1. Respiratory Problems in Post-Polio Syndrome
Dr. Marshall Reilly
Consultant Respiratory Physician
Belfast City Hospital

2. Post-Polio Previously involved muscle groups
People who had polio in later childhood or as adults
Muscle weakness
Fatigue

3. O2
CO2

4. Respiratory Centres

6. Scoliosis

7. Hypoventilation

8. Symptoms of hypoventilation
Breathlessness
Daytime sleepiness
Morning headache
Reduced intellectual function
Reduced quality of life
But frequent underestimation of symptoms

9. Respiratory failure Normal Type I (pneumonia, asthma)
Type II (nerve, muscle, chest wall)
Oxygen
12 kPa
6 kPa
Carbon dioxide
5 kPa
3 kPa
8 kPa

10. Types of Ventilation Negative pressure ventilation
Positive pressure ventilation
invasive
non-invasive (NIV)

16. Positive pressure Non-Invasive
ventilation
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

23. Case history Short of breath
Morning headaches lasting for 2 hours or so after awakening
Sleepy during the day
General tiredness
Scoliosis

24. 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

25. 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

26. Non-invasive evaluation of oxygenation

27. Jill Nelson oximetry preNIV

28. Jill Nelson oximetry post NIV

29. At review Much better No longer sleepy Headaches gone
Able to do the shopping, go on holiday, enjoy life again

30. Efficacy of NIV NMD OBS CWD < 4hrs NIV X Nickol et al 2002
i.e. aim for > 4hours use

31. Consensus Report - Chest 1999; 116:521-534

32. 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

33. Disease Categories in Europe
Lloyd Owen 2005

34. Survival: Probability of continuing domiciliary NIV
BACKGROUND--Nasal intermittent positive pressure ventilation (NIPPV) is a new technique which has rapidly supplanted other non-invasive methods of ventilation over the last 5-10 years. Data on its effectiveness are limited. METHODS--The outcome of long term domiciliary NIPPV has been analysed in 180 patients with hypercapnic respiratory failure predominantly due to chest wall restriction, neuromuscular disorders, or chronic obstructive lung disease. One hundred and thirty eight patients were started on NIPPV electively, and 42 following an acute hypercapnic exacerbation. Outcome measures were survival (five year probability of continuing NIPPV), pulmonary function, and health status. A crossover study from negative pressure ventilation to NIPPV was carried out in a subgroup of patients. RESULTS--Five year acturial probability of continuing NIPPV for individuals with early onset scoliosis (n = 47), previous poliomyelitis (n = 30), following tuberculous lung disease (n = 20), general neuromuscular disorders (n = 29), and chronic obstructive pulmonary disease (n = 33) was 79% (95% CI 66 to 92), 100%, 94% (95% CI 83 to 100), 81% (95% CI 61 to 100), 43% (95% CI 6 to 80), respectively. Most of the patients with bronchiectasis died within two years. One year after starting NIPPV electively the mean (SD) PaO2 compared with the pretreatment value was +1.8 (1.9) kPa, mean PaCO (1.3) kPa in patients with extrapulmonary restrictive disorders, and PaO (1.0) kPa, PaCO (0.8) kPa in patients with obstructive lung disease. Arterial blood gas tensions improved in patients transferred from negative pressure ventilation to NIPPV. Health status was ranked highest in patients with early onset scoliosis, previous poliomyelitis, and following tuberculous lung disease. In the group as a whole health perception was comparable to outpatients with other chronic disorders. CONCLUSIONS--The long term outcome of domiciliary NIPPV in patients with chronic respiratory failure due to scoliosis, previous poliomyelitis, and chest wall and pulmonary disease secondary to tuberculosis is encouraging. The results of NIPPV in patients with COPD and progressive neuromuscular disorders show benefit in some subgroups. The outcome in end stage bronchiectasis is poor.
Simonds Thorax 1995

35. Other issues Flu & pneumonia vaccination Avoid sedative medication
Prompt antibiotics

36. 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

37. Symptoms of hypoventilation
Breathlessness
Daytime sleepiness
Morning headache
Reduced intellectual function
Reduced quality of life
But frequent underestimation of symptoms