3/28/2010

Post-polio Syndrome Management requires careful assessment and a multidisciplinary approach.


Post-polio Syndrome

Post-polio syndrome (PPS) is the onset of new deterioration in function that may occur many years after recovery from acute poliomyelitis. PPS comprises various possible symptoms such as fatigue, weakness, joint and muscle pain, reduced respiratory function or dysphagia. PPS does not necessarily imply progressive deterioration, and can be helped by treatment. Management requires careful assessment and a multidisciplinary approach.
Definition/diagnosis
Suggested criteria for diagnosis of PPS are:1
  • Previous history of poliomyelitis.
  • Stable period after recovery (usually > 15 years).
  • New symptoms, such as generalised fatigue, new weakness, abnormal muscle fatigue and muscle atrophy.
  • Exclusion of other cause of muscle weakness (but note that problems can be multifactorial2, as discussed below).
Note:
  • There is no universal definition of PPS, but most sources use one similar to the above.
  • Some experts suggest that PPS may be diagnosed in those where there is no clear history of paralytic polio, but where the past history or investigations suggest that previous polio is likely.3
Aetiology2
There is NO reactivation of the polio virus infection (patients may need reassurance about this).

The exact cause of PPS is unknown:
  • There may be new muscle atrophy and weakness relating to changes in motorneurones.
  • Overuse or disuse of muscles play an important role.4
  • The cause of PPS symptoms may be multifactorial. For example, disabilities resulting from acute polio, combined with activities of daily living, can produce large stresses acting on abnormal muscles and joints. This may lead to other problems which contribute to PPS, such as:
    • Joint deformities
    • Osteoporosis
    • Scoliosis
    • Cervical spondylosis
    • Peripheral nerve entrapment (can occur with callipers, crutches etc.)
Epidemiology4
It is estimated that PPS may occur in 28-75% of patients with previous polio. The time interval is usually around 35 years, but may be 8-70 years after the acute polio episode.
Presentation
Common symptoms are:4
  • Generalised fatigue
  • Joint and muscle pain
  • New muscle or joint weakness
  • Muscle atrophy
  • Cold intolerance
  • Bulbar symptoms - speech, swallowing or respiratory symptoms
  • Worsening respiratory function - may present as headaches, fatigue or sleep disorder (see below under 'Respiratory and sleep problems in post-polio syndrome').
Any of these can lead to a deterioration in day-to-day functioning. A small change in clinical terms can mean a large one in its effects on daily living.
Assessment
  • Listen to the patient's story.
  • The most important question to ask is not "can you do this activity?", e.g. climbing stairs, but "howdo you do it?" This can reveal the functional change, e.g. stopping to rest half way, going upstairs by shifting on their bottom.5
  • A patient questionnaire such as "my polio life" can be useful.6
  • Multidisciplinary assessment may be needed, e.g. involving physiotherapist, occupational therapist, neurologist, orthopaedic/orthotic team and respiratory physician.
Bear in mind that various factors can make assessment more difficult:7
  • Polio survivors are used to coping and adapting. Hence the importance of asking - and observing -how patients carry out each activity.
  • Symptoms can vary from day to day and can be affected by recent activity, overuse or rest.
  • Patients may deliberately allow for a stressful hospital visit day by resting beforehand. This can give a falsely good picture. Ask what symptoms are like "on bad days".
  • Results can appear normal if patients are given long rests between tests, or if only the best result is recorded.
  • Respiratory and sleep problems are easily missed (see below under 'Respiratory and sleep problems in post-polio syndrome').
  • Anecdotally, patients with PPS have had their symptoms dismissed after apparently normal or near-normal results of tests, such as lung function or muscle power.

Investigations

These will depend on symptoms, but could include:
  • Muscle tests - but be aware that simple tests of isometric muscle strength may be insensitive5,8
  • Respiratory investigations (see below under 'Assessment of respiratory problems')
  • Sleep studies (see below under 'Assessment of respiratory problems')
  • Swallowing studies, e.g. barium swallow
  • Investigations to exclude other causes
Differential diagnosis1
Other causes of fatigue or weakness, e.g:
Other causes of pain, e.g:
Management2
A multidisciplinary approach is helpful.9 Some important aspects of management are:
  • The correct balance of rest and exercise is essential:4,10
    • Avoid overuse, as too much exercise causes increased weakness and fatigue in damaged muscles.
    • Graded exercise is beneficial - this should probably be broken up by periods of rest.
    • Non-swimming exercise in warm water often helps.
    • Many polio survivors are used to leading active lives and, to some extent, ignoring their disability. Adapting to PPS and the need for more rest may require lifestyle and employment changes.11
  • Orthopaedic and orthotic management of skeletal problems, e.g:
    • Simple supports for knee, ankle and cervical spine can improve function.
    • Replace damaged aids.
  • Muscle pain:
  • Anaesthesia requires special considerations.12
  • Nutrition:
    • Good nutrition and weight control.
    • Some patients find that a high protein diet is helpful (e.g. the post-polio institute "hypoglycaemia diet").11
  • Specific treatment of other problems, e.g. dysphagia and respiratory and sleep problems (see below under 'Respiratory and sleep problems in post-polio syndrome').
Respiratory and sleep problems in post-polio syndrome13,14,15

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.

Aetiology14

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, e.g. COPD, asthma, obesity.
  • Aspiration - if swallowing affected.
All these are likely to worsen during sleep. The pattern of respiratory impairment may behypoventilation, obstructive sleep apnoea, or both.16

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 may be positional.
  • Weak cough, chest infections.

Signs

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 patient supine during a sniff manoeuvre. When upright, 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, e.g. 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 chest X-ray 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 intermittent positive pressure ventilation (NIV or NPPV) 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".
  • Note: 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 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.
Prognosis
  • Experience suggests that the functional deterioration does not necessarily progress.
  • Fatigue and reduction in mobility may stabilise or progress very slowly.
Prevention
Prevention of acute polio infection is discussed elsewhere (see separate article on Poliomyelitis).

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:
    • Osteoporosis.
    • Obesity.
    • Respiratory problems.

Document references
  1. Thorsteinsson G; Management of postpolio syndrome. Mayo Clin Proc. 1997 Jul;72(7):627-38.; [full text]
  2. Howard RS; Poliomyelitis and the postpolio syndrome. BMJ. 2005 Jun 4;330(7503):1314-8.
  3. Halstead LS, Silver JK; Nonparalytic polio and postpolio syndrome. Am J Phys Med Rehabil. 2000 Jan-Feb;79(1):13-8. [abstract]
  4. Khan F; Rehabilitation for postpolio sequelae. Aust Fam Physician. 2004 Aug;33(8):621-4. [abstract]
  5. Boone H. Problems experienced by polio survivors and suggested solutions. Presentation for occupational therapists, given at the Neurological Occupation Therapy Conference, October 2008. Information and slides kindly supplied by Hilary Boone (personal communication), Lincolnshire Post-Polio Network, UK.
  6. My Polio Life. A patient questionnaire providing baseline information and comparison charts, for use by polio survivors and their health professionals. Lincolnshire Post-Polio Network 2007.
  7. Boone H. When test results do not match important symptoms. Conference presentation, Post Polio Association of South Florida, Miami, 2007. Presentation slides kindly supplied by Hilary Boone (personal communication), Lincolnshire Post-Polio Network, UK.
  8. 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]
  9. 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]
  10. Agre JC; The role of exercise in the patient with post-polio syndrome. Ann N Y Acad Sci. 1995 May 25;753:321-34. [abstract]
  11. Polio Outreach of Washington. A registered charity providing information for polio survivors. Accessed June 2009.
  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.
  15. Bach JR, Tilton M. Pulmonary dysfunction and its management in post-polio patients. NeuroRehabilitation 1997;8:139-153. In Lincolnshire Post-Polio Library, copy by kind permission of Dr. Bach.
  16. Hsu AA, Staats BA; "Postpolio" sequelae and sleep-related disordered breathing. Mayo Clin Proc. 1998 Mar;73(3):216-24. [abstract]

Internet and further reading
Acknowledgements EMIS is grateful to Dr N Hartree for writing this article and to Dr Colin Tidy for earlier versions. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2009. Document ID: 2637 Document Version: 23 Document Reference: bgp2385 Last Updated: 8 Jul 2009 Planned Review: 8 Jul 2011

The authors and editors of this article are employed to create accurate and up to date content reflecting reliable research evidence, guidance and best clinical practice. They are free from anycommercial conflicts of interest. Find out more about updating.http://www.patient.co.uk/doctor/Post-Polio-Syndrome.htm





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