An ounce of prevention: Proactive

approaches to lung health in ALS

Lee Guion MA, RRT, FAARC

Forbes Norris MDA/ALS Research & Treatment Center

Lung Health: Back to Basics

Multidisciplinary prevention strategies

Anatomy and physiology of the lower airway

Lungs’ innate immunity protection (early response) and

Adaptive immunity (late response) to microbial

pathogens

Lung Health: Back to Basics

Questions to consider when recommending mechanical

lung expansion and coughing treatments

Low-tech breathing and coughing techniques

Goals of respiratory care in general and lung

volume recruitment therapy specifically

Prevention of:

Chronic hypoventilation

Atelectasis (deflated alveoli)

Impaired gas exchange (decreased blood oxygen

levels/increased carbon dioxide)

Impaired lower airway secretion clearance

Pulmonary infection

Lung Health: Backs to Basics

Prevention

Vaccination

Pneumococcal polysaccharide pneumonia vaccine (initial dose and booster > 5 years)

Annual influenza vaccine

Treat symptoms of upper respiratory tract infection and seasonal rhinitis (to help reduce chances of lower airway infection)

Reduce or avoid exposure to smoke, dust, fumes that may contribute to airway inflammation

Avoid infective agents (hand washing, distance, masks)

Education about early detection of lower respiratory tract infection

Routinely measure lung function and degree of decline (treat with noninvasive positive pressure/lung volume replacement therapy when symptoms and results warrant)

Lung Health: Back to Basics

Prevention

Maximize nutrition (malnutrition immunosuppression)

Maintain good hydration (electrolyte balance) Na+/Cl-

Encourage good dental and oral hygiene (strategies when it is challenging)

Assess aspiration risk and teach avoidance techniques

Encourage movement/safe exercise (blood neutrophil counts, lymphocyte counts that lead to immunosuppression)

Encourage adequate sleep and address barriers to restorative levels of sleep

Pulmonary Ecosystem

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Lung Health: Back to Basics

Anatomy of the lower airway

Bronchi

Terminal bronchioles

Alveoli

Pulmonary vessels

Lung Health: Back to Basics

Mucociliary system: components

Conducting airways in the lung and nasal cavity (from nose to alveoli)

Secretions lining the surface of the airway (usually called mucus)

Contains a gel layer and a sol layer

Cilia (microscopic hairs that create a wave action, propelling or escalating mucus and trapped particles toward the larynx and upper airway where it is swallowed or expectorated)

Lung Health: Back to Basics

The normal lung and innate immunity

Role of mucus protection and maintenance

Humidification

warming inspired gases

Components mucociliary escalator (blanket)

transport of debris (trapped inhaled particles, cellular debris, dead and aging cells)

antibacterial activity

phagocytes and other professional scavengers --antimicrobial substances naturally present in secretion lining of respiratory tract –– which engulf and kill microorganisms

alveolar macrophages

Lung Health: Back to Basics

Physiology of the mucociliary system

Mucus: Definition

A gel with complex properties of viscosity and elasticity

Secreted by surface goblet cells and submucosalglands in the airway

Components:

Water 95%

Protein and carbohydrate 3%

Lipids 1%

Miscellaneous 1%

Lung Health: Back to Basics

Total secretions include

Goblet cells

contain granules of mucus covered by thin membrane

6800 goblet cells/sq millimeter of normal airway mucosa

respond to irritants by increasing production of mucus (split open to discharge mucus granules onto airway surface)

transported by cilia

Lung Health: Back to Basics

Total secretions include

Submucosal glands

glycoprotein (which gives mucus its physical and

chemical properties such as viscosity and elasticity)

oligosaccharide units (a sugar, that is the independent

carbohydrate unit of glycoproteins)

Lung Health: Back to Basics

Physiology of the mucociliary system

A healthy person produces up to 100 mL of mucus

every 24 hours (most reabsorbed in the bronchial

mucosa)

10 mL reaching the glottis (and inadvertently

swallowed)

Mucus and secretions are terms used interchangeably

Lung Health: Back to Basics

Physiology of the mucociliary system

Positive and negative ion exchange (largely sodium

and chloride) and reabsorption from the airways

If balance is maintained, mucociliary clearance is

normal in the absence of pulmonary diseases such as

(chronic bronchitis, bronchiectasis, and cystic fibrosis)

Lung Health: Back to Basics

Sputum: Definition

Expectorated secretions that contain:

Respiratory tract secretions (from mucus membranes)

Nasopharyngeal secretions (sinus and nasal passages)

and

Oropharyngeal secretions (saliva)

Lung Health: Back to Basics

Breathing

Normal lung: Inhalation/Exhalation

Inflation:

Pressure change from ambient to negative pressure

allows for airflow from nose to airways

Small and large bronchioles expand in diameter and

length (Enlarge early during inflation to FRC and changes

mainly in length while lungs expand to TLC)

Collateral ventilation (alveoli connected to adjacent units

through Pores of Kohn and Lambert’s canals)

Lung Health: Back to Basics

Breathing

Normal lung: Inhalation/Exhalation

Deflation:

Alveolar pressure provides the “pressure head” that

expels air from these terminal gas exchange units during

exhalation

Elastic recoil provides direct traction on small airways that

affects

intrapleural pressure surrounding the bronchi and distends

them (inhalation)

transpulmonary positive pressure (across the lungs) during

vigorous expiratory efforts

Lung Health: Back to Basics

Breathing

Normal lung

Working with normal lung function to enhance benefit,

especially in patients with decreased mobility

Breathing retraining

Confidence building

Body as friend in face of progressive motor decline

Lung Health: Back to Basics

Questions to consider

Before recommending mechanical secretion clearance therapy:

What is the pathophysiological basis for recommending secretion mobilization therapy?

Are airway secretions affecting lung function or gas exchange in any meaningful way?

Are there potential adverse effects from therapy?

What therapy technique is likely to provide the greatest benefit?

Is the cost of therapy going to be covered by third-party insurers?

What is the patient preference for one therapy over another or for any therapy at all?

(Dean Hess. RRT, PhD.)

Low-tech breathing and coughing techniques

Segmental (6-sided) and diaphragmatic breathing Visualization:umbrella, water pitcher

Pursed-lip breathing

Focus on exhalation – not inhalation (3-to-1 ratio /out-to-in)

Forced exhalation techniques

Open “huff” or closed glottis

With or without partner

With abdominal muscles or manual assistance

Active Cycle Breathing Technique

www.amyandpals.com

Benefits of early intervention with breath

training

FVC < 80% or with symptoms of dyspnea with activity

Gives patients a sense of control and reduce feelings of anxiety (the dyspnea/anxiety/rapid-shallow breathing negative feedback loop) Slide

Use anywhere, anytime

Improves exercise tolerance (incorporate into PT recommendations)

Contributes to energy conservation

Use with partner or spouse or friends (together, reinforce, they benefit too)

Benefits of early intervention with breath

training

Allows close observation of lung muscle use

(diaphragm, accessory, equal or unequal expansion).

Hands on demonstration can further this assessment

Benefit to clinician (centering, being present)

Teaching: 1:1 in clinic or group setting (support groups)

Back to Basics: Proactive Approach to

Lung Health: Summary

Early, non-mechanical techniques/strategies

Alveolar expansion

Volume replacement

Airway expansion

Coughing strategies

Prevention

Vaccinations

Avoidance of infective agents

Avoidance of inflammatory irritants

Back to Basics: Proactive Approach to

Lung Health: Summary

Improvement in overall health

Physical

Emotional

Creating environment

Positive

Hopeful

Back to Basics: Proactive Approach to

Lung Health

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