We are almost there, however…[Post Polio Syndrome]

India has been taken off the polio endemic list by the World Health Organization this February. That leaves only Pakistan, Afghanistan and Nigeria as polio endemic countries. We are almost there as far as polio eradication is concerned. However, there are around 12-20 million polio survivors living in the world today. And a bitter and largely unknown fact is that decades after recovery from polio, 60% have the risk of developing new muscle weakness and other symptoms that can lead to increased debility. These new symptoms are referred to as Post Polio Syndrome or PPS in our medical jargon. PPS affects not only “paralytic” polio survivors, but also unknown numbers of patients who had subclinical polio. The pathophysiology behind PPS is not fully understood and the latest research suggests that our reliance on pharmacological agents is of no value in PPS. 

I quote verbatim from an article (Update on current and emerging treatment options for post-polio syndrome): 

“Post-polio syndrome (PPS) refers to the clinical deterioration experienced by many polio survivors several decades after their acute illness. The symptoms are new muscle weakness, decreased muscle endurance, fatigue, muscle pain, joint pain, cold intolerance, and this typical clinical entity is reported from different parts of the world.

The pathophysiology behind PPS is not fully understood, but a combination of distal degeneration of enlarged motor units caused by increased metabolic demands and the normal aging process, in addition to inflammatory mechanisms, are thought to be involved. There is no diagnostic test for PPS, and the diagnosis is based on a proper clinical workup where all other possible explanations for the new symptoms are ruled out. The basic principle of management of PPS lies in physical activity, individually tailored training programs, and lifestyle modification. Muscle weakness and muscle pain may be helped with specific training programs, in which training in warm water seems to be particularly helpful. Properly fitted orthoses can improve the biomechanical movement pattern and be energy-saving. Fatigue can be relieved with lifestyle changes, assistive devices, and training programs. Respiratory insufficiency can be controlled with noninvasive respiratory aids including biphasic positive pressure ventilators. 

Pharmacologic agents like prednisone, amantadine, pyridostigmine, and coenzyme Q10 are of no benefit in PPS. Intravenous immunoglobulin (IVIG) has been tried in three studies, all having positive results. IVIG could probably be a therapeutic alternative, but the potential benefit is modest, and some important questions are still unanswered, in particular to which patients this treatment is useful, the dose, and the therapeutic interval [1].” 

The United States National Institute of Neurological Disorders and Stroke (NINDS) lists the following Criteria for diagnosis of PPS [2] 

• Prior paralytic poliomyelitis with evidence of motor neuron loss (which can be confirmed through a typical patient history, a neurological examination and, if needed, an EMG examination).
• A period of partial or complete functional recovery after acute paralytic polio, followed by an interval (usually ≥ 15 years) of stable neuromuscular function.
• Gradual onset of progressive and persistent new muscle weakness or abnormal muscle fatigability, with or without generalized fatigue, muscle atrophy, or muscle and joint pain.
• Symptoms that persist for at least a year.
• Exclusion of other neuromuscular, medical, and orthopaedic problems as causes of symptoms.

One of the most common questions polio survivors ask is, “How should I exercise?” This has been much debated [3]. General guidelines for patients are: 

• Maintain an active exercise program to avoid deconditioning and cardiovascular sequelae
• Avoid overly aggressive exercise (fatiguing)
• Resist the impulse to exercise through pain. 

Muscle fibers of polio survivors have very limited endurance because of the loss of aerobic enzyme activity and greater reliance on anaerobic metabolic capacity [4]. Cross-training programs, such as alternating cycling with swimming and walking, is a good way to involve different muscle groups, but such programs should be consistent in terms of repetitions, resistance, and time. Swimming is beneficial but it is also a form of strenuous exercise. If moving out of a wheelchair/assistive device to the pool involve tiring efforts than swimming should be avoided. Conservation and judicious use of the energy is the key in PPS. For most people, using daily activities as a primary way to exercise is too erratic and may lead to overuse, fatigue, and further weakness. We must evaluate our daily routine as so many times because of work pressure we ignore our body. 

Thirty one years ago, in October 1981, some 250 health care providers and polio survivors held an international symposium in Chicago to consider this question: What ever happened to the polio patient? [5]. In a summary of the proceedings of that conference the editors note “Those survivors - the former generation that pioneered the advances – seem to be ‘prematurely ageing,’ and no one really understands why.” Thirty one years later we know the name of this new collection of symptoms but the pathophysiology and management is largely skeptical. PPS is difficult to diagnose since the symptoms of presentation are usually non-specific. 


The awareness on PPS is still very poor, at least in India. Globally also, literature is incorrect when it comes to World Polio Day. I disclosed it in my previous blog post how erroneously Jonas Salk’s birthday is correlated on 24th October. Today is again 24th October and we in India celebrate Dussehra. Let’s burn the effigy of ignorance on this auspicious occasion and spread awareness on PPS. Today is also a day when devotees worship Goddess Shakti who represents strength, ability and courage. On this day, the Devas joined their energies into Shakti, a single mass of incandescent energy to kill the devil Mahishasura. Let’s explore our infinite abilities, and work collectively in killing the demon of ignorance, that is, PPS. 


We (polio survivors) aren't dead yet. Are we? 

Also read : World Polio Day - 24th or 28th October?

Polio survivor's fight to correct date of Salk's b'day

References: 

1. Farbu E. Update on current and emerging treatment options for post-polio syndrome. Ther Clin Risk Manag. 2010 Jul 21;6:307-13.
2. Post-Polio Syndrome: Identifying Best Practices in Diagnosis & Care. March of Dimes, 2001
3. Silver JK, Aiello DD. What internists need to know about postpolio syndrome. Cleve Clin J Med. 2002 Sep;69(9):704-6, 709-12.
4. Silver JK, Aiello DD. Bone density and fracture risk in male polio survivors. Arch Phys Med Rehabil 2001; 82:1329.
5. http://www.post-polio.org/edu/pphnews/PPH27-4fall11p3.pdf retrieved on 24 Oct 2012