Post-polio syndrome medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Treatment generally is limited to supportive measures, primarily leg braces and energy-saving devices such as powered wheelchairs, plus pain relievers, sleep aids, etc.

Medical Therapy

Treatment for post-polio is primarily palliative, as no reliable therapy to reverse symptoms is known. Palliative treatment includes:

Very often fatigue is the most disabling symptom of PPS, and many of those with the disease have discovered that by carefully managing energy expenditure they can prevent or reduce the worst fatigue episodes. Further, for many this "energy management" approach appears to reduce pain. Though most authorities agree that rest is an important component of post-polio treatment, there is significant disagreement as to how much rest is necessary. Some hold that the best approach is to expend the absolute minimum amount of energy necessary to enjoy a reasonable lifestyle, while others feel that there is some threshold below which energy conservation is not helpful and may in fact be harmful (due to the general effects caused by lack of exercise).

The treatment for post-polio syndrome is generally palliative and consists of rest, analgesia (pain relief) and utilisation of mechanisms to make life easier such as powered wheelchairs. There are no reversive therapies. Fatigue is usually the most disabling symptom; energy conservation can significantly reduce fatigue episodes. Such conservation can be achieved with lifestyle changes, reducing workload and daytime sleeping. Weight loss is also recommended if patients are obese. In some cases, the use of lower limb orthotics can reduce energy usage. Medications for fatigue, such as amantadine and pyridostigmine,[1][2] have not been found to be effective in the management of PPS. Muscle strength and endurance training are more important in managing the symptoms of PPS than the ability to perform long aerobic activity. Management should focus on treatments such as hydrotherapy and developing other routines that encourage strength but do not affect fatigue levels. The recent trend is towards use of intravenous immunoglobulin (IVIG)which has yielded promising, albeit modest results.[3]

PPS increases the stress on the musculoskeletal system due to increasing muscular atrophy. A recent study showed that in a review of 539 PPS patients, 80% reported pain in muscles and joints and 87% only had fatigue.[4] Joint instability can cause significant pain in individuals with PPS should be adequately treated with painkillers. Supervised activity programs, decreasing mechanical stress with braces and adaptive equipment is recommended.[5]

Because PPS can fatigue facial muscles, as well as cause dysphagia (difficulty swallowing), dysarthria (difficulty speaking) or aphonia (inability to produce speech), persons with PPS may become malnourished due to difficulty eating. Compensatory routines can help relieve these symptoms such as eating smaller portions at a time and sitting down whilst eating.[6] PPS with respiratory involvement requires special management such as breathing exercises, chest percussion with a stethoscope on regular occasions for observation of the disease and management of secretions. Failure to properly assess PPS with respiratory involvement can increase the risk of missing aspiration pneumonia (an infection of the lower respiratory tract) in an individual. Severe cases may require permanent ventilation or tracheostomy. Sleep apnoea may also occur. Other management strategies that may lead to improvement include smoking cessation, treatment of other respiratory diseases and vaccination against respiratory infections such as influenza.

Leg braces and other orthotics can reduce the stress on joints and, in some cases, muscles, and so may slow the progression of joint and muscle damage related to PPS. However, some authorities feel that many PPS patients rely on such items too much and for too long when they should be graduating to a wheelchair. Wheelchairs (particularly powered wheelchairs) and "scooters" (small battery-powered vehicles) are useful both to conserve energy and to reduce the stress on weakened joints and muscles. Non-powered wheelchairs, however, are not generally recommended since they place too much stress on arm muscles and joints and may take too much energy to operate. In some cases even the scooters are not recommended since operating the "tiller" of the typical scooter can be tiring to arm muscles. A standing frame can be used in conjunction with the wheelchair to provide alternative positioning and prevent secondary complications.

Post-polio syndrome often causes significant levels of pain, sometimes in specific muscles or joints, and sometimes body-wide. Various forms of narcotic and non-narcotic pain-relievers, muscle relaxants, tranquilizers, and sleep medications may help to deal with the pain and related sleep problems. In some cases surgery can be used to repair joint deformities, or to fuse joints (as in the back or ankle) that have become too weak.

Very few non-palliative treatments for post-polio syndrome have shown any promise, and none have been subjected to any sort of rigorous clinical testing. There are, however, a few treatments that have developed some "following" in the PPS community:

The amino acid, L-carnitine has several functions in the body, one of the most important being the transport of fatty acids into the mitochondria. Researchers in Australia have had some success using doses of several grams per day.

Coenzyme Q10is a general antioxidant, but it also plays a critical role in the function of the mitochondria, transporting electrons between the "complexes" that participate in the energy conversion cycle. A shortage of CoQ10 can cause the fatigue and muscle pain much like that experienced with PPS. Some PPS patients have reported significant improvements in their symptoms when taking several hundred milligrams of CoQ10 per day.

The pentose sugar D-ribose is the "R" in RNA and a critical component of DNA, RNA, and enzymes. It is also a component of ATP, the energy-transporting molecule produced by the mitochondria. A shortage of D-ribose can produce fatigue and muscle pain. Some PPS patients have reported significant improvement in pain and fatigue symptoms when taking on the order of 20 grams/day of D-ribose.

References

  1. Horemans, HL (2003 Dec). "Pyridostigmine in postpolio syndrome: no decline in fatigue and limited functional improvement". Journal of neurology, neurosurgery, and psychiatry. 74 (12): 1655–61. PMC 1757426. PMID 14638885. Unknown parameter |coauthors= ignored (help); Check date values in: |date= (help); |access-date= requires |url= (help)
  2. Trojan, DA (12 October 1999). "A multicenter, randomized, double-blinded trial of pyridostigmine in postpolio syndrome". Neurology. 53 (6): 1225–33. PMID 10522877. Unknown parameter |coauthors= ignored (help); |access-date= requires |url= (help)
  3. Farbu E (2010). "Update on current and emerging treatment options for post-polio syndrome". Ther Clin Risk Manag. 6: 307–13. doi:10.2147/TCRM.S4440. PMC 2909497. PMID 20668713.
  4. Ehde DM, Jensen MP, Engel JM, Turner JA, Hoffman AJ, Cardenas DD (2003). "Chronic pain secondary to disability: a review". Clin J Pain. 19 (1): 3–17. doi:10.1097/00002508-200301000-00002. PMID 12514452. Retrieved 24 December 2008.
  5. Jubelt B, Agre JC (2000). "Characteristics and management of postpolio syndrome". JAMA. 284 (4): 412–4. doi:10.1001/jama.284.4.412. PMID 10904484. Retrieved 24 December 2008. Unknown parameter |month= ignored (help)
  6. Silbergleit AK, Waring WP, Sullivan MJ, Maynard FM (1991). "Evaluation, treatment, and follow-up results of post polio patients with dysphagia". Otolaryngol Head Neck Surg. 104 (3): 333–8. PMID 1902934. Unknown parameter |month= ignored (help); |access-date= requires |url= (help)




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