Unit 5 - Intrinsic Restrictive Lung Dysfunction and Pulmonary Rehabilitation

Interstitial Lung Disease & Fibrotic Restriction

• refers to >130 lung conditions causing inflammation and/or fibrosis of lung tissue

• Fibrosis results from abnormal healing after repeated alveolar injury

• Stiff, scarred lungs reduce compliance and impair gas exchange

Interstitial Lung Disease & Fibrotic Restriction

Common causes

environmental exposures, autoimmune disease, medications, or unknown (idiopathic)

Idiopathic Pulmonary Fibrosis (IPF)

• Triggered by repeated epithelial injury to type II alveolar cells

  • Core mechanism: abnormal wound healing

• Fibroblast activation → collagen deposition → alveolar wall thickening and scarring

• Leads to stiff, noncompliant lungs and impaired oxygen diffusion

Idiopathic Pulmonary Fibrosis (IPF)

Environmental risks

smoking, microaspiration, metal/wood dust, air pollution

Idiopathic Pulmonary Fibrosis (IPF)

Genetic risks

mutations affecting epithelial repair and immune regulation

Idiopathic Pulmonary Fibrosis (IPF)

Clinical Presentation, Diagnostic Findings, and Medical Management

Affects adults ≥50; median survival 2–3 years

Idiopathic Pulmonary Fibrosis (IPF)

Symptoms

progressive exertional dyspnea, dry cough, fatigue, reduced activity tolerance

Idiopathic Pulmonary Fibrosis (IPF)

Physical exam

fine inspiratory crackles at bases, digital clubbing, ↓ chest expansion

Idiopathic Pulmonary Fibrosis (IPF)

PFTs

restrictive pattern with ↓ lung volumes and impaired gas exchange

Idiopathic Pulmonary Fibrosis (IPF)

Antifibrotic medications

nintedanib and pirfenidone slow fibrosis progression

Idiopathic Pulmonary Fibrosis (IPF)
Other management

oxygen, GERD control, transplant referral if eligible

Pulmonary Rehabilitation in IPF

• Improves short-term exercise tolerance, dyspnea, and quality of life

• Enhances medication tolerance and daily function despite disease progression

Pulmonary Rehabilitation in IPF

Interventions: Endurance training

• 3–5x/week, 20–60 min, treadmill or cycling

• ~60% max capacity based on ET

• Interval training if continuous activity poorly tolerated

Pulmonary Rehabilitation in IPF

Interventions: Resistance training

• 2–3x/week, 1–3 sets of 8–12 reps

• ~60–70% of 1-RM to improve strength and conserve energy

Pulmonary Rehabilitation in IPF

Interventions: Safety and Monitoring

• Monitor SpO₂; maintain >88% with oxygen titration

• Watch for red flags: rest dyspnea, desaturation, chest pain, unresolved fatigue

Pulmonary Rehabilitation in IPF

Interventions: Education and Long-Term Benefits

• Teach energy conservation, breathing techniques, self-monitoring

• Emphasize home continuation for sustained quality of life gains

Sarcoidosis

• A multisystem inflammatory disease of unknown cause

  • Most commonly affects lungs, but also skin, lymph nodes, and eyes

• Characterized by non-caseating granulomas

  • A granuloma is a collection of immune cells (macrophages, lymphocytes) formed in response to persistent inflammation

• Pulmonary features progress from alveolitis → granuloma formation → fibrosis

• Variable course; many cases spontaneously resolve

PT and Rehabilitation Considerations

• improves exercise capacity and dyspnea; variable effects on fatigue and QOL

  • Rehab should be individualized, considering multisystem involvement

PT and Rehabilitation Considerations

Endurance training

• 2–3x/week, 20–60 min/session

• Treadmill, cycling, or stepper

• Start around 60–80% of 6MWT speed or use RPE

PT and Rehabilitation Considerations

Resistance training

• 2–3x/week, 1–3 sets of 8–12 reps

• 60–70% of 1RM or fatigue within rep range

• Targets steroid-related weakness and deconditioning

PT and Rehabilitation Considerations

Fatigue

• common (up to 90%), not always related to lung function

  • Exercise does not worsen fatigue; may improve it

  • Use energy conservation and pacing strategies

PT and Rehabilitation Considerations

Functional monitoring

6MWT, SpO₂, dyspnea & RPE scales

PT and Rehabilitation Considerations

Inspiratory muscle training

may help select patients

PT and Rehabilitation Considerations

Watch for red flags

chest pain, worsening dyspnea, persistent fatigue

Lung Cancer

NSCLC

• ~85% of cases (adenocarcinoma, squamous, large cell)

  • Adenocarcinoma common in smokers and non-smokers

Lung Cancer

SCLC

• ~15% of cases; fast-growing, often metastatic at diagnosis

• Strong smoking association (+squamous)

Lung Cancer

Risk factors

tobacco, radon, asbestos, diesel exhaust, genetics

Lung Cancer

• Often diagnosed late; 5-year survival ~25% if localized

• PT involvement spans pre-op to palliative care, based on disease stage

Lung Cancer

Tumors

• may arise in central airways or peripheral lung tissue

• Can cause restrictive and/or obstructive impairments, depending on locatio

Lung Cancer

General symptoms

dyspnea, fatigue, cough, reduced exercise tolerance

Lung Cancer

Local effects

• Airway obstruction → atelectasis, pneumonia, V/Q mismatch

• Pleural invasion → malignant effusions

• Pericardial spread → arrhythmias, cardiac effects

Lung Cancer

Frequent metastasis sites

Brain, bone, liver, adrenal glands, lymph nodes

Lung Cancer

PTs must consider…

tumor burden, pleural effects, and treatment-related changes

Lung Cancer

Diagnostic Workup

• Bronchoscopy allows direct tumor visualization and biopsy

• Additional methods: CT, PET scan, and needle or surgical biopsy

• TNM staging evaluates tumor size, lymph node spread, and metastasis

• PFTs may show restrictive, obstructive, or mixed pattern

Lung Cancer

Medical and Surgical Management

• Surgery may reduce lung volume → dyspnea, decreased chest mobility

• Radiation can cause fibrosis and fatigue

• Chemotherapy side effects: fatigue, neuropathy, myelosuppression

• Immunotherapy may cause inflammatory effects (e.g., pneumonitis, myositis)

Lung Cancer

PT role

early mobility, breathing exercises, pacing, fatigue management

Lung Cancer

PT Examination: Post-Operative Focus

• Chest expansion & accessory muscle use

• Diminished breath sounds, possible crackles

• Thoracotomy/VATS/chest tube scars; assess UE ROM

  • Pain may limit deep breathing& movement

• Screen for bone or brain mets if new pain or neuro signs

• Check for neuropathy if on neurotoxic chemo

• Assess exercise tolerance (e.g., 6MWT)

  • Monitor vitals, SpO₂, and dyspnea during activity

Rehab Strategies in Outpatient Lung Cancer Care

Aerobic training

3–5x/week, 20–40 min/session, moderate intensity

Rehab Strategies in Outpatient Lung Cancer Care

Resistance training

2–3x/week, major muscle groups, moderate loads

Rehab Strategies in Outpatient Lung Cancer Care

Breathing retraining

or inspiratory muscle training if indicated

Rehab Strategies in Outpatient Lung Cancer Care

Flexibility/stretching

target thorax and shoulders post-surgery/radiation

Rehab Strategies in Outpatient Lung Cancer Care

Balance training

for patients with neuropathy or gait deficits

Rehab Strategies in Outpatient Lung Cancer Care

Mind-body options

(e.g., yoga, Tai Chi) for flexibility, stress reduction

Rehab Strategies in Outpatient Lung Cancer Care

Energy conservation and pacing strategies

reduce cancer-related fatigue

Rehab Strategies in Outpatient Lung Cancer Care

Advanced disease

low-intensity, symptom-guided activity to preserve function

Overview of Respiratory Impairment from Neuromuscular Dysfunction

• Restriction = reduced lung volumes, not airflow

• Neuromuscular dysfunction → impaired chest expansion

• ↓ Inspiratory effort → ↓ tidal volume

• ↓ Expiratory force → weak cough, secretion buildup

• ↑ Risk: atelectasis, hypoventilation, infection

• Seen in: SCI, ALS, GBS, MG, dystrophies

Spinal Cord Injury

• Injury level = loss of ventilatory muscle control

• High cervical SCI → diaphragm weakness or paralysis

• ↓ Intercostals/abdominals → ↓ chest wall expansion, cough

• Exam: paradoxical breathing, ↓ chest excursion, weak cough

• Respiratory Intervention Considerations: assisted cough, breathing retraining, abdominal binder

  • Supine diaphragm facilitation supports early retraining

Motor Neuron Disease: Amyotrophic Lateral Sclerosis

progressive loss of respiratory muscle strength

Motor Neuron Disease: Amyotrophic Lateral Sclerosis

Early sign

nocturnal hypoventilation, fatigue, morning headache

Motor Neuron Disease: Amyotrophic Lateral Sclerosis

Bulbar signs

↑ aspiration risk, ↓ airway protection

Motor Neuron Disease: Amyotrophic Lateral Sclerosis

Exam

↓ chest expansion, weak cough, paradoxical breathing

Motor Neuron Disease: Amyotrophic Lateral Sclerosis

Respiratory Intervention Considerations

• IMT, assisted cough, chest mobility, breathing retraining

• NIV (e.g., BiPAP, mouthpiece) supports gas exchange

Peripheral Nerve & Neuromuscular Junction Disorders: GBS

rapid onset, ascending paralysis, risk of early ventilatory failure

Peripheral Nerve & Neuromuscular Junction Disorders: MS

fluctuating weakness, worsens with exertion, improves with rest

Peripheral Nerve & Neuromuscular Junction Disorders: MS + GBS

↓ tidal volume, ↓ cough, ↑ risk of infection or crisis

Peripheral Nerve & Neuromuscular Junction Disorders: PT

• airway clearance, energy conservation, gradual retraining

• Monitor closely for fatigue and evolving respiratory status

Pulmonary Embolism

Definition & Pathophysiology

• acute blockage of pulmonary artery, most often by a clot from DVT (part of VTE spectrum)

• Hallmark issue: impaired perfusion → V/Q mismatch & hypoxemia.

• Dead space ventilation: alveoli ventilated but not perfused.

• Inflammation, pain, or infarct may produce an acute restrictive pattern (↓ lung expansion)

Large PE

↑ pulmonary resistance → acute pulmonary hypertension & RV strain.

Smaller PE

may cause infarction, pleuritic pain, and hemoptysis

PE: Risk Factors & Clinical Presentation

Classic symptoms

sudden dyspnea, pleuritic chest pain, tachypnea, anxiety

PE: Risk Factors & Clinical Presentation

Other symptoms

cough, hemoptysis, tachycardia, or signs of DVT (leg swelling, tenderness)

PE: Risk Factors & Clinical Presentation

Massive PE

hypotension, syncope, shock

PE: Risk Factors & Clinical Presentation

Diagnosis

D-dimer (screening), CT angiography (gold standard), V/Q scan, LE ultrasound

PE: Risk Factors & Clinical Presentation

Imaging

• EKG may show sinus tachycardia

• Echo may show RV strain

PE: Risk Factors & Clinical Presentation

PT red flag

sudden SOB/chest pain post-op = STOP & refer

PE: Risk Factors & Clinical Presentation

Anticoagulation

alters treatment – modify for bleeding risk

PT Implications After PE

Common findings

SOB, tachycardia, ↓ SpO₂, pleuritic pain, accessory muscle use

PT Implications After PE

O₂ support

common; lungs may sound normal or have pleural rub

PT Implications After PE

Mobility

Begin once stable on anticoagulants or with IVC filter

PT Implications After PE

Start low-level activity

monitor vitals, avoid bleeding-risk techniques

PT Implications After PE

Breathing

Use diaphragmatic & splinted breathing to improve ventilation

PT Implications After PE

Upright positioning

relieves dyspnea; encourage movement

PT Implications After PE

Educate

on pacing, red flags, and med adherence

PT Implications After PE

Refer to rehab

if deconditioning or pulmonary HTN persists

Pulmonary Rehabilitation

Overview & Indications

• a comprehensive, multidisciplinary program for chronic respiratory diseases.

• Combines individualized exercise, education, and behavioral strategies.

• Similar to cardiac rehab but focuses on breathing and oxygen use.

• Commonly used for COPD, ILDs, CF, bronchiectasis, asthma, and post-lung surgery.

• Intended for medically stable outpatients with chronic functional limitations

Pulmonary Rehabilitation

Benefits & Rationale

• PR breaks the cycle of dyspnea, inactivity, and deconditioning.

• Increases exercise tolerance and reduces breathlessness.

• Improves muscle efficiency and reduces ventilatory demand.

• Enhances psychological well-being and reduces isolation.

• Leads to fewer hospitalizations and improved daily function

Pulmonary Rehabilitation

Program Structure & Initial Assessment

• Programs run 8–12 weeks with 2–3 sessions/week including exercise and education.

• Delivered by a multidisciplinary team (PTs, RTs, nurses, physicians).

• Initial evaluation includes history, PFTs, symptom scales, and physical exam.

• Functional testing (e.g., 6MWT) helps guide individualized prescription.

• Health-related Quality of Life (e.g., St. George’s Respiratory Questionnaire) used to assess how the patient perceives their condition.

• Patient goals are set to shape the rehab plan and motivate participation

Pulmonary Rehabilitation

Exercise Training Components

Includes aerobic, resistance, inspiratory muscle, flexibility, and functional training

Pulmonary Rehabilitation

Exercise Training Components: Aerobic

targets 20–30 minutes using interval or continuous formats

Pulmonary Rehabilitation

Exercise Training Components: Intensity

guided by dyspnea scale and SpO₂ monitoring, not just heart rate

Pulmonary Rehabilitation

Exercise Training Components: Strengthening

improves limb and respiratory muscle performance

Pulmonary Rehabilitation

Exercise Training Components: Functional tasks

practiced to simulate real-world challenges and goals

Pulmonary Rehabilitation

Education & Psychosocial Support

• Patients learn about their condition and how to manage it day to day.

• Inhaler technique, breathing strategies, and airway clearance are emphasized.

• Nutrition, weight management, and smoking cessation are addressed.

• Group support helps reduce anxiety and builds resilience.

• Patients leave with action plans and self- management skills for long-term success