Sunday 29 September 2019

Neurologist David Blacker on locked-in syndrome

 "I knew it was vital to get through to him, and make him understand there was hope" 
        
          Many of you may already be acquainted with Peter Coghlan, whose book "In the Blink of an Eye Reborn" charts his incredible recovery from locked-in syndrome against the odds.* His determined fight for freedom not only inspires many stroke and brain injury survivors, but also helps medical professionals understand this rare and frightening condition from a patient's point of view. 
         For the benefit of those involved in the treatment and rehabilitation of LIS, here is the foreword to the book provided by Pete's Neurologist - Clinical Professor David Blacker, MBBS, RACP.  


Professor David Blacker
          "I’ve been involved with the care of stroke patients for more than 20 years and have witnessed the full range of human emotions, tragedies and triumphs, experienced by stroke survivors and their loved ones. I have frequently shared in the grief, frustration and pain, and still find the task of delivering bad news distressing. There are however, some shining moments of glorious and spectacular recovery; Peter’s story is one of them. So often the media describes a patient’s recovery as “against all odds”, with the doctors pessimistically quoted as saying, “You’ll never walk again,” or “You will need to go to a nursing home.” Whilst some reports of Peter’s story have included such phrases, they were never from me; in fact, my interactions with Peter have always been the exact opposite. 
Peter was admitted to the ward in mid-2011. I hadn’t been involved directly with his initial care, but was soon aware of his circumstances and what had happened. I had long been a proponent of mechanical thrombectomy for severe stroke due to clotting within the large arteries of the brain, and have been involved with this therapy since the mid-1990s. It took until 2015 for this approach to be finally proven as a technique, so attempts to use it prior to that date were certainly regarded as ‘cutting edge’, or possible a little ‘cowboyish’. In situations like Peter’s, though, the decision-making is relatively simple - a very fit, active young patient facing death or severe disability. In my view, there was no hesitation in trying everything. Other clinicians might have been more conservative, particularly prior to the publication of the 2015 data, for fear of leaving Peter alive and facing devastating disability. In any case, Peter was transferred from a peripheral hospital in the middle of the night, to the major tertiary hospital, and one of interventional neuroradiology fellows, in conjunction with my colleague, made some urgent decisions, and proceeded to mechanically extract a lengthy clot from Peter’s basilar artery during the early hours of the morning. 
In this book, Peter describes in graphic detail the horror of awakening in the intensive care unit, and the gradual realization of his situation; the ‘locked-in syndrome’ (LIS). In summary, individuals with LIS find their fully active mind, trapped in a completely paralysed body, usually due to a stroke affecting the middle section of the brainstem; a critical section of neural tissue connecting the brain to the upper spinal cord. Typically, all four limbs are paralysed, and the nerves controlling speech, swallowing and breathing are affected. The patient is unable to speak, and observers may assume they are comatose and not aware of the surrounding environment. Communication can sometimes be established through eye-blinking, and other subtle facial movements.  One of the most famous cases is described in the book, and movie, “The Diving Bell and the Butterfly”. 
Shortly after he was transferred to the neurology ward, I was formally asked to consult on his case, particularly to assess his suitability for transfer to the neurology ward of the rehabilitation hospital, where I also worked.  Despite seeing hundreds of stroke patients each year, I had only been involved with four cases of locked-in syndrome (who had survived) due to stroke before I met Peter; but this was more than most of my colleagues. Two early patients remain highly dependent, one in a nursing home, the other with full time family care at home. Another patient had been spectacularly saved by thrombectomy more than 24 hours after symptom onset, and progressed through rehabilitation quite rapidly. I presented his case at a meeting to highlight what could be done. I contrasted his outcome with another patient who didn’t do so well initially, but ultimately underwent a long rehabilitation process with some gains, and returned home, and resumed a productive life. She came tantalisingly close to walking but, due to a number of issues, couldn’t quite get back on her feet. Around the time that Peter came in, I was feeling particularly enthusiastic about neuroplasticity and rehabilitation. The famous book written by Norman Doige, ‘The Brain That Changes Itself’, was receiving a lot of popular press, and I’d just heard him give a public lecture, affirming many of the basic principles that I’d been working with for years. Although it wasn’t really new knowledge for me, it was inspiring and exciting for the public and patients to have some hope provided. In the book, Doige describes the recovery process of a doctor’s father who suffered a severe brainstem stroke. That inspirational description included a phase where the patient crawled like an infant in the process of re-learning to walk. I felt “fired up’ by these descriptions and eager to apply this the next time I had a suitable patient with LIS.  
When I carefully reviewed the details of Peter’s case, I was quietly optimistic. His MRI showed that the damage to a critical portion of the brainstem known as the pons was incomplete. An important few millimeters of tissue known as the corticospinal tract seemed to be relatively intact. This region is the ‘wiring’ which transmits electrical signals from the brain to the body. It’s incredible what difference a few millimeters of damage, one way or the other, can make. What I saw on the scan gave me hope, but what I saw in Peter when I met and examined him gave me even more hope. I saw a fit, relatively young man who was determined. I knew it was vital to get through to him, and make him understand there was hope, and I tried to do this when I met him. I was surprised to later read in his first book, how great the impact of my decision to get him into the rehabilitation hospital was; from my side of the bed there was no doubt that was the only thing to do. It’s easy to forget how the things doctors say and do really stand out in the minds of patients. I had to be realistic though, and warn him there was plenty of work to do, and that it would be a long road. 
So Peter’s rehabilitation journey began. Typically I’d visit the rehabilitation hospital once or twice a week and attend a detailed meeting where the nurses and the therapy staff would give their reports on patients’ progress.  The highlight for me was seeing patients in normal clothes in the gymnasium working with the physiotherapists, rather than being stuck in a bed wearing pyjamas. I think that environment is crucial because it can be very inspiring and motivating, as well as tough. Patients get to see others in action as well, at different stages in rehabilitation, and with different levels of age and disability. Sometimes a sense of friendly competition arises; other times, disappointment can be the result. The team clinical psychologist has a crucial role to help guide patients and their relatives through the “bumps in the road”, that the long journey of rehabilitation must take when a patient has profound neurological injury like the LIS. Peter certainly had ‘bumps’ to face, including at least two frightening episodes of pneumonia.  But Peter overcame the challenges to walk out of ‘Shents’. The video of that still makes me cry! 
There was much more to follow for Peter after discharge from inpatient rehabilitation; in some ways the intensity of his efforts accelerated. Around this time I was quite enthusiastic about long distance running, and a member of the West Australian Marathon Club. Another member named Daryl Howel has cerebral palsy, and has famously competed in marathons and ultra-marathons around the world. This made me wonder what Peter could do, and somewhere along the line I challenged him to join me in the Perth City to Surf, initially planning to participate in the 4km walk. Peter’s determination of course quickly changed the target to the full 12km event. It was a wet and cold day when Peter and I completed the course, which was being deconstructed as we slowly covered the distance, but a glorious achievement and milestone for Peter. A couple of years later we went even further, walking 16km in the John Hughes Big Walk. On a beautiful clear day I think Peter could have walked forever! 
There are many other things that Peter has done including his generous support and help for other patients, and there are many more great things in his future. Peter’s inspiration also helps me show up for work every day. Stroke medicine is demanding. Most days I interact with patients and families who are going through the worst thing that has ever happened to them. The stress, grief and shock associated with stroke is enormous, and I admit that despite all my years of working in the field it still sometimes overwhelms me and gets me down. When it does though, there is no better way for me to find inspiration than to think of Peter Coghlan."  

Professor David Blacker  

*Available from Amazon

US: 

UK: