary infection (for noncystic fibrosis bronchiectasis)
bronchial wall destruction. o Most common cause of noncystic fibrosis
o Results in the accumulation of copious amounts of bronchiectasis
bronchial secretions and blood that often become foul- o A possible mechanism for post infectious NCFB is a
smelling because of secondary colonization with significant lung infection during early childhood which
anaerobic organisms causes anatomic alterations of the developing lung that
• Thoracic infection and irritation may lead to secondary allows persistent bacterial infections
bronchial smooth muscle constriction and fibrosis. o Continuous bacterial infections lead to bronchiectasis
• The small bronchi and bronchioles distal to the affected • Primary ciliary dyskinesia
areas become partially or totally obstructed with secretions. o Individuals with a mucociliary disorder or an
• This condition leads to one or both of the following anatomic immunodeficiency disorder involving low levels of
alterations: immunoglobulin G (IgG), IgM, and IgA.
o Hyperinflation of the distal alveoli as a result of • Inflammatory disorders
expiratory check-valve obstruction o Inflammatory bowel disease, most often in those with
o Atelectasis, consolidation, and fibrosis as a result of chronic ulcerative colitis
complete bronchial obstruction • COPD
• Other Etiologic Factors:
Reid classification subdivides bronchiectasis into the following three o Foreign-body aspirations
different patterns: o Tumors
• Varicose Bronchiectasis (Fusiform Bronchiectasis) o Hilar adenonapthy
o Bronchi are dilated and constricted in an irregular o Bronchial airway mucoid impaction
fashion similar to varicose veins o Tracheobronchial abnormalities
o Results in a distorted, bulbous shape o Vascular abnormalities
• Cylindrical Bronchiectasis (Tubular Bronchiectasis) o Lymphatic abnormalities
o Bronchi are dilated and rigid and have regular outlines o Advanced age
similar to a tube o Malnutrition
o Dilated bronchi fail to taper for 6 to 10 generations and o Socio-economic disadvantage
then appear to end abruptly because of mucous o Alpha1-antitrypsin deficiency
obstruction • Causes of bronchiectasis are classified into:
• Cystic Bronchiectasis (Saccular Bronchiectasis) o Acquired bronchial obstruction
o Bronchi progressively increase in diameter o Congenital anatomic defects
o End in large, cystlike sacs in the lung parenchyma o Immunodeficiency defects
o Causes the greatest damage to the tracheobronchial o Abnormal secretion clearance
tree o Miscellaneous disorders (e.g., alpha1-antitrypsin
▪ The bronchial walls become composed of fibrous deficiency)
tissue alone – cartilage, elastic tissue, and smooth Table 14-1. Causes of Bronchiectasis.
muscle are all absent Category Specific Examples Diagnostic Tests
Acquired Bronchial Obstruction
A. MAJOR PATHOLOGIC OR STRUCTURAL CHANGES Foreign-body Peanuts; chicken bone; Chest imaging;
• Chronic dilation and distortion of bronchial airways aspiration teeth fiberoptic
• Excessive production of often foul-smelling sputum bronchoscopy
• Bronchospasm Tumors Laryngeal papillomatosis; Chest imaging;
airway adenoma; fiberoptic
• Hyperinflation of alveoli (air trapping)
endobronchial teratoma bronchoscopy
• Atelectasis (Figure 14-1, E)
Hilar adenopathy Tuberculosis; PPD; chest
• Consolidation and parenchymal fibrosis
histoplasmosis; imaging; fiberoptic
• Hemoptysis secondary to bronchial arterial erosion sarcoidosis bronchoscopy
COPD Chronic bronchitis Pulmonary
function tests
Rheumatic disease Relapsing polychondritis Clinical syndrome
(RP); tracheobronchial of RP/cartilage
amyloidosis biopsy; biopsy for
amyloid

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Mucoid impaction Allergic Total and Young’s syndrome Obstructive azoospermia Sperm count
bronchopulmonary aspergillus with sinopulmonary
aspergillosis; specific IgE; infections
bronchocentric specific Miscellaneous Disorders
granulomatosis (BG); aspergillus IgG; Alpha1-antitrypsin Absent or abnormal Alpha1-antitrypsin
postoperative mucoid aspergillus skin deficiency antitrypsin synthesis and level
impaction test; chest function
imaging; biopsy Recurrent Alcoholism; neurologic History; chest
for BG aspiration disorders; lipoid imaging
Foreign-body Peanut; chicken bone; Chest imaging; pneumonia pneumonia
aspiration tooth fiberoptic Rheumatic disease Associated with Rheumatoid
bronchoscopy rheumatoid arthritis and factor;
Congenital Anatomic Defects That May Cause Bronchial Sjorgen’s syndrome antiSSA/antiSSB;
Obstruction salivary gland
Tracheobronchial Bronchomalacia; Chest CT imaging MRI or biopsy
abnormalities bronchial cyst; cartilage Inflammatory bowel Crohn’s disease; History; lower
deficiency (Williams- disease ulcerative colitis gastrointestinal
Campbell syndrome); endoscopy;
tracheobronchomegaly imaging studies;
(Mounier-Kuhn colonic biopsy
syndrome); ectopic Inhalation of toxic Ammonia; nitrogen Exposure history;
bronchus; fumes and dusts dioxide, or other irritant chest imaging
tracheoesophageal fistula gases; smoke; talc;
Vascular Pulmonary (intralobar) Chest CT imaging silicates
abnormalities sequestration; pulmonary Chronic organ Bone marrow, lung and History; PFT;
artery aneurysm rejection following heart lung chest CT imaging
Lymphatic Yellow-nail syndrome History of transplantation transplantation; with inspiratory
abnormalities dystrophic, slow associated with and expiratory
growing nails obliterative bronchiolitis views
Immunodeficiency States
IgG deficiency Congenital (Bruton’s Quantitative C. DIAGNOSIS
type) immunoglobulin • Chest radiograph
agammaglobulinemia; levels; o reveal such findings as
selective deficiency of immunoglobulin ▪ overinflated lungs or marked volume loss,
subclasses (IgG2, IgG4); subclass levels; ▪ increased opacities
acquired immune globulin impaired ▪ dilated fluid-filled airways
deficiency; common response to ▪ crowding of the bronchi
variable immunization with ▪ atelectasis.
hypogammaglobulinemia; pneumococcal
• Bronchoscopy
Nezelof’s syndrome; vaccine
o Bronchograms can confirm cylindrical, cystic, or
“bare lymphocyte”
varicose bronchiectasis
syndrome
▪ crowding of the bronchi
IgA deficiency Selective IgA deficiency ± Quantitative ▪ loss of bronchovascular markings
ataxia-telanglectasia immunoglobulin ▪ severe cases: honey-combing, air-fluid levels,
syndrome levels fluid-filled nodules
Leukocyte Chronic granulomatous Dihydrorhodamine o Bronchoscopy was once the gold standard for the
dysfunction disease (NADPH oxidase 123 (DHR) diagnosis of BCFB
dysfunction) oxidation test; • High-resolution computed tomography (HR-CT) scan
nitroblue o virtually replaced bronchography as the best tool for
tetrazolium test; diagnosing NCFB
genetic testing o diagnosis is made on the basis of an internal diameter
Other rare humoral WHIM syndrome; Neutrophil count; of a bronchus that is wider than its adjacent pulmonary
immunodeficiencies hypergammaglobulinemia quantitative artery, a failure of the bronchi to taper, and the
(CXCR4 mutation, M immunoglobulin visualization of bronchi in the outer 1 to 2 cm of the lung
CD40 deficiency, levels fields
CD40 ligand o used to better clarify the findings from chest radiograph
deficiency, and and standard CT scans, and mapping airway
others) abnormalities that cannot be identified on routine films
Abnormal Secretion Clearance of the chest
Ciliary defects of Kartagener’s syndrome; Chest x-ray • Spirometry testing
airway mucosa ciliary dyskinesis showing situs o used to determine if the bronchiectasis is primarily an
(formally called impaired inversus; obstructive or restrictive lung pathophysiology, and
ciliary motility syndrome) bronchial biopsy; arterial blood gas measurements can confirm if the
ciliary motility patient has mild, moderate, or severe bronchiectasis.
studies; electron
microscopy of
sperm or
respiratory
mucosa
Cystic fibrosis Typical early childhood Sweat chloride;
(mucoviscidosis) syndrome; later genetic testing
presentation with
predominantly
sinopulmonary symptoms

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II. CARDIOPULMONARY CLINICAL MANIFESTATIONS
ASSOCIATED WITH BRONCHIECTASIS • Chest Assessment Findings
o Obstructive in nature:
A. CLINICAL DATA OBTAINED AT THE PATIENT’S BEDSIDE ▪ Decreased tactile and vocal
▪ Hyperresonant percussion note
• The disease may create an obstructive or a restrictive lung ▪ Diminished breath sounds
disorder or a combination of both. ▪ Wheezing
• If the majority of the bronchial airways are only partially ▪ Crackles
obstructed, the bronchiectasis manifests primarily as an o Restrictive in nature (atelectasis and consolidation):
obstructive lung disorder. ▪ Increased tactile and vocal fremitus
o By contrast, the majority of the bronchial airways are ▪ Bronchial breath sounds
completely obstructed, the distal alveoli collapse, ▪ Crackles
atelectasis results, and the bronchiectasis manifests ▪ Whispered pectoriloquy
primarily as a restrictive disorder. ▪ Dull percussion note
• If the disease is limited to a relatively small portion of the
lung, the patient may not have any of the following typical B. CLINICAL DATA OBTAINED FROM LABORATORY TESTS
clinical manifestations of bronchiectasis. AND SPECIAL PROCEDURES
i. THE PHYSICAL EXAMINATION
• Vital Signs
o Increased respiratory rate (Tachypnea)
o Several pathophysiologic mechanisms operating
simultaneously may lead to an increased frequency of
breathing (respiratory rate – RR):
▪ Stimulation of peripheral chemoreceptors
(hypoxemia)
▪ Decreased lung compliance and increased
ventilatory rate relationship
▪ Anxiety
o Increased heart rate (pulse) and blood pressure
• Use of accessory muscles during inspiration
• Use of accessory muscles during expiration
• Pursed-lip breathing (when pathology is primarily obstructive
in nature)
• Increased anteroposterior chest diameter (Barrel Chest) –
when pathology is primarily obstructive in nature
• Cyanosis
• Digital clubbing
• Peripheral edema and venous distention
o Because polycythemia and cor pulmonale are
associated with severe bronchiectasis, the following
may be seen:
▪ Distended neck veins
▪ Pitting edema
▪ Enlarged and tender liver
• Cough, sputum production, and hemoptysis
o hallmark of bronchiectasis: chronic cough with
production of large quantities of foul-smelling sputum
o 24 hour collection of sputum is usually voluminous and
tends to settle into several different layers
o Streaks of blood are seen frequently in the sputum,
presumably originating from necrosis of the bronchial
walls and erosion of bronchial blood vessels
o Frank hemoptysis may also occur but is rarely life
threatening.
o Secondary bacterial infections are frequent because of
the excessive bronchial secretions.
o Commonly cultured from the sputum of patients with
bronchiectasis:
▪ Haemophilus influenzae
▪ Streptococcus
▪
▪ Pseudomonas aeruginosa
▪ Various anaerobic organisms
o Productive cough seen in patients with bronchiectasis
is triggered by the large amount of secretions that fill the
tracheobronchial tree.
▪ Stagnant secretions stimulate the subepithelial
mechanoreceptors – produce a vagal reflex that
triggers the cough
▪ Subepithelial mechanoreceptors are found in the
trachea, bronchi, and bronchioles, but are
predominantly located in the upper airways

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C. ABNORMAL LABORATORY TESTS AND PROCEDURES
• Hematology
o Increased hematocrit and hemoglobin
o Elevated white blood count (WBC) if patient is acutely
infected
D. SPUTUM CULTURE RESULTS AND SENSITIVITY
• Streptococcus pneumoniae
• Haemophilus influenzae
• Pseudomonas aeruginosa
• Anaerobic organisms
E. RADIOLOGIC FINDINGS
• Chest Radiograph
o Obstructive in nature:
▪ Translucent (dark) lung fields
▪ Depressed or flattened diaphragms
▪ Long and narrow heart (pulled down by
diaphragms)
▪ Enlarged heart (when heart failure is present)
▪ Tram-tracks – tram-lines seen in cylindrical
bronchiectasis, parallel or curved opacity lines of
varying length caused by bronchial wall thickening
▪ Areas of consolidation and/or atelectasis may or
may not be seen
▪ Lungs become inflated – increased FRC and
depressed diaphragms
o Restrictive in nature:
▪ Atelectasis and consolidation
▪ Infiltrates (suggesting pneumonia)
▪ Increased opacity
▪ Generalized bronchiectasis: overinflation of the
lungs
▪ Localized bronchiectasis: when atelectasis and
consolidation develop as a result of bronchiectasis,
an increased opacity and reduced lung volume are
seen in these areas on the radiograph
F. BRONCHOGRAM
• Bronchography – injection of an opaque contrast material
into the tracheobronchial tree
o Routinely performed on patients with bronchiectasis
o Replaced by CT scan of the chest
G. COMPUTED TOMOGRAPHY (CT SCAN)
• Increased bronchial wall opacity is often seen. Bronchial
walls may appear as:
o Thick
o Dilated
o Characterized by ring lines or clusters
o Signet ring-shaped
o Flame-shaped
• Confirms specific type of bronchiectasis
• Confirms atelectasis, consolidation, fibrosis, scarring and
hyperinflation
• Excellent tool to use for Lung Mapping – ability top map out
and determine precisely where the chest physical therapy
would be delivered, or exactly where surgical resection of
lung should be performed
III. GENERAL MANAGEMENT OF BRONCHIECTASIS
• General treatment plan is aimed at controlling pulmonary
infections, airway secretions, and airway obstruction and
preventing complications
• Routine parts of the treatment:
o Daily chest percussion
o Postural drainage
o Effective coughing exercises to remove bronchial
secretions
• Prescribed during periods of exacerbation:
o Antibiotics
o Bronchodilators
o Expectorants
• Childhood vaccinations and yearly influenza vaccinations
help reduce the prevalence of some infections
• Avoidance of upper respiratory infections, smoking, and
polluted environments helps reduce susceptibility to
pneumonia in these patients
• Surgical resection for patients who respond poorly to therapy
or experience massive bleeding
IV. RESPIRATORY CARE TREATMENTS PROTOCOLS
• Oxygen Therapy Protocol
o To treat hypoxemia, decrease the work of breathing and
decrease myocardial work
▪ Hypoxemia that develops in bronchiectasis is
caused by the pulmonary shunting associated with
the disorder
▪ Hypoxemia may not respond well when true or
capillary pulmonary shunting is present
• Bronchopulmonary Hygiene Therapy
▪ Directed cough
▪ Exercise breathing programs
▪ Autogenic breathing
▪ Forced expiration
▪ Chest physical therapy
▪ Suctioning
▪ Positive expiratory pressure
▪ Oscillatory PEP
▪ High frequency chest wall compression
o High frequency chest compression devices
“pneumovest” – chest percussion
▪ Chest wall pain may limit their usefulness
▪ Expensive
• Lung Expansion Therapy
o Attempts to keep distal lung units inflated may involve
the use of deep breathing and coughing and incentive
spirometry
• Aerosolized Medication Therapy Protocol
o Sympathomimetic and parasympatholytic agents are
commonly used in selected patients with bronchiectasis
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 to induce bronchial smooth muscle relaxation –
reversible airway obstruction
o Use of corticosteroids is discouraged
▪ May cause bronchospasm and are not approved
for use in bronchiectasis by the FDA
• Mechanical Ventilation Protocol
o Invasive and noninvasive mechanical ventilation may
be necessary to provide and help improve alveolar
ventilation and eventually return the patient to
spontaneous breathing
o Continuous mechanical ventilation is justified when the
acute ventilatory failure is thought to be reversible
▪ When acute pneumonia exists as a complicating
factor

V. MEDICATIONS COMMONLY PRESCRIBED BY THE

PHYSICIAN
• Expectorants
o Ordered when oral liquids and aerosol therapy alone
are not sufficient to facilitate expectoration
o Clinical effectiveness is doubtful
• Antibiotics
o Administered to treat associated respiratory tract
infections