REGISTRATIONS & MISCOMMUNICATIONS
Today ( 25 November 2017) marks 29 years since Steve and I walked into a room in Wandsworth Registry Office in South West London. And after a short but beautiful ceremony we both signed a piece of paper. A piece of paper that officially bound us as husband and wife.
Huntington’s disease (HD) was in that room that day in many shapes and forms. Not just in Steve’s father (seen in the background just behind us in the above photo), who was what I would call Middle Stage symptomatic. It was also in Steve waiting in the wings to make its appearance.
For a so-called ‘rare disease‘ I found out later, by way of the HDA Message Board , that The Registrar also had the faulty gene. I got talking to the daughter of The Registrar who mentioned her mother had worked at Wandsworth Registry Office around the time we wed. When we compared notes, she verified her mum’s signature was alongside ours on the Certificate! Was/is it also in Steve’s siblings who were present too? I hope not!
As usual by way of marking the day, I have come in to update the blog in memory of Steve and write a new poem.
My previous wedding anniversary blogs and poems can be seen here:
2012 SELF INDULGENCE
I mentioned in my blog of January 2017 that I had heard of the sad passing of one of Steve’s cousins. In September another of Steve’s cousins sadly died. He was only 44 and had also inherited the HD gene. It was his younger brother whom I had referred to in my January blog.
The funeral service was a fitting tribute for an avid fan of science fiction. From the Star Wars themed music and coffin (including a replica lightsaber placed on top complete with sound and strobing light) to the readings from books of favoured authors. This one I found particularly touching and uplifting:
“Everyone must leave something behind when he dies, my grandfather said. A child or a book or a painting or a house or a wall built or a pair of shoes made. Or a garden planted. Something your hand touched some way so your soul has somewhere to go when you die, and when people look at that tree or that flower you planted, you’re there.
It doesn’t matter what you do, he said, so long as you change something from the way it was before you touched it into something that’s like you after you take your hands away. The difference between the man who just cuts lawns and a real gardener is in the touching, he said. The lawn-cutter might just as well not have been there at all; the gardener will be there a lifetime.”
― Ray Bradbury,
Very beautiful and well chosen words indeed.
Developments since my last blog in May 2017 – ON THE WINGS OF AN EAGLE & DOVE; CARRIES FAITH, HOPE, & LOVE include:
THE PAPAL BLESSING
The Papal Blessing appears to have been a great success. They streamed the event on the day and I was pleasantly surprised at how much time The Pope took when going into the Audience and walking among the rows and rows of those invited. For some reason I had assumed it would be like you see on the television where he stands up on the balcony and addresses the masses below. Instead, it was a much more involved and personalised event. A short video giving highlights of the Event can be seen HERE.
As indicated in my last blog I was not intending to go to Rome for the Papal Blessing. However, I was thrilled to see a certain little dog (aka MiniSybil) was able to attend along with a fellow MiniSybil. They even made the BBC News! See HERE ‘Felt dog from Leeds has audience with Pope Francis’.
When Jackie and I first discussed to potential of getting MiniSybils world-wide using the #SybilOnTour branding, I don’t think either of us realised her and her team’s creations would capture the world in such an amazing way!
See HERE for a map of places where people have homed/taken MiniSybils and HERE for some of the photos sent in (including Celebrities). If you have been lucky enough to home a MiniSybil, please do send in a snap to let us know how they are doing. Contact us on Twitter via @TDainton and @jaq421 .
Sadly, there is another passing I need to raise at this point. That of Sybil herself. Jackie’s little Border Terrier who was/is the inspiration behind the design of MiniSybil.
Sybil will be missed by Jackie and her family immensely but lives on through all the joy she has brought them personally; as well as the HD Community who adopted her as a kind of mascot. We shall all miss her hats and costumes and antics stealing the show sometimes in Jackie’s home videos such as ‘The Singing Corner’ sessions.
DUTY TO WARN & OTHER LEGAL CASES
Shortly after posting my last blog the decision was announced. The full transcript of the judgement can be read HERE. In essence, it was found that there are grounds for the case to go to trial.
the Claimant’s case is arguable. I would allow the appeal, quash the Order striking out the claim, and remit the case for trial.
The legal profession is obviously taking as close interest in this case and there have been a number of blogs appraising the implications. One such blog is The GenomeEthics blog which can be read HERE
Therefore, it seems that the Court of Appeal took account of the existing evidence of professional opinion in determining what might be in the public interest in the question of whether it would be just, fair and reasonable to impose a duty to disclose. This seems to be part and parcel of the right approach to determining public interests. However, are the views of patients and the public also relevant to determining what is in the public interest here? If so, what are those views? Maybe it’s time we asked.
I am unable to get details of a date set for trial as yet. Will there even be a trial?
If I put my HD Community hat on I really hope it does progress to trial as there are a lot of issues and grey areas that need to be clarified; not just for patients and their families but for the medical and the social-care profession as a whole.
If I put my other hats on; having been a HD Carer and someone previously in management within a governmental office, I have to say I would be surprised if the Claimant and the Trust did not settle out of court if they can. This being so as to avoid the unwanted publicity and costs (for both parties even if legal aid applies) and the ethical Pandora’s box for the NHS and social care sector.
We shall see how far it gets…
Another case made the News in September involving a HD patient. This one concerned the matter of end of life care. The news report can be read HERE ‘Court ruling not needed to withdraw care, judge says’ For more in-depth details, the transcript of the judgement can be read HERE.
I still consider myself eternally grateful that I was able to keep Steve at home, and that he was able to communicate his wishes to me even at the very end. I have very mixed feelings about the above court ruling. My heart goes out to the family and it is so sad that they were put in the position of needing to go to court. It can’t have been easy either for the Treating Team.
It is becoming clear that HD is being recognised for the complex disease that it is. The laws and guidelines need to go way beyond what is already available. No doubt HD is helping pave the way for a shake up in the legal and medical system which will impact on many other diseases and medical conditions as well. By forcing questions about whether the status quo is fit for purpose.
On the last page of the 114 page Report, under Annex E – Case Reports, a HD case is used to illustrate checklists and templates are useful but cannot be interpreted as rigid. Something, no doubt, that every tribunal panel would like to think they applied anyway. Sometimes it’s not that simple though…
Of course, the case is being used to demonstrate a way of thinking which goes beyond applying to HD patients only. However, it was encouraging to see a HD case cited as a way to get people thinking again. Being in effect the closing statement and the last word as it were, it made me smile to see recognition is being given to how HD patients do not fit into simplified thinking. Just seeing the words Huntington’s disease in black and white on such a document prepared for the judicial system is fantastic awareness!
The below is extracted from the Report:
The patient was born in 1965. Like her mother and brother, she has Huntington’s disease. She was made subject to Guardianship under the Mental Health Act in 2013, and then the Court of Protection made a Standard Authorisation in 2015.
She lived in a specialist nursing home. The essence of the case was that Guardianship was no longer necessary in view of the Standard Authorisation. The Upper Tribunal found that the First-tier Tribunal had correctly recognised the importance of having the (Section 18) power to ensure that the patient returned to the nursing home if she left.
There would be practical difficulties in operating under the Mental Capacity Act 2005 to achieve this. Staff had to consider using their powers to ensure her return to the home without delay. The patient’s condition was also deteriorating and the tribunal properly referred to the crucial power under Guardianship to ensure treatment and provide access to the medical team. It was also submitted that the tribunal had failed to follow the guidance given in KD v A Borough Council and the Department of Health  UKUT 0251 (AAC).Here Charles J had set out a lengthy checklist of matters to consider in cases such as this.
Judge Jacobs in the Upper Tribunal accepted that KD was relevant, but said that it was important to recognise that the checklist was not legislation and should not be elevated to that status. As Charles J had himself said at paragraph 67 of KD, ‘it is likely to assist’. But the checklist must not be interpreted as a rigid template. Ultimately, every case is different and what matters is the substance of the tribunal’s reasoning rather than whether a tribunal’s reasons follow a particular format.
On the posthumous search for Steve’s medical notes/genetic data and his CAG Count I finally have the available records as requested back in January. The 40 working days service level agreement clearly too ambitious when the service was outsourced. I think I would have still been waiting had I not put in a strong complaint and got someone to take ownership of looking into things further.
Has getting the papers given me what I was looking for? One word, two letters, NO.
I have a plethora of letters and printouts of data on appointments; notes scribled by doctors; notes of my calling the Practice with queries; prescriptions; texts for Flu Jab reminders and copies of my letters to people (at least I now know they didn’t just throw them in the bin).
There are a number of references to ‘confirmed diagnosis’ but none that actually provide the CAG count. The nearest document is the letter sent by Sarah Tabrizi to our Doctor which can be seen below as redacted by me:
It is fair to point out, when requesting medical notes it is made clear the archives do not hold records which are the responsibility of individual hospitals to hold and maintain. By that token I was always aware I might need to directly approach the hospital where Steve’s blood was taken for testing and the Biomarkers Study. I was hoping that in the process of informing the doctor, more data would have been shared and the CAG reading included. Oh well…
The whole process of trying to get records has worn me down somewhat and I have decided to make the next step (approaching Professor Tabrizi’s hospital) something for my 2018 projects list. To be continued I dare say.
One thing I will be doing though before the end of this year is seeing my own doctor about my own health issues. I am having a forced change of medication but more about that in my next blog.
While there, I may ask my doctor to double check what they actually have on their records for me? I mention this in here as one of the things that struck me when looking through Steve’s records was the number of inaccuracies!
Steve’s main language being recorded as ‘Albanian’ for a while. I feel this is a mistake which is unacceptable in the case of someone with a proven HD diagnosis and increasing communication and cognitive difficulties.
There is no way in a million years that I or Steve would have told them this. We are talking in 2006 when I needed to attend with him to register after moving again. Therefore it looks like probable human error when adding data to the patient database. Or perhaps worse, laziness where the in-putter couldn’t be bothered to scroll past the ‘A’s option as far as ‘E’ for ‘English’.
I dread to think of how frustrating things would have been for Steve had I not been with him during appointments and treatments. Imagine my not being there and his treating team assuming his comprehension of language and use of words, when his cognitive and physical powers were compromised, could be down to English not being his first language!
When we moved again a few months later to where we finally ended up, fortunately it looks like the error was corrected as we had to re-register again. This time the right data was taken/inserted.
Such a pity no one ever thought to ask us during those last three registrations how come Steve would have gone from speaking English to Albanian overnight and then back to English? Does this suggest every re-registration involves staff having to type in all the information from scratch in the 21st Century? Qesharak!
As for my pet-hate of the use of ‘Huntington’s Chorea’ as opposed to ‘Huntington’s disease’, I was very disappointed seeing the use of the old name so often. As the above picture illustrates, chorea heads the record (E: Huntington’s chorea). The date of Jan 2004 instead of 2005 is also wrong.
Sure, HD was suspected by me in 2004 but we didn’t see a doctor until Jan 2005 about it. Therefore, technically, there was no visual diagnosis or proven diagnosis in Jan 2004. I am glad we didn’t have any insurance companies to deal with that s all I can say…
We are not just talking receptionists; doctors; and others who may not have come across the disease often if at all before. On occasion we are talking a Consultant Neurologist and members of his Team. Specialists in neurological disorders whom I would have liked to have thought were more enlightened.
I wrote a poem in my book – Curse in Verse and Much More Worse – to illustrate my passion for correcting the name. The poem was kindly read to video by Carol Royle and can be seen HERE. At the end I show the wording on Steve’s 2011 death certificate which states ‘Huntington’s Chorea’.
That name was not taken from me I hasten to add. The Registrar was very sympathetic to my frustration (she had once lived next door to a HD patient) but explained she was bound by law to enter the same wording/name as had been used on the paperwork provided by the doctor who had issued the confirmation of death for their purposes.
I hope, by now, more people in neurology and the supporting teams have been made aware of the nature of HD not just being a movement disorder. The change of name having been in place now for many years.
MY OWN AWARENESS EFFORTS
There is not really a lot to report by way of my being proactive and taking on awareness ideas. However, I was thrilled to be approached by the Scottish Huntington’s Association (SHA) a few months back and asked if I would be willing to submit something for their blog writing initiative ‘Shout Huntington’s Aloud’.
Going back into a blog I was writing at the time of being a full-time carer to Steve, I submitted a few entries and was thrilled to be featured as the inaugural ‘Guest Blogger’. My entry can be seen HERE.
I hope to work more with the SHA on future writing projects, and hopefully with the HDA too at some stage.
Also, in the last few weeks, the HDA announced a Christmas Campaign. The initiative invites people to light a bauble or star on a virtual Christmas tree for a small donation.
A special personalised dedication can be written and is displayed on a ‘tour’ around the tree. It makes very poignant reading and is a great visual representation of just how much, and how far, HD touches generations of people.
After making my own donation and dedication to Steve, the HDA asked me to help them in their social media push by providing a few words and a picture they could use. I submitted some wording and four snaps for them to choose from. As at time of writing this blog you can see the Campaign details HERE and HERE. I’m glad they chose the snap of me and Steve together enjoying life. As much as Christmas can be a sad time it is a time to reflect on happy times too.
Before I close this blog with a new poem, I just thought I would share another spooky event.
I tend to draft my blogs well in advance and update and add stuff along the way before the time comes to post. Several weeks ago I put in the opening snap of Steve signing the wedding certificate expecting it to be the only photograph available.
I was sad that I never found the one of me signing which I was sure had been taken by the wedding photographer. For the wedding album itself we chose only one signing snap. However, I was convinced I had not imagined I’d seen at least a proof version of me signing as well.
Over the years, before Steve died especially with so many moves of home etc, I have lost lots of items and photographs. Over the years, since Steve died, I have asked so many family members and friends to search for any photos they have of Steve or both of us to the point of being a nuisance!
On November 12th I met up with my sister at a cemetery to take flowers to dad. Remembrance Sunday and also it is the weekend before the 32nd anniversary of his death on November 15th.
My sister handed me an envelope containing some wedding proofs which had somehow come detached from the set I still have. She found them when looking for something else. She had been surprised to find them having thought she had given me everything by now, and I was convinced I had looked through everything when at her place over the six/nearly seven years since Steve passed!
You have probably guessed by now what was in amongst them. Yes, the snap I had been pining for even though only in my own mind as my sister was not aware I had been looking for it all these years. Or in particular for this blog entry where I was so sad I thought it was lost forever.
Make of that what you will but I’d like to think it was a posthumous anniversary present from Steve.
The photo is inserted after my poem. Thank you Sweetheart!
‘Everyone must leave something behind’ but as I look around,
No flowering plants; no works of craft; no hand-made items found.
I guess that as the years went by, where HD came to stay;
Items culled through every move meant loss along the way.
For one so gifted with your hands it’s sad there’s nothing left,
To show how you had made your mark. A home now so bereft.
But take another look around; with every single view,
The home is filled with items kept or here because of you.
The photos on the wall you took, or taken when together,
Prove that you existed then and will exist forever.
As for touching something else to help ‘your soul’ live through…
You touched my heart; my mind; my soul. I’m me because of you!
Posted on November 25, 2017, in Uncategorized and tagged Huntington's; HD; Huntington's disease; medical law; CAG; grief; The Pope; SybilOnTour;. Bookmark the permalink. Leave a comment.