THE FAIRNESS OF AWARENESS
Today (14th May 2015) would have been Steve’s 54th birthday. I wanted to write a post to commemorate Steve as I have done in previous years. In fact, my first WordPress post was written on 14 May 2011.
The first posts were not so much about Steve, but about the book I had just self-published – Curse in Verse and Much More Worse. Even if this were to be my last blog entry, I’m glad I carried on with adding posts as it is lovely to have a kind of diary – come – archive covering the years after Steve’s transition. Not forgetting Steve in the process but remembering our life together and celebrating him!
This is a very long post so the chances are people will switch off before getting to the snaps of Steve at the end (and my new poem too). On that basis I am popping in a couple of snaps early. It’s Steve’s party and he can smile if he wants to!
This year, Steve’s birthday falls slap bang in the middle of Huntington’s disease Awareness Week (11th – 17th May).
I haven’t done much this year for Awareness Week in terms of trying to get publicity for HD. I think I reached my peak in 2013 by blagging my way onto a local radio show which I blogged about HERE and HERE.
I have, however, done a series of adapted snaps to put out through the Week via social media.
Today’s offering is below the photo of me and Steve on his Birthday in 1998.
The photo to the left was taken a few years earlier on a trip to Bournemouth. Both show Steve’s lovely smile which seems to slant upwards to the left cheek, making it dimple more.
The photo shown is part of my #TrustMeImaRuby series (Trust Me I’m a Ruby and my own jokey take of the phrase “Trust me, I’m a doctor”). The others can be seen as part of an awareness slideshow I created which can be seen HERE.
Ruby (right) and Ruby-Tuesday (left) are two of the ‘cats’ featured in my poem ‘The Jealousy’. I figured people have a thing about cute cats (me included) so I would get a better response if showing them rather than my own ugly mug. I also featured them in my video for Pie in The Face Day aka PITF. See the video HERE.
PITF was a call to arms for HD awareness by Sarah Parker Foster who is a fellow blogger. More about Sarah’s blogging later on.
Since my last blog, I attended a screening of the film ‘Do you really want to know?’ which was screened at the Genesis Cinema in London. The screening was organised by Genetic Alliance UK and the audience was made up mainly of students with an interest in genetics.
A Guest Panel was also in attendance comprising of Charles Sabine; Rachel Jeffares who is, SWAN UK Representative, and Georgina Hall, Consultant Genetic Counsellor from Manchester Centre for Genomic Medicine.
Following the screening there was the opportunity to discuss the film and the implications of clinical research and testing with with the Guest Panel . The implications covered many areas; not just those raised in the case of the film and ‘rare’ conditions. It also raised the ramifications of the recent commercialisation of making genetic testing available to the general public by companies such as 23andMe. In case you were wondering… HD is one of the health conditions not covered by 23andMe’s Personal Genome Service.
As previously mentioned, Charles Sabine was on the Panel. I was thrilled to be able to finally meet him in person and give him a signed copy of my book. Charles gets a mention in my book under the poems ‘The Owl and the Ostrich’ and ‘The APPG’.
I’ve added a couple of photos below although my photos do leave a lot to be desired. I took along Steve’s old (non-digital) Canon SureShot. I kind of like to think he would be pleased I still use it. I suspect even with a digital camera though I still would have been rubbish at getting the framing right! I hope people can make out Charles is holding a copy of the book lol.
That makes my collection up to two HD heroes now as I have the snap of Jimmy Pollard with my book as well in my Blog Dedication Not Medication. That said… anyone who actually bought the book is a STAR so if anyone reading this blog wants to send in a photo with the book I would love to add you in here!
For those wanting to buy a copy of Do you really want to know? you may have seen that the DVD is currently only available for sale in the USA and Canada. However, since the screening it has appeared in full on YouTube.
I’m not sure whether it was put on there by the people connected with distribution. It may therefore be taken off at some stage but, in the meantime, I have added a link HERE as it really does need to be seen more widely.
It can make tough viewing in places but there are a lot of messages of hope in it too! The message of hope is also reinforced in the following video which Charles presents as part of a recent CHDI Conference.
It seems there has never been a better time to sign up for becoming involved in research. Whilst a ‘cure’ may still be a long way off, it is still key to address the individual symptoms within the illness in order to make living with HD as manageable as possible. This is both for those with the illness/at risk and also their loved ones and carers.
If you have read my May 2013 Blog – One dose of Tweetment or two? you will already have seen I put up a ridiculously long list of medication or treatments that had been mentioned on the HDA Message Board as being prescribed in connection with those being treated for HD.
Some might argue there are too many drugs on the market already and, as my ‘The Cocktail’ poem points out, the interactions with other meds/substances in the body needs to be considered.
However, what an amazing thing the CHDI and also Enroll-HD are doing for the HD Community insofar as all the research is focused on HD! By that token surely there is far greater likelihood of the drugs/treatments not only being more effective, but those working in the field will have a better idea of the real issues those with HD have in the first place. That must surely help filter out more the ‘hit and miss’ approach which we seem to have at the moment?
To put it another way…
I went into NHS Choices recently. I put in ‘Huntington’s disease’ in the search part on ‘Browse medicines by condition’ to see what recommended medication is currently available. See HERE for the result. Text reproduced below for those who can’t access the links:
Medicines A-Z – Medicines for Huntington’s disease
Over-the-counter medicine. Medicine with this icon can be bought without a prescription.
Now it’s fair to say more is covered in the NHS Choices webpage that covers Huntington’s disease, but as you can see there is only one drug (Tetrabenazine) listed in the medications by condition section.
It makes it even sadder that the description says :
Information specific to: Tetrabenazine 25mg tablets when used in Huntington’s chorea.
Tetrabenazine (tet-ra-ben-az-een) is a medicine which is used in huntington’s chorea.
The information in this Medicine Guide for Tetrabenazine varies according to the condition being treated and the particular preparation used.
Tetrabenazine is used to treat Huntington’s chorea. It works by depleting certain chemicals in the brain which cause the abnormal movements. As the activity of these chemicals is reduced, Tetrabenazine can help control jerky, irregular and uncontrollable movements in people with chorea.
I would have felt happier had I seen our NHS had enough enlightenment of the illness to call it ‘Huntington’s disease’. Or were they to use the phrasing differently. For example ‘used in treating chorea’ or ’used in treating chorea within those with Huntington’s disease’. As it stands, it suggests the name is still Huntington’s chorea.
Anyone who has read my poem ‘The Name Game’ will be well aware that I have an issue with the medical profession and the Health Service not looking beyond the chorea aspects of the illness and seeing it is far more complex! No excuse for the NHS website to be using such poor wording on an easily reviewed and update-able website in May 2015.
So that was what they have for HD. One drug for a thing they call Huntington’s chorea. Surely similar diseases like Parkinson’s will sadly be just as misunderstood and misrepresented? But then I remembered there is so much more awareness of Parkinson’s.
I put in ‘Parkinson’s disease’ on the same website and got THIS Reproduced below:
Medicines A-Z – Medicines for Parkinson’s disease
Over-the-counter medicine. Medicine with this icon can be bought without a prescription.
- Amantadine hydrochloride
- (a brand ofApomorphine Hydrochloride)
- Apomorphine hydrochloride
- (a brand ofProcyclidine Hydrochloride)
- (a brand ofRasagiline Mesilate)
- (a brand ofCabergoline)
- (a brand ofCarbidopa Monohydrate/Levodopa)
- (a generic version ofStalevo)
- (a brand ofBromocriptine Mesilate)
- Pramipexole dihydrochloride monohydrate
- Procyclidine hydrochloride
l could be wrong here but did anyone spot the difference? [Inserts eye rolls at this point and hopes the reader hasn’t come down with finger rsi having scrolled down so far…]
Chances are some of those meds can work for HD too. Indeed, some are listed in One dose of Tweetment or two? as having been prescribed for HD. I would therefore never suggest they are not an option just because they don’t come under the HD label. God knows we have to be grateful for anything which can help.
However, wouldn’t it be WONDERFUL if a set of new drugs could be discovered? Drugs which the doctors and others could refer to for those who walk into their clinics with HD, in the knowledge that they have been tried and tested for that condition in particular?
This being the case rather than our loved ones simply being given treatments which were developed with other conditions or patient groups in mind, perhaps actually less complex than HD? Tetrabenazine is the only one on the HD list to date. Can this mean no one has confidence enough to expand that list to other suitable drugs? And by that token, acknowledging the wider aspects of HD such as depression; psychosis; saliva control at later stages etc.
If developed with HD at the forefront, the drugs could be more effective; less contradictions may apply, and ultimately branded for the disease providing more medical awareness and recognition. This won’t happen though if drugs can’t get out in the public domain in the first place. To do that there needs to be human testing on those who would be most likely to show real outcomes and, dare I say, side-effects.
I appreciate not everyone may be lucky enough, or even suitable enough, to get on a research programme. Of course, not everyone is willing to participate even if asked to take part.
For our own part when Steve and I first went to Queen Square, to the National Hospital for Neurology and Neurosurgery, we were asked to be part of Dr Sarah Tabrizi’s study on Biomarkers. Steve did not want to be part of it. In fact, he was so upset just being in the hospital that he said he didn’t want to go back at all.
In the end he agreed to be seen at another hospital which was more local and didn’t have Neurology in the title as such. Although we were booked in with the ‘HD Multidisciplinary Clinic’ of that hospital I had to make out it was just a normal clinic. Steve wouldn’t have agreed to be there at all bless him had he been aware the majority of those around us were there for HD too.
I often wonder if Steve would have been here with me now had I coerced him into being part of research, or put my foot down and made him go to The National who were, and are, a Centre of Excellence in the field of HD. That would have given us more access to current research and trials and maybe more HD specific monitoring and care.
That’s not to say we didn’t receive good care in the main through the services we used, it’s just that the non-specialist hospital may not have had as much HD knowledge and kudos. I wanted the best for Steve if that was possible, but not at the expense of terrifying him in the process.
I took the view that I still had to respect Steve’s wishes. His body; His mind; His life and ultimately His death. The decision was His and that is something we had agreed way down the line before symptoms started. If he got HD I would remember that. It wasn’t easy at times as you can imagine.
No one should feel an obligation to be a ‘guinea pig’. That’s not what Charles; Jeff Carroll ( in the Do you really want to know? film) or anyone else is saying. It has always got to be down to personal choice. To have a choice though you need to be aware of what the choices are in the first place. It’s about THE FAIRNESS OF AWARENESS.
Going back to the subject of films, it has been very exciting to see there are more films and documentaries being made about HD. They will serve to help those caught up in the disease understand it better for their own perspective and those of their carers; advocates and researchers.
The carers; advocates and researchers, in turn, get to see the other side of what it might mean to have HD or be at risk. Of course, such films and documentaries will also serve to educate a wider audience such as the general public!
You can see the trailer for The Inheritance HERE.
Another HD related film, which is being critically acclaimed, is Lucy Walker’s ‘The Lion’s Mouth Opens’. In fact Lucy’s film had been short-listed for Best Documentary Short, Academy Awards 2015.
You can see the trailer for The Lion’s Mouth Opens HERE
Interestingly it was only a few years ago (2009), at another CHDI conference, that it was predicted one day there would be a film receiving an Academy Award covering the subject of HD.
Julie Friedman, who is a filmanthropist, gave the CHDI Keynote Speaker’s presentation in Cannes. She presented to a room full of scientists in a most remarkable and personal way.
You can see Julie’s Cannes presentation HERE.
If you watched the video you may have been surprised as I was at how many films and shows have been made over the years which feature HD. Maybe someone ought to make a list?
I mentioned Sarah Parker Foster at the beginning of this rather long blog. Sarah has produced a fantastic website/blog which is helping to list all the films; book; social media resources and a whole pile of other amazing and useful stuff which will be of so much help to the HD community.
Sarah has the HD gene herself. She is symptomatic so it takes an enormous amount of energy and concentration to blog and construct her work. Take a look at the site ‘Sarah’s HD List: aggregated, curated, and updated information about Huntington’s Disease all in one place’ HERE.
As you will see, there is an incredible amount of work that has gone into it! Sarah invites us all to make use of the information and also to help by way of adding data information and sources, or helping up-date data/correct misinformation as things move on so quickly in the world of Huntington’s.
Lisa is the writer of the renowned and best-selling book Still Alice, The book went on to be made into the award winning film starring Julienne Moore who plays a linguistics professor diagnosed with Early-Onset Alzheimer’s disease.
Inside the O’Brien’s is a wonderful book and beautifully written. Lisa uses a family in Boston to throw light on how HD is a family disease. It delves into the myriad of physical; emotional; social and even financial issues which families with HD in their lives, or the threat of it, can have thrust upon them.
It is clear from the book that Lisa went beyond just drawing on her own neurological background knowledge where she has a degree in Biopsychology and a Ph.D. in Neuroscience from Harvard University. She obviously immersed herself in the challenges and experiences of the characters.
It therefore came as no surprise to read about Lisa’s help from, and direct involvement with, some of the members of the HD community in one of Sarah’s blogs. See Sarah’s blog covering some of the background to the book HERE.
In the book the central character – Joe – hears of his test results on St Patrick’s Day. It was St Patrick;s Day ( 17th March 2005) that Steve and I walked out of the National Hospital for Neurology and Neurosurgery having had it confirmed through his blood test Steve’s symptoms were that of Huntington’s disease.
I mentioned above how he refused to go back there. Blood was taken for the Biomarkers. I also gave blood for the study as part of the control group. Steve’s tenseness and jerkiness at the time meant he didn’t have such a good experience with having blood drawn for the research.
I could have continued on my own for the control group readings.The thing is,it kind of felt like I wanted to reject the place in solidarity with Steve. He would have felt I was colluding with the enemy had I gone back.
I remember I kept telling Steve we would still be going for our usual St Patrick’s Day Guinness, and that we had even more of an excuse now! That wasn’t a nod to drowning our sorrows. We were brought up in the age when there was still the notion that Guinness was good for the blood.
As strange as it would seem we were too far gone down the HD line to have been in a state of shock at hearing the news we had just been given. If anything, I think had the blood test come back as negative we would have been in a state of shock wondering what on earth had been happening to us in the last few years?
Actually, it’s fairer to say ‘I’ would have been in a state of shock as Steve would have been more inclined to be relieved,and believe I was making everything up and acting over cautiously or sensitively.
Sadly it wasn’t just in my head. Steve’s father had HD and we had been aware of the risk of 50/50. Various other relatives had succumbed to HD so it was natural to put two and two together. The denial for Steve still conveniently came into play along the way however.
That said, I later came to wonder how much of Steve’s denial was simply being misinterpreted or misunderstood? I came to think much later on it could be more sadly a symptom called ‘organic denial‘ as Dr Jane Paulsen describes it in A Physician’s Guide to the Management of Huntington Disease (see more below); or anosognosia as Lisa refers to in her book. Like many others, I didn’t even know there was such a thing until fairly recently.
It is important to distinguish unawareness in HD from the more familiar kind of denial in order to avoid thinking of people with HD as suffering from a purely psychological problem. Unawareness in HD is thought to result from a disruption of the pathways between the frontal regions and the basal ganglia. It is sometimes called “organic denial,” or anosognosia, and is a condition that may last a lifetime.
Organic denial can be confusing to health professionals, friends, and family members who may interpret the individual’s unawareness of symptoms as a wilful decision to ignore what is known to be true, when the reality is that the individual simply does not perceive that what is happening to him or her represents symptoms of a disease.
It may be that the person can talk about problems she is having, such as memory problems, involuntary movements or issues with balance, but not acknowledge that he or she has HD. In such a case, the focus should be on mitigating the individual symptoms without repeatedly confronting the issue of the underlying diagnosis.
Where there is noncompliance with therapy or nursing care because of unawareness, it may be useful to develop a contract that creates incentives for compliance while sidestepping discussion of the diagnosis.
Anyway…Going back to that fateful St Patrick’s Day… We went to a pub not too far away from the Neuro Hospital and were the only ones in it. It was a lovely sunny day but we sat inside at the far end. The pub was virtually empty but it still felt like we wanted to hide in the corner.
Steve was very quiet, of course, but the music came on in the background and we started talking about the songs; and made other chit chat about stuff and I agreed he didn’t have to go back to the place if he didn’t want to. I felt so totally sorry for him bless him.
A couple of pints later and we were making our way home via the delights of a packed underground and packed train on a warm March early evening. Steve was wobbly anyway from HD. I have a slight limp from my scoliosis (not to mention the gammy right foot). On top of that, being only 4 foot 6 inches tall other pedestrians mistake me for the invisible woman at times and barge right into me. Steve had been my counter-weight and support anyway (in so many ways) over years alcohol or no alcohol permitting. We made a fine pair at the best of times.
Lisa’s book made me cry in places but it also made me smile too. I read this particular part and couldn’t help but smile. Steve was my best friend and I was his and we would stagger along through life physically and metaphorically speaking.
“Come with me. Let’s get you a cuppa tea”
Rosie acquiesces. They walk together, listing and pitching, bumping off the hallway walls and each other’s hips. A drunk wife and a husband with HD. They make a fine pair. As they lurch down the hallway and finally make it to the kitchen, it occurs to Joe that this is the best anyone can hope for in life. Someone you love to stagger through the hard times with.
It reminded me of what I was trying to convey in my poem ‘The Best Is Yet To Be’:
‘Grow old along with me,
The best is yet to be’;
It’s clear when Browning wrote those lines,
He hadn’t met HD!
Meant to retire together;
Married same age as me;
We both envisaged a company clock,
And a bungalow by the sea!
In our forties and home together;
O.A.P.s we’ve become before time.
We cannot do ‘fun’, or hols in the sun;
Our mates they are all in their prime.
But there’s one thing we will have forever,
And our friends will one day come to see,
That we may not have grown old together,
But our time was the best it could be.
Between March 2005 and Jan 2011 we still managed to pack in a lot of living and I’d like to think a lot of loving each other too.
Seeing my dodgy camera skills when I got the photos back from the developers, and looking at the snaps of Charles , I didn’t berate myself for being rubbish at taking photos. They made me smile as they captured Charles looking dapper, bubbly and happy. Just like he is in many of the videos I have seen over the years.
I have a whole host of snaps of Steve displayed around the room. Some are very well taken though I say so myself. Others leave a lot to be desired. Looking beyond the imperfections of my poor camera skills, and seeing the perfections in Steve’s wonderful smile is all that matters. Those lovely blue eyes and that dimpling of the left cheek where Steve’s smile always seemed to rise more to the left than the right. That’s what I see now rather than how poorly framed Steve was in the lens. That’s what matters more!
The beguiling/smiling eyes and dimples.
Looking at your photos now,
I don’t see imperfection.
Twinkling eyes and lovely smiles,
Stand out in your reflection.
With a smile from ear to ear;
Subtle smiles included,
Show an inner sense of cheer.
Cheeky grins from long ago,
And some from time much nearer;
All bring back your sense of fun,
And make your story clearer.
Many of us see two letters;
‘HD’ stands for illness.
Use another meaning,
And the letters stand for stillness.
High of Definition,
Is another way of seeing,
Clarity of vision,
And a better way of being.
Interference now stripped out,
And clarity of view,
Makes it even easier,
To see you were still you.
The above pictures include:
- Taken by me circa 1994 at our flat in Erith
- Birthday meal in Greenwich (Cafe Rouge) 2007
- Taken by me at the flat in Greenwich just before going to the 2007 Birthday meal
- Taken at a Halloween Party in 1987. Steve went as Count Dracula. For a laugh, during the evening he put on our friend’s ‘Wizard’ wig and let me take a photo
- Taken at the start of our Funchal Toboggan Ride. Cruise excursion May 2006. The video hyper-link on the name is not ours I hasten to add.
- Taken on Llandudno Pier in 2007.
I tend to draft my posts well in advance of an event date and add bits as I go along. Hence the length of some of them. In drafting this one I was wondering about whether I should cut out some of the snaps of Steve I had planned to put in?
Then I thought [insert your own expletive here] IT! I want to share information about HD but the blogging, especially on days like today, is about Steve and in his memory first and foremost!
Then, as if to say I shouldn’t hold back, a few days ago I got a private message in FaceBook. The person wrote:
Trish, I randomly came across the YouTube video you made for Steve and it brought me to tears. Since the song was “In your eyes” I made sure that I looked into his eyes for each picture, and I know he was a very special man. You keep parts of him alive to share with others and it is an honor to have a glimpse at who he was.
I was very touched by that message. It made me even more determined to share my photos and videos of Steve. People may still scan through quickly if they bother to read the blog at all. At least I am still keeping Steve alive and central to my thoughts and research for the blog. Sending me a message again? Bless him… He is my inspiration for all that matters after all. I’m sure he’s still looking out for me.
Accepting all I’ve done and said
I want to stand and stare again
‘Til there’s nothing left out, oh whoa whoa
It remains there in your eyes
Whatever comes and goes
Oh, it’s in your eyes