Wednesday, September 2, 2009

Dr Betty Dowsett - progress report and Happy Birthday!

Vivienne Mills, from the Colchester ME Support Group, recently visited Dr Betty Dowsett in her Nursing Home.   
Vivienne reports that Betty looked a lot better than when she last saw her at Easter time this year and took her for a trip round the grounds of the Home in her wheelchair.
On 19th September it will be Betty's birthday and we hope that some people who know Betty will want to send her send a card. Vivienne has kindly offered to collect and take the cards to Betty at the Nursing Home.
Cards can be sent to Betty via Vivienne Mills at:  Mrs V Mills, 24 Hills Crescent, Prettygate, Colchester, CO3 4NU.
Dr. Dowsett is responsible for the following useful advocacy information:
Excerpts from Dr. Dowsett's analysis:
[during outbreaks of polio, ME patients in the surrounding areas, weren't prone to polio. ~jvr] 
WHAT ARE THE LATE EFFECTS OF ME?  Most doctors with substantial experience of examining these patients would agree that the outlook for any individual is unpredictable. Case records need to be kept up to date for prolonged periods because patients who have remained clinically stable over 40 years or more and have worked normally for most of their lives are still subject to significant late effects. These include: overwhelming fatigue both physical and mental; cognitive disturbances; muscular and joint pain; muscular weakness and wasting; difficulty with breathing; episodes of hypothermia and low blood pressure; problems with swallowing and voice production as well as sudden attacks of breathlessness while sleeping. The similarities of these symptoms to those complained of by sufferers from the Post-polio syndrome, is striking and requires further explanation.
Successful immunisation against only 3 polio viruses among some 69 enteroviruses currently in circulation is deemed to have solved all problems!
WHAT IS THE POST-POLIO SYNDROME? [5,6,7]  Poliomyelitis is an acute enteroviral infection with a wide range of clinical manifestations and multi-organ involvement (a fact which was frequently overlooked by physicians dealing with large numbers of dangerously paralysed patients, between 1940 and 1950). 95% of people who contract the infection remain symptom free or suffer only a trivial non-specific respiratory or gastrointestinal illness as in ME.  Some 5% of those contracting the minor illness develop muscle weakness or paralysis before more serious or fatal complications supervene. The diagnostic distinction between "paralytic" and "non-paralytic polio" was entirely arbitrary in the days of the big epidemics. In fact, the category of "non-paralytic polio" contained many patients with mild or temporary paralysis and with encephalitis, which occurs in patients reaching the later stages of this illness. Modern studies indicate that overt paralysis in these patients depends entirely on the percentage of spinal nerve cells destroyed. For damage to be visible as weakness or paralysis at least 50%-60% of the nerves controlling muscular action must be damaged or destroyed. Thus, patients with less damage who may only have had a minor illness, and some who were asymptomatic can still present many years later with a classic Post-polio syndrome.  Recent publication[6,8] of this information (originally derived from studies made in 1955) has resulted in a re-definition of the post-polio syndrome and will certainly include many patients currently seen in ME clinics. 
IS IT POSSIBLE THAT MANY PATIENTS DIAGNOSED AS HAVING ME ARE SUFFERERS FROM AN ILLNESS CLINICALLY IDENTICAL TO "NON-PARALYTIC" POLIO? [6,8]  Yes, undoubtedly! This is an important question with fundamental implications for further research into the diagnosis, treatment and prevention of both disabilities.  Modern research published currently in a dedicated supplement of the American Journal of Physical and Medical Rehabilitation by the Editor and 3 leading research teams[6,8], indicates that part of the current difficulty in obtaining a clear diagnosis of the post-polio syndrome lies in the error of dividing acute poliomyelitis into "paralytic", "non-paralytic", "abortive" and "sub clinical" categories. It has to be recognised that there is a wide range of nerve damage in every patient. The Post-polio syndrome may therefore include:  a. Patients whose nervous system damage was not clinically obvious at the time of diagnosis.  b. Those who had minimal paralysis for a short period and were misdiagnosed as non-paralytic polio.  c. Those patients suffering from infection due to non polio enteroviruses with potential to cause nervous system damage and the "Post polio" syndrome, equal to that of polio viruses e.g. Coxsackie viruses A9, A7; Coxsackie B viruses 1-6; ECHO virus 9; Enteroviruses 70, 71 - all of which have been implicated in outbreaks of ME or epidemics clinically identical to paralytic poliomyelitis.  d. Patients with symptoms clinically identical to the Post-polio syndrome whose nerve damage arises from some other cause, for example, local muscle problems due to metabolic dysfunction, the effects of persistent virus infection, immune reaction to fragments of viral genetic material etc.  It is essential that patients with clinical symptoms suggestive of Post-polio syndrome should be referred to a Physician to exclude other nervous diseases (eg, Motor Neurone Disease), and especially those which are treatable. 
IS IT NECESSARY TO DIFFERENTIATE BETWEEN THE LATE EFFECTS OF ME AND THE POST-POLIO SYNDROME? [8,9,10]  Not really, even if it were useful or practicable to do so at present, as the two conditions are clinically identical and similar in respect of neuroanatomical, neuroendocrine, neuropsychological electroencephalographic and other techniques, including brain imaging and molecular biology, as indicated by a remarkable series of research papers published by Bruno and colleagues over the past 20 years.
HOW MAY SYMPTOMS OF THE LATE EFFECTS OF ME AND POLIO BE EXPLAINED? [9,10]  It has to be accepted that some degree of encephalitis has occurred in all these cases and that the areas chiefly affected include the upper spinal motor and sensory nerve roots and the spinal nerve networks traversing the adjacent brain stem (a nerve centre controlling all vital bodily functions which is always damaged). The most troublesome symptoms of both conditions are progressive muscle weakness, fatigue and pain, and the commonest cause of relapse over use of repaired nerve networks and an inappropriate response to physical or mental stress in combination with the increasing effect of normal aging.  FATIGUE:  This is almost always central and due to damage affecting the Reticular activating system (which keeps the brain awake and alert as well as maintaining some control over muscular activity). Fatigue is characteristically intermittent, but profound and incapacitating and related even to minor activity. [14]MUSCLE WEAKNESS AND WASTING:  This may have a central cause (as above) or a local origin due to loss of motor units controlling individual muscles (including the breakdown of repair to these over time). Metabolic, immune or ongoing viral injury to muscle fibres, are other possibilities where infection persists.  PAIN:  This is a severe symptom which is difficult to treat and is usually due to dysfunction of the thalamus, an important sensory relay station in the brain stem. Failure to produce natural painkillers (e.g. endorphins and encephalins), may be an additional factor.  INAPPROPRIATE REACTION TO PHYSICAL OR MENTAL STRESS:  This also arises from injury to the brain stem which normally controls the production of cortisol (a steroid required for stress control) via the hypothalamus, pituitary and adrenal glands . In the absence of an efficient response, even minor stress can cause catastrophic collapse in these patients.
NB. Because of the many and varied symptoms arising from encephalitic damage to the brain, all symptoms reported, however bizarre they may seem, must be taken as possible evidence of organic disease.
MANAGEMENT:  Despite promising reports from the USA of anti-enteroviral agents[18], and of Dopamine receptor agonists[9] (to correct some deficiencies in neurotransmission) no specific medical treatment is yet available in the UK and the main principles of management rely upon conservation of energy, reduction of stress, and simplification of manual tasks at home or at work. These objectives cannot possibly be achieved without financial and social support, aids to mobility, house conversions and suitable rehabilitation facilities. In the USA it is claimed that (with counselling, if necessary, for those who find such adjustments to life style difficult) 91% of patients will stabilise in view of the fact that, at this stage, the disability is only slowly progressive. Patients have to be cautious about drugs, especially those acting on the central nervous system including psycho-active preparations and alcohol. In general, these patients need less anaesthetic but higher doses of pain killers than usual and more time to convalesce from surgery. There are now many new options for muscle problems including modern orthoses and corrective surgery. 
Some Immediate Steps that Could Be Taken[7,17,18,19,20]. These patients could be referred to NHS rehabilitation clinics and welfare facilities as for any other chronic neurological disease but physiotherapy must include exercise suitable for patients with some damaged muscle fibres which have been overused while others are normal and liable to deconditioning[7]. Separate "ME" and "Post-polio Clinics" are more expensive and often inaccessible. We should be educating doctors and paramedics now about the very common and seriously disabling effects of neglect
Research workers must be encouraged and appropriately funded to work in this field. However they should first be directed to papers published before 1988, the time at which all specialised experience about poliomyelitis and associated infections seem to have vanished mysteriously![11,12,13]  

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