When Quinns ended up in hospital last year caught up in a dystonic (choreic) storm nothing was working to break him out of it. We had exhausted all our strategies at home and everything the doctors tried also failed. He ended up heavily sedated to control the movements and on a ventilator to keep him breathing. He had no quality of life. The only option left to try was described as ‘routine brain surgery’.

After being flown to the Evelina hospital in London he was fully assessed, and with his new diagnosis of GNA01, was deemed an appropriate candidate for a Deep Brain Stimulator (DBS). Although far from routine for children, DBS has been used for adults with movement disorders like Parkinson’s for a number of years. 

Usually candidates spend about a year preparing for the device to be fitted. They undergo assessments to monitor involuntary movements so that changes can be tracked after the DBS is fitted. For children the surgery is carried out under general anaesthetic. A week or so after surgery the DBS device is turned on. Changes to the electrical pulses are then made slowly with frequent trips to hospital.

In Quinns’ situation speed was of the essence. There was no time for any preparation. Two small electrodes were implanted into his brain sending electrical impulses to the part of his brain that controls his movements. The device was turned on immediately with the simple aim to break the dystonic crisis and get him out of intensive care. 

Thankfully it worked. His movements lessened allowing the levels of sedation to come down and finally he made it off the ventilator. But what does having a Deep Brain Stimulator mean for him now?

To keep the device working well we need to remember to charge it every day. The electrodes are connected to and powered by a small battery pack which is implanted in his tummy. We hold a charging device to the battery pack in his tummy for about 15 minutes. It is a straightforward process that has quickly become part of his evening routine.

Like someone with a pacemaker he will need to alert airport security to the presence of his device when he travels and he will need to have any MRI scans in a designated DBS centre. He should also avoid activities that risk dislodging the probes or damaging the wires.

He will need to make regular trips to the Evelina in London for check ups and for any adjustments to be made to the device. The DBS should last about 25 years and will hopefully help prevent any future storms.

While he has been recovering, as well as his body being a bit stiller, we have also seen slight differences in his hand function compared to before the surgery. Although the initial aim was to rescue him from the dystonic crisis it is hoped that further improvements can be made to his movements by tweaking the electrical pulses. 

This far from routine brain surgery has not only saved Quinns’ life but it may also improve his quality of life.