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Prof Ari Zimran spoke about pain management and ozone treatment at the 5th Conference. He said that the next generation of people with Gauchers disease will hopefully not have to suffer pain from the disease.
'Patients should not need to suffer pain,' stressed Prof Zimran at the Conference. 'Pain is when a patient says that it hurts. It can come from bone crises or general bone pain, after surgery or during pregnancy and labour. Depression, anxiety, anger, lack of sleep can all lower people's pain threshold but patients should not need to have to live with pain. Doctors must try and do something about it.
'Treating pain in people with Gauchers disease can be tackled in many ways:
'The barrier to effective pain management is usually lack of knowledge and skills by the physicians and unnecessary concerns by the patients regarding the opiate group of drugs,' added Prof Zimran. 'There is fear of tolerance, addiction and dependency but this should not deter patients from getting proper treatment. In fact, these do not occur when the drugs are given for severe pain and with appropriate follow-up.
All these can be used when necessary.
Enzyme Replacement Therapy
Prof Zimran said that in the data of the ICGG (which is the largest Gaucher registry and was initiated by Genzyme in 1991 following the approval of Ceredase), there are about 300 patients who had bone pains before they began enzyme replacement therapy; 60% of them continued to have pain after one year of treatment and 50% after two years. However hopefully the level of pain will not be the same for new patients who start enzyme replacement therapy now as their symptoms will probably not be as severe at the start of treatment.
Enzyme replacement therapy as a means to treat bone pain achieves that goal better by preventing the bony complications in the first place when the drug is administered at an earlier stage of symptomatic disease.
Replacement of hips, knees and other joints may be necessary and are an effective way of relieving pain. These operations are usually performed when the patients suffer from severe pain and limitation of movement following the progression of avascular necrosis of the joints.
Long term follow up of such patients has taught us that despite the metabolic disorder which makes the bones weaker, the long term survival of the prosthese (joint replacement) is comparable and as good as in other individuals who undergo the same operations but who do not suffer from Gauchers disease.
In a recent study from our center which assessed the outcome of total hip replacement in young patients, which was published last year, it was shown that the average survival in non-revised hips (the original hip replacement) is more than eight years (the range was one to 24 years), and that all but one patient (out of 21) noted decreased pain and decreased need for pain medications after their hip replacement.
All prosthesis (replacement) types were equally well maintained with an excellent rotation, adduction and abduction (movements) in the majority of the patients; similarly, the placement was excellent with only few cases of migration of the prosthesis components.
Hopefully these operations will also be less frequent as patients start treatment earlier.
Prof Zimran described a trial with ozone therapy that he conducted with patients who still suffered from pain.
A trial of nine patients, three men and six women, took place at a private clinic in Israel as this treatment is considered alternative or compli-mentary. Their average age was 47 years. All were being treated with Cerezyme and pain medication. Altogether they received 134 ozone treatments.
The treatment requires a generator mixing oxygen and ozone. Blood was drawn from the patient into a vacuum bottle to which the ozone-oxygen mixture is bubbled and then immediately re-transfused to the patient. This treatment, known as auto-hemotherapy, was administered weekly over a 12 week period.
The patients were asked to fill in a pain questionnaire detailing average pain felt over the last 24 hours, ability to walk, conduct daily work, quality of sleep, quality of life in general and intensity of pain during the 24 hours after ozone therapy.
All nine patients reported an improvement after the first four or five weekly infusions and there was a complete or partial elimination of the use of pain drugs. The patients felt better each time.
Prof Zimran said he would like more studies into this form of therapy, especially a placebo-controlled study before one can make a medical recommendation regarding this form of treatment yet the early experience is encouraging.
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Source: Gauchers News March 2002.
© Copyright Gauchers Association 2002