7. Symptom Management: Pain


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These treatments are not however a cure for Fabry Disease, and they do not completely eliminate all of the findings or symptoms of Fabry Disease, so pediatricians also need to treat the specific problems in addition to treating the global disease. So the enzyme infusion therapy that we currently have treats the underlying cause of Fabry Disease by eliminating or minimising the amount of storage and thereby minimising the damage to the cells and to the organs in the body. But, the patients who have symptoms may still require treatment for their symptoms. Pain being the key symptom in children with Fabry Disease, what should the pediatrician do for it? One approach is to note that a lot of times there are specific triggers for pain. A lot of patients with Fabry Disease will be very sensitive to either cold weather or hot weather, and they will often get pain if they push their bodies, so to make a patient with Fabry Disease go out and run a mile it is very likely they will experience pain from that. We need to avoid those triggers and allow the patients to opt out of the things we know are going to trigger pain. It is important to recognise that the pain in Fabry Disease  responds poorly to conventional analgesics so using the most common ones is a bad choice for Fabry Disease because it damages the kidneys, and this is a kidney disease. Narcotic pain medications will help in pain management in a severe crisis situation, but they are not good for long term maintenance of pain control. So the mainstays of pain management are neuroleptic medications, and there are two categories of those, the anti-seizure medications, Neurontin, Lyrica and Tegretol are the ones that are most frequently used, and antidepressant medications is the other category, and there are several of those that have been used successfully to treat, not because the patients are depressed but because it stabilises the nerves and helps to decrease the pain events that the patients experience. The drawback of the treatment of the pain with medications is that the medications have potential side effects, including fatigue, difficulty with focussing, so it is important both to not over treat, but also not to ignore pain.

 

 

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