Many people suffer with Tremors. A Tremor is an unintentional rhythmic muscle movement involving oscillations of one or more parts of the body. Orthostatic Tremor (OT) is one of the rarer Tremors.
The National Tremor Foundation (the NTF) aims to promote knowledge and awareness of all types of tremors, to support people who have tremors, and to sponsor research into cures. Many (perhaps the majority) of the members of the NTF suffer from Essential Tremor (ET) or they are related to ET sufferers. But within the membership there is an OT group. It is spread across the UK, and the members either have OT or they are closely connected to someone who does.
At a meeting of this group in May 2008, it was agreed to prepare a document describing what it is like to have OT, based on a survey of the experiences of those in the Group at that time. The result was entitled Living with Orthostatic Tremor. At the time of this first survey, there were 25 members in the Group. In the intervening years, the group has grown so that now (early in 2013) there are 80 members who are suffering with OT, and another 22 who are part of the group as husbands/wives/partners/carers.
Because of this growth and the passage of time, the members decided in the autumn of 2012 to conduct a fresh survey with a view to updating Living with OT. Early in 2013, questionnaires were sent out. These were the same as before with just two additional sections. The objectives were to compare the results for the “old” members and to take account of the response from the “new” members. About two thirds of the members completed the questionnaire.
So here is a revised version of Living with OT. References to “the survey”, “the questionnaire” or “respondents” relate to the 2013 exercise survey unless indicated otherwise.
As with the first edition, there has been no input from any medically qualified person. So it remains simply an attempt to explain in lay terms what it is like for people to live with OT.
The name, Orthostatic Tremor is intended to be a literal description of what it is: a tremor which occurs when the person is standing upright. It is a rare condition characterised by rhythmic contractions that occur in the legs immediately after standing. It is that, but it is frequently much more. It is recognised as progressive, so, in a sense, this literal description is quite likely to describe the condition at the outset only. This has become more recognised recently and the term “OT plus” has been coined to describe those who have more than the basic symptoms. The term “Primary Orthostatic Tremor” (abbreviated to “POT”) is a way of describing the basic or pure condition. The “plus” element is the progression to a more disabling stage. For the purpose of this paper, the term “OT” is used as an overarching name, to include POT and OT plus. (The distinctions between POT and OT plus are developed further in the Appendix)
The main aspects of the OT experience can be summarised as:
OT varies from person to person, to some extent reflecting POT and OT plus. Moreover, individual sufferers find their own experiences changes from time to time - sometimes with a cause (e.g. stress or exertion) but sometimes without any identifiable reason. Then there are variations in the parts of the body affected. In the survey, the vast majority reported tremor in one or more parts other than their legs, i.e. hands, arms, face/head, feet or trunk. In some cases this may be ET rather than OT.
For everyone who has OT, standing, sometimes for just a few seconds, is an endurance. It appears that the tremor prevents the muscles from doing what they are supposed to do. The result is that the person feels that their legs will buckle under them. This can result in actual falling. In the survey, about two thirds reported actual falls. However, people who have OT learn how to prevent falls by avoiding open spaces, keeping close to anything (or anyone) that will support them. There may be a “freezing up” of the legs - what one respondent referred to as being “rooted to the spot”. Persistence, perhaps with support from a wall or something similar, is likely to result in pain and greater than normal fatigue later, perhaps for the next few days. Standing is (as you would expect) the single biggest problem for those who have OT. Any activities involving standing (queuing, browsing in shops, a buffet meal, etc) are at best difficult and quite likely impossible. Theoretically, the tremor and associated pain disappear when the person sits or lies down. This always brings a welcome relief, but for some there is still tremor and/or pain when at rest – sometimes linked to greater than normal exertion.
In the early stages of OT, walking may not be affected too much. The OT person may find that they walk quickly and are unable to walk slowly. But as the condition progresses, the walking distance tends to reduce. The actual distance which can be walked will vary, and at worst it is virtually none at all. One respondent stated “could still walk distances when diagnosed (but now can) barely walk at all” A clear passage is required – no people or objects in the way. But conversely, open spaces where there is no means of support are a problem. There is a feeling of stepping out into no man’s land, and a need to cross this territory as quickly as possible, ideally to reach a point where there is something to sit on. In the survey, over 80% assessed their symptoms with walking as either moderate or severe. One respondent made the comment “tremor does not completely go when walking” and another said that walking was no longer possible without pain. Just one respondent rated difficulty with walking as not experienced at all.
OT (or at least OT plus) impairs other movements. A prime example is rising from a chair – almost three quarters rated their problems with this as moderate or severe. It seems as though the process of communicating the brain’s requirements to the body is sluggish. (The medical name for this may be Akinesia). Those who have OT may be very restricted in what they can do generally. It can be a case of being almost an invalid, but with the added problems of the variations, the inherent feeling that “I ought to be able to do it”, and the pain that can result from having made the additional effort.
This is a very debilitating part of the condition. OT persons frequently suffer from extreme exhaustion or fatigue, sometimes accompanied by excessive perspiration. Basic routines such as showering/dressing can take much longer than normal and require a period of rest for recovery. Even after a day of relatively little physical activity, they may feel unable to get out of a chair, or needing sleep. Additional exertion can produce extra tremors as well as the exhaustion. There is also weakness, exacerbated by the lack of normal physical exercise.
Nearly 90% reported some pain when active and over half indicated that they have some pain when resting. This is mainly in the legs and back (although some respondents’ back pain was related to other conditions or circumstances). It is usually associated with standing or walking, in some cases exacerbated by poor posture. In addition, there may be an involuntary trying to grip the floor by screwing up the feet, which also generates pain.
It is recognised that stress exacerbates tremors. This is certainly true for OT. There is a vicious circle whereby the tremor gives rise to stress, and then the stress increases the tremor and so on. In this context, stress must be considered very broadly - it includes any unusual situation or activity -almost any emotion, can be the trigger. So anger, annoyance, concern, frustration, and even excitement or anticipation can be a problem. Particular difficulty is caused by situations such as a crowded place - nowhere to sit, exertion involved, and the risk of falling. Any crowded place is a problem – and a crowd could be just a few people if the available space is limited. The OT person appears rude if they barge into people or demand a clear path. One respondent to the first survey stated “sometimes I am so desperate I want to push people aside violently”. Use of public transport is difficult or impossible for many OT people, so getting out and about is very difficult for those without partners or carers.
The OT person experiences much frustration and dependency on spouses/partners/carers, and lack of confidence when separated from them. Simple household tasks such as cleaning, cooking, and even making a cup of tea (or carrying it to another room) are either difficult or impossible. This sense of frustration and dependency is no doubt experienced by all disabled people. But for OT people there is an added dimension in that few people can understand the problem – close relatives and they themselves struggle to understand why they are unable to do certain things. And the rarity of the condition adds to this. One respondent summed things up with these words: “I don’t think people really understand. They just think you have wobbly legs, and not all the other ailments associated with OT. Your social life becomes practically nil because you always have to worry about trying to find somewhere to sit.”
There are misconceptions about OT, particularly how it may progress from the initial aspect of not being able to stand for very long. Examples, and the realities, are:
You won’t need to use a wheelchair
You will probably need one at least for certain situations, and you are likely to need one (or a scooter) whenever you go out, and possibly when you are at home
You won’t fall
You will do all you can to avoid situations liable to cause you to fall, and you may still fall sometimes
It’s all in the mind
It’s probably all in the brain, but when the brain sends wrong signals to the body via the nervous system, there is not much you can do about it
You can do it if you make the effort
Sometimes you will exert yourself to do something despite the difficulty – but there will be a cost in terms of pain/exhaustion
There are no known cures for OT, but many people are prescribed drugs to ease the symptoms. About two thirds of respondents were taking some drugs, the main one being Clonazepam. However, Pregabalin or Lyrica may have been under-reported: it has emerged since the original survey, so it required respondents to add it to the pre-printed list. The assessed benefits of all the drugs varied from none to significant. Major side effects (such as weight gain) as well as lack of benefit were the factors resulting in many people having tried drugs but given them up. In some cases, continuation was based on an expectation (or even the experience) that withdrawal would make things worse, rather than an identified benefit.
About two thirds of respondents had tried alternative treatments. In most cases the benefits were marginal – e.g. one person referred to Aromatherapy as “pleasant but no help”.
About two thirds of those who returned the questionnaires use a wheelchair. Some find that a wheelchair is essential for going out and/or moving around at home. Others use them only occasionally – such as at airports. Just over a third of respondents use a scooter. This is down on the first survey. However, people’s needs do change as their condition develops. One respondent said that she could no longer use a scooter as she is unable to walk to it. A wheelchair can be more flexible giving greater access, for example to disabled toilets. It is also recognised as a genuine aid for disabled people, whereas scooters are viewed by some in a less favourable light.
Walking sticks appear to be of limited value, but some people use them to identify themselves as disabled. Nearly half the respondents find they benefit from portable folding seats.
There are a variety of other aids in use, including home fixtures such as grab rails and stairlifts. What one person finds helpful may be unsuitable for others, and the same individual may find that what suited at one time does not at a later stage.
Those who were doing the questionnaire for the second time were asked not to refer to the original responses. The comparison of symptoms did not show a consistent deterioration. However, this was almost certainly because the questionnaires did not provide a sufficient range of responses (no professional input and a second round not being anticipated). In addition, the number of people completing the survey for a second time was low.
All respondents said that progression since diagnosis was either moderate or severe. No one said there had been minor or no deterioration. The same question but related to the last year or so produced a wider range of responses. Even so, 80% said there had been moderate or significant deterioration in this time frame. One member who has had OT for 25 years wrote: “The condition will deteriorate and no medication really helps.” Another said “Everything I do becomes difficult”.
Just over 40% of respondents said they had ET albeit with qualifications such as “slightly” or “probably” from some people. A few had other conditions such as Restless Legs Syndrome. Some referred to conditions such as Arthritis which are perhaps not strictly related. Nearly three quarters reported Depression.
The questionnaire asked if there was anything which could be identified as having triggered the OT. In both the first and second surveys, there were references to various traumas. Some thought it was hereditary, with known other sufferers in the family. Many could not identify anything. One person referred to a particular trauma but also to symptoms going back to childhood which were not understood at the time. It seems possible that some people are born with OT and experience mild problems with standing in early life. The full blown condition then comes out later, often with some trauma being the tipping point.
There is no doubt that OT is a debilitating condition. Those who have it are, to a greater or lesser extent, unable to lead a full independent life. Their problems are exacerbated by the general lack of understanding, even on the part of health professionals.
They are helped in a variety of ways, including partners/carers (but some are on their own); mutual support from others with the same condition (including via the Internet); a determination to maintain a positive attitude; and for some a Christian faith and the benefit of prayer/attending church. There is a general feeling that the experience of living with OT should be made more widely known.
There appears to be a general acceptance that about 30% of people who have POT will go on to develop OT plus. The survey suggests that this may underestimate the true extent of OT plus. By the very fact that there is progression from one to the other (for some), a group of OT sufferers may comprise:
It would take a very clever person to divide this mythical group into these separate categories!
If you have POT you will have a problem with standing for more than a short period of time. If you have OT plus or are progressing that way, you will have the standing problem, but in addition, difficulty with walking and some or all of the following:
Unsteadiness, poor gait, gait failure, postural instability, poor walking speeds, OT tremor in arms trunk and head, difficulty rising from chairs, poor balance, problems with the dark and open spaces, exhaustion, poor mobility, shuffling of the feet, back pain and leg pain, a stoop, stress related tremor, adrenalin induced tremor, dizziness and depression. (1)
Another way of describing it is to say that if you have POT you will still be able to live a reasonably normal life. The standing problem will be a nuisance, and you may use a wheelchair or scooter occasionally such as at airports; but you will probably not consider yourself disabled. But if you have OT plus you will, most likely:
(1) From Dave Watts website (www.orthostatictremor.co.uk)