10 ago. 2019

Respiratory problems in PPS are an important cause of symptoms and complications, including sleep disorders.




Post-polio syndrome  [13][14]

Importance

  • Respiratory problems in PPS are an important cause of symptoms and complications, including sleep disorders.
  • They may be under-diagnosed or inadequately assessed.
  • Treatment can improve both quality of life and prognosis.

Aetiology[14]

Respiratory problems in PPS may be due

to one or more of:
  • Respiratory muscle weakness.
  • Bulbar impairment - this may affect control of the upper airway or the respiratory cycle. If the upper airway is affected, there may be obstructive sleep apnoea.
  • Skeletal deformity - scoliosis or chest wall stiffness.
  • Other pathology - eg, chronic obstructive pulmonary disease (COPD), asthma, obesity.
  • Aspiration - if swallowing is affected.
All these are likely to worsen during sleep. The pattern of respiratory impairment may be hypoventilation, obstructive sleep apnoea, or both.

Symptoms

Respiratory failure can develop insidiously - symptoms may be subtle or unnoticed. Breathlessness may not be a symptom in patients with limited mobility. Possible symptoms are:
  • Sleep disruption, eventually leading to insomnia, daytime sleepiness or fatigue.
  • Morning headaches, irritability, poor concentration, anxiety or depression.
  • Abnormal sleep movements, nocturnal confusion, vivid dreams.
  • Breathlessness which may be positional.
  • Weak cough, and chest infections.

Signs

These may be subtle - possible signs are:
  • Unexplained tachypnoea.
  • Use of accessory muscles.
  • Abdominal paradox - this is inward movement of the abdomen on inspiration while the upper chest expands.
    • May be best seen with the patient supine during a sniff manoeuvre. When upright, it can be missed, as the diaphragm passively descends at the beginning of inspiration.
  • Severe, untreated nocturnal hypoxaemia can cause pulmonary hypertension, giving signs such as raised JVP and ankle oedema.

Assessment of respiratory problems

  • Listen to the patient's story and preferences.
  • Assess:
    • Voice and cough.
    • Chest deformity.
    • Observe patients in realistic situations - eg, doing repeated tests or actions, and doing everyday actions in which they may be using the necessary breathing muscles to achieve another task.
  • Investigations:
    • Peak flow and cough peak flow.
    • Spirometry:
      • Both seated AND supine spirometry are needed.
      • A sensitive indicator of respiratory muscle weakness is reduction in maximal inspiratory pressure.
    • Oximetry (and possibly capnography).
    • Sleep study (polysomnogram).
    • ECG and CXR if appropriate.
    Full sets of lung function tests and arterial blood gases may not be helpful in this scenario, unless intrinsic lung disease is suspected.

Management of respiratory problems

There are various options - choice will depend on the patient's individual situation and preferences.
Night-time mechanical ventilation is often used. This helps by resting the respiratory muscles at night, and preventing deterioration of respiratory function during sleep. It also treats the secondary sleep disorder.

Supportive measures include:
  • Not smoking.
  • Avoiding sedatives and alcohol.
  • Optimal weight and nutrition.
  • Pneumococcal and influenza vaccination.
  • Postural support if needed.
  • Prompt treatment of chest infections.
  • Techniques such as assisted cough or glossopharyngeal breathing ('frog breathing').
  • Chest expansion exercises.
Assisted breathing options are:
  • Non-invasive ventilation (NIV), also called non-invasive intermittent positive pressure ventilation (NIPPV), is often useful - see box below.
  • Rocking bed:
    • This helps breathing by rocking a patient consecutively head up and head down. It is surprisingly effective, especially where muscle weakness is mainly diaphragmatic.
  • Pneumobelt:
    • This gives intermittent abdominal pressure ventilation and is useful for daytime assistance.
  • Negative pressure ventilation:
    • Examples are tank ventilators (iron lung), jacket ventilators (Tunnicliffe), and cuirass ventilators. The devices are cumbersome, and mainly used where NIV is not tolerated, or to provide 'respite' from NIV
  • Tracheostomy ventilation.

Non-invasive ventilation and 'bi-levels' explained[13][14]

NIV increases alveolar ventilation. It is provided by a portable ventilator and a tightly-fitting nasal or facial mask or nasal 'pillow'.
  • NB: NIV is NOT the same as continuous positive airway pressure (CPAP). CPAP is useful for obstructive sleep apnoea because it maintains the upper airway. It is not normally indicated for hypoventilation from respiratory muscle weakness.
People with neuromuscular disease may have difficulty breathing in, so require NIV with higher inspiratory than expiratory pressures. This can be provided using a 'bi-level' ventilator:
  • Bi-level ventilators were developed by modifying CPAP. The inspiratory positive airway pressure (IPAP) and expiratory positive airway pressure (EPAP) settings are adjusted separately.
  • The difference between IPAP and EPAP is called the span.
  • For example, a patient may require an IPAP of 14 and an EPAP of 3.
  • Sensitive flow triggers enable normal breathing to be supported. There may be a back-up control to provide ventilation if respiratory effort fails to trigger a breath.
The symptoms of post-polio syndrome are slowly progressive, with periods of stability lasting 3-10 years.
Prevention of acute polio infection is discussed elsewhere (see separate article onPoliomyelitis).

Prevention of PPS is not much discussed in the literature. Given the various known contributing factors, it seems possible that PPS problems might be reduced by:
  • Careful management of exercise and daily living activities to optimise muscle and joint use, and prevent overuse or disuse.
  • Correct maintenance of aids and prostheses.
  • Monitoring and early treatment of associated/contributing problems such as:

Further reading & references

  1. Trojan DA, Cashman NR; Post-poliomyelitis syndrome. Muscle Nerve. 2005 Jan;31(1):6-19. [abstract]
  2. EFNS guideline on diagnosis and management of post-polio syndrome; Report of an EFNS task force, European Federation of Neurological Societies (2006)
  3. Halstead LS, Silver JK; Nonparalytic polio and postpolio syndrome. Am J Phys Med Rehabil. 2000 Jan-Feb;79(1):13-8. [abstract]
  4. Howard RS; Poliomyelitis and the postpolio syndrome. BMJ. 2005 Jun 4;330(7503):1314-8.
  5. Khan F; Rehabilitation for postpolio sequelae. Aust Fam Physician. 2004 Aug;33(8):621-4. [abstract]
  6. Boone H; Problems experienced by polio survivors and suggested solutions. Presentation for occupational therapists, given at the Neurological Occupation Therapy Conference, October 2008.
  7. My Polio Life; A patient questionnaire providing baseline information and comparison charts, for use by polio survivors and their health professionals, Polio Survivors Network, 2007
  8. Boone H; When test results do not match important symptoms. Conference presentation, Post Polio Association of South Florida, Miami, 2007.
  9. Hildegunn L, Jones K, Grenstad T, et al; Perceived disability, fatigue, pain and measured isometric muscle strength in patients with post-polio symptoms. Physiother Res Int. 2007 Mar;12(1):39-49. [abstract]
  10. Davidson AC, Auyeung V, Luff R, et al; Prolonged benefit in post-polio syndrome from comprehensive rehabilitation: a pilot study. Disabil Rehabil. 2009;31(4):309-17. [abstract]
  11. Polio Outreach of Washington
  12. Lambert DA, Giannouli E, Schmidt BJ; Postpolio syndrome and anesthesia. Anesthesiology. 2005 Sep;103(3):638-44. [abstract]
  13. Howard RS, Davidson C; Long term ventilation in neurogenic respiratory failure. J Neurol Neurosurg Psychiatry. 2003 Sep;74 Suppl 3:iii24-30.
  14. Lincolnshire Post-Polio Information Newsletter. Volume 5, issue 9, June 2006. Issue covering respiratory problems.
Original Author: Dr Colin TidyCurrent Version: Dr Colin TidyPeer Reviewer: Dr John Cox

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