By: Emily and Ian Johnstone

Emily and Ian have written this story, with some minor edits by HODA Admin

My name is Emily. I am a 47 year-old mum of one daughter, Ella, and wife to a husband, Ian. We live in the old city of Oxford in the United Kingdom. Coincidentally, our neighbourhood is famous for being home to the famed children’s storytellers JRR Tolkien, CS Lewis and Lewis Carol, but also to no less than five hospitals, one of which is a centre of neuroscience and neurosurgery. The latter is very relevant to the telling of my story 🙂   

  

To start at the very beginning, in April 2013 I suffered a brain stem hemorrhagic stroke caused by a presumed cavernoma. My recovery began positively, first at the local John Radcliffe Hospital and later at home. Within two months I was speaking and walking fairly well with support. But progress was slow enough that I was referred into a rehabilitation hospital, the Oxford Centre for Enablement.

Enter HOD, right on cue

Then, in August that year, while still at the OCE, I developed a tremor, in my thumb. This then moved quite rapidly to my right arm, then leg and later in my torso. I went from walking outside, with support to needing a wheelchair.  At the same time, very peculiarly, the pitch of my voice was rising. Unbeknown to me, I was developing dystonic tremors in my larynx and a palatal tremor.  My speech worsened rapidly due to this dystonia and also dysarthria. At the same time, my  nystagmus and ataxia – caused initially by the stroke – all worsened. Instead of incrementally improving, I was rapidly getting worse.

All of this became pretty overwhelming, particularly the tremors and the loss of speech function. In November 2013 – seven months after my stroke – I was diagnosed with bi-lateral hypertrophic olivary degeneration (HOD). I was lucky in that a very experienced neurosurgeon at the local John Radcliffe Hospital took an interest in my care. I remember him drawing a diagram of the brain stem, the olives and the nerve that travels to them. He said how the bleed I had suffered caused the nerve to slowly die back and lead to the HOD. Driven by concerns about the tremors and speech, he referred me to a movement disorder clinic, again here in Oxford, where I tried various medications.

Can Deep Brain Stimulation Help? (Spoiler alert: It helped Emily)

I was also put on the list for Deep Brain Stimulation (DBS) . I had surgery in September 2015. This involved the implanting of electrodes, each in a different part of my brain and attached to a power and control unit in my chest. The DBS unit sends out rhythmical electrical signals via the electrodes in my brain, which are intended to disrupt and – as far as possible – cancel the ‘noise’ caused by the damage in my brain. The DBS team at the John Radcliffe, which by coincidence is a centre of research in the field, knew that the technology had had positive results in reducing tremors in the upper and lower limbs.

What was questionable was what impact, if any, it might have on my speech. Of course the palate is on the mid-line. So the ‘noise’ was probably coming from both sides of my brain simultaneously. So, they decided it was worth trying something rarely done: insert electrodes on both sides of the brain. Not only that, they would insert a total of four electrodes. One in the thalamus and one  in the global pallidus internus or GPi on each side of the brain. It was an audacious step, but I was so grateful for their efforts to address both the tremors and the speech. 

Emily’s Deep Brain Stimulation: Before and After

Prior to surgery I had a MRI scan under sedation in order to get a clear visualisation of my brain. Because my tremors were now quite profound, I had to be sedated in order to achieve the necessary precision. I had psychological and brain function testing, which happens both before and after the surgery so they can assess any unintended effects. The operation itself took around four hours. I was then in hospital for a week while the team carried out tests to ensure the DBS was working.

It was genuinely miraculous when they turned it on: my tremors abated almost completely and my speech seemed to improve too. I then had another general anaesthetic where they put the wires under the skin attached them to the power and control unit in my chest, which also sits under the skin below my left collarbone. The stimulator is then switched off for about 6 weeks while you recover, which is hard. To go through all that surgery, to have a brief, bright, magical taste of remission  and then to be just the same is quite a blow. But when they finally switched it on for good, it was worth it! 

Life with DBS and reduced tremors

I then went through 2-3 years of the team tweaking the DBS inputs every 8-12 weeks – with 4 electrodes with 4 contacts each there are a lot of different combinations. This can be done through the skin by placing a programming device on my chest, which speaks to the control unit. I charge the unit in the same way by placing an induction charger on my chest 2-3 days a week, normally while watching TV – it’s a breeze, actually. And, as long as I keep that up, the unit should last 25 years.

I only have 2 electrodes switched on now, the left side thalamus and GPi to combat the tremors in my right arm and leg, and the benefit is still profound. Unfortunately the four electrode, bilateral approach didn’t seem to work, and so I still have a minor palatal tremor, nystagmus and ataxia. My speech has improved slightly but whether this is as a result of the DBS or speech therapy is unknown. Both teams like to take credit! 

Emily and her continuing journey

So, the DBS has worked amazingly well on stopping the upper and lower limb tremors. Nine years on, I haven’t had any problems at all with the technology. I still use a wheelchair due to the ataxia and dystonia, but I hardly tremor, which is such a huge relief. While I know that tremors are only one symptom of HOD, to me they were a big part. I never knew what a gift it is just to be still.

So, this is my story. Not quite as weirdly wonderful as Alice in Wonderland but magical all the same!

Your Turn! Have your had any interventions that helped with your symptoms? Do you have questions about Emily or her journey with DBS? Tell us in the comments!