Respiratory Problems in Post-Polio Syndrome

Dr. Marshall Reilly

Consultant Respiratory Physician

Belfast City Hospital

Post-Polio

• Previously involved muscle groups• People who had polio in later

childhood or as adults

• Muscle weakness• Fatigue

O2

CO2

Respiratory Centres

Scoliosis

Hypoventilation

Symptoms of hypoventilation

• Breathlessness• Daytime sleepiness• Morning headache• Reduced intellectual function• Reduced quality of life

But frequent underestimation of symptoms

Respiratory failureNormal Type I

(pneumonia, asthma)

Type II (nerve, muscle, chest wall)

Oxygen 12 kPa 6 kPa 6 kPa

Carbon dioxide

5 kPa 3 kPa 8 kPa

Types of Ventilation

• Negative pressure ventilation

• Positive pressure ventilation– invasive– non-invasive (NIV)

http://en.wikipedia.org/wiki/Image:Iron_Lung_ward-Rancho_Los_Amigos_Hospital.gif

• Copenhagen 1950’s – Polio outbreak resulted in first use of positive pressure ventilation

• Early 1980’s - Long-term positive pressure ventilation via tracheostomies

• Late 1980’s Rideau et al and Delaibier et al both showed successful treatment of DMD and polio associated respiratory failure with nasal positive pressure ventilation

Positive pressure Non-Invasiveventilation

Case history

• Short of breath

• Morning headaches lasting for 2 hours or so after awakening

• Sleepy during the day

• General tiredness

• Scoliosis

Establishing ventilation

Admit to hospital for 3-4 days

• Accommodate to mask

• Use intermittently during night

• Tolerate most of night

• Education for patient and relatives/carers

Change in Blood Gases with nocturnal non-invasive ventilation

13/8/01 16/8/01 4/3/08

pO2 (kPa) 5.5 6.8 9.2

pCO2 (kPa) 7.52 6.98 6.2

Non-invasive evaluation of oxygenation

At review

• Much better

• No longer sleepy

• Headaches gone

• Able to do the shopping, go on holiday, enjoy life again

Efficacy of NIV

X

NMD

OBS

CWD

< 4hrs NIV

Nickol et al 2002i.e. aim for > 4hours use

Consensus Report - Chest 1999; 116:521-534

Chronic Noninvasive ventilation• Restrictive chest wall disease: scoliosis thoracoplasty obesity/hypovent• Stable neuromuscular: post polio myopathies neuropathies

spinal muscular atrophy• Progressive neuromuscular Duchenne MD MND/ALS• Neurological CCHS Spinal cord lesions CVA• Airway diseases

Disease Categories in Europe

0%

20%

40%

60%

80%

100%

Lung/airways Thoracic Cage NeuromuscularLloyd Owen 2005

Survival: Probability of continuing domiciliary NIV

Simonds Thorax 1995

Other issues

• Flu & pneumonia vaccination

• Avoid sedative medication

• Prompt antibiotics

CONCLUSIONS

• Noninvasive ventilation can reverse hypoventilation in patients with neuromuscular disease

• Let your doctor know if you develop any of the symptoms of respiratory failure

Symptoms of hypoventilation

• Breathlessness• Daytime sleepiness• Morning headache• Reduced intellectual function• Reduced quality of life

But frequent underestimation of symptoms