Why did you or family decide that DBS was the treatment option for the family member?
Our daughter has a very rare progressive form of Dystonia, early onset primary torsion generalized Dystonia. She was pretty much stable until the age of nine but from then on it was a constant battle to help her manage the effects of her form of Dystonia. She had an Intrathecal Baclofen pump implanted (which delivers the drug Baclofen through a tiny tube that is surgically placed in the spine) when she was 12 which for 18 months did help to alleviate the symptoms and we then managed her dystonia with a combination of various drugs and therapy but by the time she had turned 14, it was clear that we were not managing and that her Dystonia was progressing at a fast rate.
She was losing weight rapidly and she could no longer eat or sit up to eat, so she needed to be fed by a Percutaneous Endoscopic Gastrostomy (PEG) a soft tube placed into a hole in the stomach by a surgeon. She was in constant pain, her body twisting and locking into painful postures. We never knew from day to day what would happen and we spent many, many weeks rushing to Emergency and then in hospital trying to manage the pain and constant movements. By the time DBS was suggested we knew that it was her only hope for a life free from some of the effects of her Dystonia.
What needed to be considered before DBS was provided?
We did have to undergo several drug trials to see if anything would make a difference for her that would control her Dystonia without surgery. She was tested to see if she had a genetic form of Dystonia and she went through a number of examinations by her Doctors who wanted to be sure that DBS was the correct surgery for her, but most importantly we all had to fully understand about DBS.
We all needed to understand that this was going to involve a commitment for us all for the rest of her life and that it could not be taken lightly. It was not a cure, there were no guarantees that it would work and that it was not going to completely “fix” everything, and we need to be clear that with Dystonia it can take up to three years before the full effect of DBS is seen.
We also needed to know that this was going to involve a life style change for all of us, but particularly our daughter, because she was going to need to be “charged” regularly and she would need to make sure she tested the battery implanted in her chest every day. We were going to have to be aware of the repercussions or issues if the stimulation was turned off accidently as she would instantly go into a dystonic storm if this occurred. She had to be prepared that she was going to have to spend time every week “recharging” as she has a rechargeable device implanted. She also needed to know that she would need to be careful, that she could “break” things such as the leads if she fell.
Did you need to prepare for the surgery? Any lifestyle changes?
Apart from reading and researching quietly ourselves, as much as we could about DBS to understand the procedure and the risks that she was facing from it, we really couldn’t prepare as such for the surgery.
By the time our daughter had her surgery we had been fortunate or lucky to be well educated about what to expect on the day of surgery and after, and her team of Doctors had made sure we were very clear about the operation itself, the risks and the outcome. It perhaps helped that she was still a child so there was an extra degree of care around us as her carers to help us understand.
We knew that she would be asleep for the operation, which can be done for Dystonia, unlike DBS for Parkinson’s, but life was not good for her and we were all very much living life on a day to day basis, never knowing what would happen next. Having had a form of early onset Dystonia, DBS had always been considered as an option so I think for us in our case, there had been very long and extensive discussions over a very long time that enabled us to be very prepared for what we were about to begin. She had lost a lot of weight and so we did have to wait for another 6 months to try and get her to a better weight but in the end we all knew that this was not going to be possible while her Dystonia was continuing the path that it was, so it was decided that she needed the surgery sooner rather than later.
What did you expect the benefits would be?
To be totally honest, we went into the DBS with very low expectations because there were no guarantees that it would even work. As a family we decided that we would set our goals low so that we didn’t experience disappointment if things did not live up to the hype. Yes we had seen the “miracle” cures, where people could walk again for example, but we really didn’t know how true these stories were or what the background of each person’s story or dystonia was so we decided to keep things realistic and aim for a reduction in the pain she was going through daily. After that anything else was a bonus.
Is long-term management or maintenance required?
We know that we must keep the battery in her chest fully charged and at the moment its life is only 10 years before it will need to be replaced, so we are all aware that she will need another operation to do this in the future. Initially you do spend a lot of time with your Neurologist as they adjust the settings to find an optimum level. For some this is a very slow process and a gradual wind up, for others and we were in this category, it was a faster process, with the settings being changed often while we worked out what suited her best.
Other than that DBS does not need much management apart from regular charging, but if we are unsure about anything we have great support from Medtronics who are always at the end of the phone to check with.
From a rehabilitation point of view, it has been an interesting experience because there is not a lot of experience in Australia, in the rehabilitation of someone who has had DBS for Dystonia. It has been a case of live and learn and sometimes we have pushed her too far and had to back off as she hurt herself. We needed to understand that there were many muscles that had been switched on and overused for a long time, but there were also just as many that had been inactive and now these needed to be identified as those overactive muscles had done all the work for them and they had spent many years in the background not working. We also had to learn patience and that she needed to take the time to relearn all the tasks that she could not do and not to rush things because every time we rushed her, we would experience a setback.
As a Carer how has it affected you or family members?
Our daughter’s Dystonia had progressed to a stage that she was no longer able to do anything for herself and required 24 hour intensive care. We were all hoping that DBS would be the answer for her and fortunately she has had remarkable or noticeable results from it, which has surprised everyone. She is now back happily enjoying life, not completely free from her dystonia, but certainly 80% better than she was prior to it. She now laughs again, enjoys going out with us, is back at school full time for the first time in 4 years and is pain free. It certainly has improved our life as a family together and we are able to think of a future that she is a part of again. We have no idea if she will ever regain her ability to walk again but we are certainly more optimistic about what lies ahead. Having been through the journey we did with our daughter’s Dystonia, we all now embrace the day, enjoying what we have now and we don’t try to look back too much at what was.
Are there any other aspects of the DBS Journey that you feel a carer or people should know?
I do believe that everyone should go into this with a very clear understanding that it is not a cure, and that in some cases it may only give limited results or no results at all. It is still not understood fully why DBS works as it does, so there is always an element of chance with this treatment, and I believe so long as you understand that, then this is certainly an option worth considering if you are thought to be a candidate by your Neurologist. Only your Neurologist can really guide you on this, and it is important that you do listen to what they are saying with regard to the success rates and also the risks, because as with every operation there are always risks. After all, as it was said to us, this is Brain surgery.
ADSG would like to thank the Carer who has agreed to share their story.
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