This document discusses proactive approaches to lung health in ALS patients. It emphasizes early, non-mechanical techniques to promote alveolar expansion, airway clearance, and coughing. These include breathing exercises and manual techniques. It stresses the importance of prevention through vaccinations, avoiding infection and irritants, and maintaining overall health and wellness. The goal is to delay lung dysfunction progression and respiratory complications through multidisciplinary strategies that improve both physical and emotional well-being.

1. An ounce of prevention: Proactive
approaches to lung health in ALS
Lee Guion MA, RRT, FAARC
Forbes Norris MDA/ALS Research & Treatment Center

2. 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

3. Lung Health: Back to Basics
 Questions to consider when recommending mechanical
lung expansion and coughing treatments
 Low-tech breathing and coughing techniques

4. 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

5. 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)

6. 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

7. Pulmonary Ecosystem
 Photo

8. Lung Health: Back to Basics
Anatomy of the lower airway
 Bronchi
 Terminal bronchioles
 Alveoli
 Pulmonary vessels

9. 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)

10. 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

11. 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 submucosal
glands in the airway
 Components:
 Water 95%
 Protein and carbohydrate 3%
 Lipids 1%
 Miscellaneous 1%

12. 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

13. 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)

14. 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

15. 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)


16. 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)

17. 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)

18. 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

19. 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

20. 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.)

21. 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

22. 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)

23. 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)

24. 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

25. Back to Basics: Proactive Approach to
Lung Health: Summary
 Improvement in overall health
 Physical
 Emotional
 Creating environment
 Positive
 Hopeful

26. Back to Basics: Proactive Approach to
Lung Health
 Photo