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Most people with Gauchers disease suffer from pain sometime during their life, writes Susan Lewis It can be an acute, agonising bone crisis, often described as a heart attack in the bone, the persistent ache from damaged bones and stiff, inflamed muscles or perhaps a sharp hot pain. No doubt you can describe other types of discomfort but have you found a way to relieve, control or live with that pain apart from taking pain killers (either bought over the counter or prescribed)?
Pain must be one of the most difficult things to describe. You know you have got it but finding the correct words to explain exactly where it is and how it feels is often hard. With Gauchers disease, it can be almost anywhere in your body. Hips, back, knees, shoulders, arms, ankles; sometimes in one place, sometimes another. Some-times the pain travels down your leg or arm, sometimes you can pinpoint exactly where it is. Sometimes it lasts for ages; sometimes only for an hour or so. How do you describe it to your doctor? Especially when it may not be there at that precise moment when you are sitting in front of him or her but may reappear once youµve left the consulting room. One way is to write down a description when the pain occurs or shortly afterwards and document how often and severe it becomes.
DrugsEnzyme replacement therapy will hopefully strengthen your bones in the long run and reduce the fequency of bone crises. Possibly bisphosphonate drugs eg Fosamax (alendronate) or Didronel (etidronate) will increase the mineral density in your bones if you suffer from osteoporosis (thinning of the bone) but Prof Cox believes bisphosphonates will not be expected to have any action on bone crises or avascular necrosis (death of the bone).
So what can or do you do to relieve pain when it occurs? Pain killers (either prescription or bought over the counter) is one way although Dr Mehta advises that patients with severe pain are occasionally tempted to exceed the recommended doses of analgesics (pain killers) and they should avoid this temptation.
Non-Drug TherapiesVarious forms of non-drug therapies are described in this article but maybe you have found your own way to control your pain. If you have, let me know and I will print your experience in the next Newsletter (with your first name or anonymously if you wish). And if you have tried something that didnµt work or made things worse, please also tell me. In this way we might all be able to help each other.
Physiotherapy may be prescribed by your doctor if you complain of pain. There are physiotherapy departments in most hospitals although there may be a waiting list. You can also see a physiotherapist privately, either in their own consulting rooms or one may visit your home but this can be costly especially if you need to see them more than a couple of times.
Different physiotherapists may have different ways of treating you. Some may massage you or manipulate your bones, some may advise on a series of exercises; or a combination of both. Even if their ideas are basically the same, their gentleness of touch or their general attitude to you may vary. It may be worthwhile to try another if the one you have is not to your liking.
Rachel Morgan, a chartered physiotherapist recommends: 'Remain mobile and as flexible as possible and donµt get overtired.'
Hydrotherapy is a way of exercising your body in a warm pool, usually the water is at body temperature. You can often move your bones more easily as the water carries your weight. Again the methods and exercises may vary depending on the therapist you have. Some hospitals have hydrotherapy pools and you can ask your hospital consultant or GP to refer you.
An ordinary swimming pool may be of use but the water is often cool and if you stand still doing exercises, you may soon become chilled and this will counteract any benefit you can get.
Nevertheless swimming is an excellent form of exercise for people with Gauchers disease if they are not in severe pain and it may even dislodge a pain.
Exercise as described above, swimming is a good exercise for almost everyone, whether or not they have Gauchers disease. Ideally you should swim twice or three times a week for about 20 minutes but if you are only just beginning, take it more slowly and work up to it. Swimming strengthens your muscles without putting any weight on your bones.
Walking in the fresh air, in the park or in quiet roads, is also good for your bones and muscles. You will probably feel better for it even if you need to relax with a hot drink afterwards.
Some people with Gauchers disease may attend exercise or yoga classes. If it suits you, do it but the problem with doing exercises with other people is that you can push yourself too far to keep up. If you canµt do what they do, it may become depressing.
Hydration - Drink Fluids It is important that individuals have an adequate fluid intake and they keep themselves warm and well hydrated, advises Dr Mehta. Hydration is a good way of promoting blood flow through bone. One of the ways in which exercise, hydrotherapy, physiotherapy and simple massage help is that they promote blood flow. Drinking plenty of fluids help this. However excessive alcohol causes dehydration and should be avoided.
Passive Mobilisation in Water One member with Gauchers disease recommends passive assisted movement, preferably in a warm pool. David Katz, who practises as a manual therapist, writes: 'It is my belief that passive assisted movement allows the body to unlearn unhealthy conditioned movement patterns. With chronic injury, movement patterns evolve for many reasons such as trying to limit pain upon movement and joint damage causing movement restriction, with the result of compensatory adaptations in other areas of the body.
'I have found that as a result of bone damage, the surrounding soft tissue reacts to the condition and becomes angry and inflamed causing a reduction in the range of movement. If there is not a severe deformation of either or both joint surfaces, then regaining range of movement is a simple yet delicate process.
'Regarding passive mobilisation it is important that the person being treated should be relaxed to gain full benefit. As there is usually some pain at the site receiving treatment, a certain amount of apprehension may exist that the therapist may elicit further pain while treating; as a result I have discovered that the sessions should be carried out in warm water pools that soothe, support and warm the entire body thus enhancing the therapy.
Passive mobilisation is when a therapist holds stable the affected joint and fully supporting the limb begins to move it, mobilising the joint through its available range of movement. Gradually the range increases.'
'A TENS machine is used for pain relief. TENS stands for Tran-scutaneous Electrical Nerve Stimulation,' writes Maxine Daniels who is a member of the Association's Executive Committee and works in a Osteoporosis Clinic.
'It works on the same principle as rubbing your elbow after you have knocked it. The brain becomes aware of the touch messages reaching it, which block or lessen the pain messages getting through. The action of the TENS machine leads to the body producing more natural pain killing hormones (endorphins).
'The TENS machine has small electrode pads which are placed on the skin over the painful area. These pads are connected to a machine (about the size of a small personal stereo) which attaches to a belt around the waist. The controls of the machine are ad-justed to produce a tingling sensation which can help block out the pain messages. The sensation should not be unpleasant. By controlling the inten-sity and frequency of the pulses, you can help control your pain.
'Some people do not find the TENS machine effective in reducing pain. Others use it in combination with other pain-relieving therapies including medication, physiotherapy or hydrotherapy.
Individuals may be recommended them by their doctor or physio-therapist. The machines can be purchased or sometimes loaned from physiotherapy departments. They cost £40 upwards. It may be possible to try one for 21 days on trial.'
Challenging Arthritis Arthritis is a general term covering many skeletal conditions. Many of the bone symptoms of Gauchers disease are similar to other arthritic conditions. Challenging Arthritis is a course run by Arthritis Care to help people over 18 manage their arthritis. Their leaflet says: ?The course is always delivered by people with arthritis who have been trained by Arthritis Care. It helps make the most of the support you can get from doctors, nurses, physical therapists and occupational therapists. It introduces relaxation techniques. It helps you work out which exercises will help you. It helps you improve communications with your doctor. Above all, it is about what you can do for yourself. There is one session a week for six weeks. The session lasts about two and a half hours. There are discussions, brainstorming sessions and brief lectures. Everyone practises making weekly realistic 'contracts' with themselves to extend control over life with arthritis. You will be asked to read sections of the course book. '
The courses take place across the UK, mostly in the Autumn and Spring. The cost is £2 a week for six weeks plus £12.50 to purchase the course book entitled The Arthritis Helpbook by Kate Lorig and Jim Friers (Edition 4). The book can be bought separately from the course from Arthritis Care (see next para-graph for details) but can be obtained on loan as part of the course. Kate Lorig, who designed and developed the course, is a professor in the school of medicine at Stanford University, California, working with behavioural science and chronic disease. She also has Gauchers disease.
If you want to try one of these courses or buy the book, contact Arthritis Care, 18 Stephenson Way, London NW1 2HD, tel 0171 916 1500 ext 444. If you have attended one of these courses or intend to do so, let us know how you get on.
Will it Work for You? The therapies mentioned in this article are not recommendations but treatments which other people have experienced and some have benefitted from. They cannot be vouched for and you should consult your doctor about their appropriateness for your own condition.
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Pain Management Programmes
Source: Gauchers News July 1999
© Copyright Gauchers Association 1999