Locked In Syndrome
April 23rd 2008 03:29
A while ago I wrote an article on this website about one of my favourite actors from the 1980s, Jon Blake (real name Paul) who suffered a terrible accident after filming his final movie, leaving him with an acquired brain injury.
This time of year, with ANZAC Day almost upon us, I tend to think about Paul and his accident, often digging out my DVD of the Australian miniseries, ANZACS, and watching it for the umpteenth time. In the series, Paul’s character, Flanagan, joined the Australian Infantry as a Gallipoli reinforcement following the first major battle of the campaign and Flanagan is actually based on a real person from that time.
Anyway, this wasn’t supposed to be a post about ANZACS, Paul’s accident or ANZAC Day. The reason I’m here today is to write about the condition I understand that Paul is suffering from. It’s a rare condition called Locked In Syndrome which many people may have actually heard of but not really known much about.
Locked In Syndrome is a neurological disorder where the patient suffers complete or near-complete paralysis of all voluntary muscles in their body. The only muscles the patient remains in control of are those which determine eye movement, allowing the sufferer to communicate in a limited way through blinking. It has been described as being the “closest thing to being buried alive”.
The patient can’t walk or talk, are incontinent and many need to be fed through a tube, however they remain remarkably aware of their surroundings, being able to think and reason, with no loss of cognitive function, and enjoy many of the things they used to before the condition occurred, such as watching TV and listening to music.
Locked In Syndrome is caused by damage to specific portions of the lower part of the brain and brainstem, distinguishing it from “persistent vegetative state” where the upper part of the brain which is damaged. Common causes of this rare condition include stroke, brain haemorrhage, diseases of the circulatory system, diseases which destroy the myelin sheath surrounding nerve cells, medication overdose or traumatic brain injury, which Paul suffered following his devastating car accident.
There currently isn’t a cure for Locked In Syndrome or any standard treatment, although therapy called Neuromuscular Stimulation can be helpful. This uses electrodes to stimulate muscle reflexes. Full recovery just doesn’t happen although some patients can regain some minor muscle control over time.
One famous sufferer of the condition was French journalist Jean-Dominique Bauby who experienced a massive stroke, leaving him only able to communicate by blinking his left eyelid. Otherwise he was totally paralysed and unable to talk. The Diving Bell and the Butterfly is a book written by Bauby following his stroke, dictated to a scribe using blinks from his left eye. The scribe recited the alphabet in order of highest frequency letters (E S A R I N T U L etc) until Bauby’s blink indicated which letter would come next. Each word took an average of two minutes to blink out and he had to blink around 200,000 times to complete the book.
This amazing publication details Bauby’s life following the stroke and what it’s like to be trapped inside a unresponsive body. The book was published in March 1997, selling 150,000 copies in the first week. Two days after the book was published, Bauby died.
For another story detailing the experiences of a sufferer of Locked In Syndrome see this article from the British Medical Journal. Nick’s story gives a small insight into this condition for non-sufferers. Really Long Link
I can’t claim to be an expert on this subject, in fact I can’t even claim to know any more than the average layperson. However, I wanted to raise awareness of the condition with a hope that research will eventually find a cure or, at least, ease the suffering of patients struggling with Locked In Syndrome. Any condition that can be described as being akin to being buried alive is worthy of attention and sufferers are definitely worthy of our thoughts.
At this time, my thoughts go out to Paul and his family and friends, particularly his son, Dustin, who has had to take control of his father’s care since the death of Paul’s mother, Mascot, mid last year.
Jon Blake in character
This time of year, with ANZAC Day almost upon us, I tend to think about Paul and his accident, often digging out my DVD of the Australian miniseries, ANZACS, and watching it for the umpteenth time. In the series, Paul’s character, Flanagan, joined the Australian Infantry as a Gallipoli reinforcement following the first major battle of the campaign and Flanagan is actually based on a real person from that time.
Anyway, this wasn’t supposed to be a post about ANZACS, Paul’s accident or ANZAC Day. The reason I’m here today is to write about the condition I understand that Paul is suffering from. It’s a rare condition called Locked In Syndrome which many people may have actually heard of but not really known much about.
Locked In Syndrome is a neurological disorder where the patient suffers complete or near-complete paralysis of all voluntary muscles in their body. The only muscles the patient remains in control of are those which determine eye movement, allowing the sufferer to communicate in a limited way through blinking. It has been described as being the “closest thing to being buried alive”.
The patient can’t walk or talk, are incontinent and many need to be fed through a tube, however they remain remarkably aware of their surroundings, being able to think and reason, with no loss of cognitive function, and enjoy many of the things they used to before the condition occurred, such as watching TV and listening to music.
Locked In Syndrome is caused by damage to specific portions of the lower part of the brain and brainstem, distinguishing it from “persistent vegetative state” where the upper part of the brain which is damaged. Common causes of this rare condition include stroke, brain haemorrhage, diseases of the circulatory system, diseases which destroy the myelin sheath surrounding nerve cells, medication overdose or traumatic brain injury, which Paul suffered following his devastating car accident.
There currently isn’t a cure for Locked In Syndrome or any standard treatment, although therapy called Neuromuscular Stimulation can be helpful. This uses electrodes to stimulate muscle reflexes. Full recovery just doesn’t happen although some patients can regain some minor muscle control over time.
One famous sufferer of the condition was French journalist Jean-Dominique Bauby who experienced a massive stroke, leaving him only able to communicate by blinking his left eyelid. Otherwise he was totally paralysed and unable to talk. The Diving Bell and the Butterfly is a book written by Bauby following his stroke, dictated to a scribe using blinks from his left eye. The scribe recited the alphabet in order of highest frequency letters (E S A R I N T U L etc) until Bauby’s blink indicated which letter would come next. Each word took an average of two minutes to blink out and he had to blink around 200,000 times to complete the book.
This amazing publication details Bauby’s life following the stroke and what it’s like to be trapped inside a unresponsive body. The book was published in March 1997, selling 150,000 copies in the first week. Two days after the book was published, Bauby died.
For another story detailing the experiences of a sufferer of Locked In Syndrome see this article from the British Medical Journal. Nick’s story gives a small insight into this condition for non-sufferers. Really Long Link
I can’t claim to be an expert on this subject, in fact I can’t even claim to know any more than the average layperson. However, I wanted to raise awareness of the condition with a hope that research will eventually find a cure or, at least, ease the suffering of patients struggling with Locked In Syndrome. Any condition that can be described as being akin to being buried alive is worthy of attention and sufferers are definitely worthy of our thoughts.
At this time, my thoughts go out to Paul and his family and friends, particularly his son, Dustin, who has had to take control of his father’s care since the death of Paul’s mother, Mascot, mid last year.
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Comment by Head Injury / Traumatic Brain Injury/ Locked in syndrome Pakistan
Comment by Andrea
Diet and Health
V8 Supercar Pitstop
My thoughts are with you regarding your other son, Ubaid Pasha. No parent should ever have the heart-breaking task of burying a child, however old that child is.
Stay strong and I wish you all the best.
A.
Comment by Head Injury / Traumatic Brain Injury/ Locked in syndrome Pakistan
Perhaps someone of you have seen Indian movies.Many of them show a criminal gang or mafia, we are here in Pakistan practically facing them.It is another part of my story but i can not write more or identifying them because i am not in position to say but assure you that still struggling.I request you all to pray for me. Anwer Kamal Pasha
Comment by Andrea
Diet and Health
V8 Supercar Pitstop
A.