One Mans’ Trash…

A lot has happened since I last wrote an entry for my blog.  I had been consented into a clinical trial for haemophilia gene therapy and I was going to keep you updated regarding progress.  Well, the update is that there was no progress.  After being consented by my consultant haematologist, I was required to go through a set of health checks.  This included having a blood test to check for antibodies to the viral vector being used in the study, an ECG (heart tracing), and a fibroscan (a liver test which uses ultrasound).  The results were not what I expected.  My fibroscan showed that I have stage 1 fibrosis of the liver, which knocked me a little; my ECG showed features of left ventricular hypertrophy (an enlarged heart), which knocked me a lot; and my blood test showed that I have antibodies to the viral vector being used in the clinical trial.  The results of the blood test meant that I was immediately excluded from the clinical trial.  This was disappointing, but I know that this wouldn’t be the only gene therapy clinical trial for haemophilia that I would have the opportunity to be involved in, so that was okay.  It was the results of the ECG and the fibroscan that had me more concerned.

 

For several weeks I wondered what I was going to do?  It wasn’t long before my consultant approached me about another clinical trial, and I was going to say ‘yes’, but I couldn’t get the results of the ECG and the fibroscan out of my head.  How would left ventricular hypertrophy and liver fibrosis affect me if I were to go ahead and join another trial, especially as the vector is designed to insert the gene into the liver?  This led me to the decision to write to my consultant and explain that under the circumstances, unil I knew more and was comfortable with the outcome, I couldn’t consent to a gene therapy trial.  My consultant referred me to a hepatologist to discuss the fibroscan results, and I made an appointment with my general practitioner (GP) – it’s what we call a family doctor in the UK.

 

My GP requested another ECG, a chest X-Ray, and a blood test for brain natriuretic peptide (BNP – before you ask, no, it’s not a right-wing political party, it’s a hormone released by granular cells in the right atrium of the heart).  Strangely, the second ECG came back normal, as did the chest X-Ray.  The blood test, however, showed increased levels of BNP.  The increase wasn’t sufficient for further treatment, well, not in my health authority area, but in other health authority areas it would have prompted further investigation.  With all this my GP wrote to a cardiologist requesting advice and guidance as to what to do next?  I’m still waiting on the response, but to me that is an indicator that things aren’t as bad as I may have first thought.

 

My visit to the hepatologist went better than expected.  With everything that has happened in the past, I thought that maybe something was up with my liver relating to hepatitis C.  It couldn’t possibly be due to alcohol because I don’t drink alcohol and never have.  So my mind was racing away with fears of cirrhosis and the future prospect of liver failure.  However, the registrar that saw me soon put those fears to rest.  They ordered another blood test for hepatitis C (for those interested it’s the polymer chain reaction, or PCR) and then took me through the fibroscan results explaining that for my age and weight (let’s just say I’m not as skinny as I used to be and leave it there) the result was as would be normally expected.  I explained my fears and the fact that I don’t drink, and the response was still that this result would be normally expected.  This simple statement made the hairs on my arms stand on end.  I’m okay!  I’m really okay!  I had to supress the urge to fist pump the air, but I’m certain it was evident that I had a sudden influx of adrenalin.  Thank goodness the doctor didn’t test my blood pressure!  The doctor did, however, explain that there was no reason that I should not consider another gene therapy trial, should the opportunity present itself, as my liver is not compromised.  This was music to my ears.

 

I decided to give it a couple of weeks before I approached my consultant again to request consent forms for the clinical trial that was previously mentioned to me, but before I could do so, the world changed.  The NEWS became full of stories of a new debilitating respiratory virus affecting and killing people in Wuhan, China.  The virus is called SARS-CoV-2 and the illness it causes is called COVID-19.  The world became gripped by what was going on, and then news of the virus spreading to other countries became a daily occurrence.  First Wuhan was shutdown, then large swathes of China.  It was only a few weeks until a pandemic was declared by the World Health Organisation (WHO).  When it reached the UK it wasn’t long before a new phenomenon became the norm – social distancing and quarantine.  As I write this blog entry my family and I have been social distancing in our home for just over 6 weeks.  The whole country, and most of the world, has shut its doors.  Businesses are closed, the roads are virtually empty, and there have been 195,438 reported deaths globally, with 19,506 of these in the UK.  I still haven’t been consented into that clinical trial, but when I think about it, I am glad that this is the case.  You see, part of the protocol for the clinical trial included the possibility of having to take corticosteroids to supress my immune system in order to facilitate the gene therapy vector to infect my liver with sufficient copies of the Padua factor IX gene to get me to express around or above 100% of normal factor IX levels.  In the context of a new world order where social isolation is the norm because of a viral pandemic which is killing thousands, suppression of my immune system is not an appealing option for me.  Right now I am glad to be with my family, and we are healthy, happy, and most importantly, alive.

 

This is not all that has transpired since I last penned, okay, typed, a blog entry.  As you may recall, in December 2017 I resigned my membership of the Haemophilia Society over their decision to change the name of the charity.  I won’t go into it all – you can always read my previous blog posts for that.  Well, I learned the other day that the CEO, Liz Carroll, had resigned her position with immediate effect.  I don’t know the reasons why she resigned, and I have no intention or desire to find out.  However, this did reignite a spark within me to once again realign myself to the society.  I admit that I didn’t see eye to eye with Liz over various issues – hence my resignation, but irrespective of that I do know that she did a lot of good for others and in other people’s eyes, so I wish her well in whatever her future holds, and I hold no animosity toward her.  But with her departure it became apparent to me that I could now test the water and reapply for membership to the Haemophilia Society, which I have done.  This does not mean that I have changed my position, but it does mean that hopefully I can once more feel united with others in furthering and championing the cause of haemophilia and other bleeding disorders whilst remembering the sacrifices and efforts of all those who have done so, and more, to this day from the inception of the Society.  I have since learned that my membership application has been accepted, and I am also delighted to have received welcome messages from old friends whom I won’t embarrass here (you know who you are).

 

Until my next blog post all I would say is this:

 

Stay home.  Stay safe.  Protect the NHS.

About Face… Maybe?

It’s not often I say this, but I have a confession…  I finally made a firm decision regarding a question which has been on my mind since it was first proposed to me by Professor Charles Coutelle at a conference in Manchester in 2005.  I have since been asked the same question many times, and each time I gave the same, or a broadly similar answer.  That is, until a few weeks ago.  My thoughts now have radically changed.  The conference I referred to was either the second or third Annual Conference of the British Society for Gene Therapy (now called the British Society for Gene and Cell Therapy – see www.bsgct.org), and Professor Coutelle was among the audience of a talk I was asked to give as a patient with a genetic condition – severe haemophilia B.  At that time Professor Coutelle was in charge of a research group performing in-utero gene therapy for haemophilia B, and had achieved proof of concept in mouse models.  His question to me, which he posed at the end of my talk, was thus:

 

“lf I could offer you an injection to cure your haemophilia, would you take it”?

 

What type of a question that is, eh?

 

I didn’t answer him straight away, instead taking a moment to gather my thoughts and give a reasoned response.  My reply was along the lines of this:

 

“No, I don’t think I would.  I’ve lived with haemophilia for a long time and it has shaped my life in many ways.  I know my physical limitations, I understand when I need treatment, and it is part of who I am.  As an adult I have fewer bleeds than when I was younger, so injecting isn’t really that much of a bother or hinderance to my life.  The main issue I have is pain and mobility issues due to my arthritis which is caused by years of internal bleeding into joints such as my ankles and elbows.  If you could take away the arthritis, I’d bite your hand off (figuratively), but if you take away my haemophilia, then what is left of me?  Who am I now?  I would have to re-learn how to live life, so I think that gene therapy is for the younger generation – those who have not yet developed painful and arthritic joints; those who have not received infected clotting factor; those whose lives are clean sheets.  For people like me, gene therapy is a non-issue.”

 

Since this talk, I have been asked to return to talk at the BSGCT public engagement days of their annual conferences on a number of occasions.  I have also been asked to talk at the European Society for Gene and Cell Therapy public education day at The Hague in the Netherlands in 2014.  Each time I have been asked the same question, though in recent years it has been posed by sixth form science students.

 

My mind has been pretty much settled on this matter for around 14 years.  Then, a few weeks ago, I was in a routine outpatients appointment at my haemophilia Comprehensive Care Centre (CCC) in Newcastle when my consultant mentioned to me that there was a phase 3 clinical trial of a new gene therapy for haemophilia B, and that my CCC had been selected as a participating site.  They had looked for suitable patients on their lists and I fit the initial inclusion criteria – am I interested in participating?

 

This blew me away.  I’ve read about gene therapy for many years, I have personal friendships with professionals in the field, and here I am facing that same question once more, although this time it is not hypothetical, it is real.  The stark reality changes everything, but more important than that is the potential this has for future generations of haemophiliacs – some of whom will be my descendants.  As attractive as it is for me to participate as a subject on a personal level, I believe I have a duty to participate as a subject to help facilitate a new therapy for future generations which goes far beyond mere symptom management.

 

So, I said “yes”!

 

2 weeks ago, I formally consented into the study.  Earlier today I had my first blood tests taken (13 tubes of blood!), and in the coming weeks I will be provided with an electronic treatment diary.  However, there is a catch.  If I have been exposed to the particular strain of virus that is being used as the vector (vehicle carrying the gene) and I have antibodies to it, I will be automatically excluded from the study.  Therefore, right now I am waiting to hear the results of today’s blood tests.  It will take approximately 2 weeks for the results to come back, and if they come back saying that I am antibody negative, then I will officially begin the 6 months baseline data collection phase of the trial.  Near the end of this initial phase I will be re-tested for antibodies to the vector.  This is because during this period is it possible that I may have sero-converted (that’s a technical term for catching the virus and developing an immune response to it).  This is a possibility because the type of virus being used as the vector is adeno-associated virus (AAV) which is a naturally occurring, relatively non-pathogenic virus similar to the common cold.  There are various sub-types of this virus (known as ‘serotypes’), and the particular type being used in this experiment is AAV-8.  If, at the end of the baseline data collection phase, my second blood test for antibodies is reported as being negative, then I will be given one intravenous injection of the therapy and I will be regularly monitored for circulating clotting factor IX trough levels, antibodies to the vector, liver function tests, and so forth.  From the phase 1 and phase 2 clinical trials performed using this particular vector and gene (it’s the padua variant of the factor IX gene which will be transfected into my cells) the data are promising.  In many cases severe haemophilia patients have developed sufficient circulating clotting factor IX levels to alter their phenotype from severe to mild, and others from severe to moderate.  Either of these options is an amazing thought… that is if my antibody screens come back negative and I manage to reach this stage of the trial.

 

I have been thinking a lot today about Professor Charles Coutelle and his initial, somewhat audacious question, and I have come to the conclusion that it was he who laid the foundations in my mind which prepared for this moment.  I have much to thank Charles for, even though he may not have realised how poignant his question to me was at that time.

 

At this point I feel it would be wrong of me to not also mention others who have significantly influenced me in respect of this decision over the years.  They have been, and many remain, active sources of information, support and inspiration.  I owe them a great debt of gratitude for helping me glimpse an understanding of the journey I am about to embark upon, and their names are:

 

Professor Simon Waddington

Professor Len Seymour

Dr Suzie Buckley

Dr Renee Watson

Dr Glenn Pierce

Professor Rafael Yanez-Munoz

Dr Natalie Ward

Jacqui Marr

A New Chapter Begins.

I have not written an entry for my blog for the best part of a year – that’s a long time!  For those of you who follow me, I apologise, but I must admit that it was a conscious decision to walk away for a while.  As you will have seen from the last few posts I have struggled to reconcile myself to the events and revelations which preceded and lead up to the public inquiry into contaminated blood and blood products.  I have also struggled with the involvement and reaction of organisations which I once believed were there to help and support people with haemophilia.  I attended a meeting where there were many active campaigners giving voice to their horrific and heart-wrenching experiences, and their opinions regarding what should happen with respect to the upcoming inquiry and its frame of reference.  During that meeting someone said something to me which made me stop and think.  It was a person for whom I harbour great respect.  Their personal sacrifices to fight to publicise and correct the injustices of the contaminated blood disaster are second to none.  He explained how his mental health suffered from his encounters with individuals and organisations determined to hide the truth and evade justice in previous inquiries, and how he expected this to happen again once the public inquiry began.  This made me think long and hard about my own mental health and wellbeing.  I am not ashamed to say that I quickly came to the conclusion that I should step back from my own campaigning for a while for the sake of my mental health and also to guard my family from the stress and worry that comes with experiencing the deteriorating mental health of a close family member.  I believe that this was the correct decision for me and for my family.

 

During the interceding time from my last post the public inquiry has begun.  There are many brave and courageous people involved along with legal representation of various affected people and groups.  I therefore hope that the inquiry will be swift, thorough, leave no stone unturned, and provide the justice and closure which is long overdue.  I, along with many others, will be following it closely.  The Department of Health has already issued an apology in its opening statement to the public inquiry.  This is a good start, but considering the physical and mental suffering, pain, and deaths that have ensued from this disaster over the years, there lies ahead many mountains to climb.

 

There have also been advancements in haemophilia treatment since my last post.  A number of new treatments are in either phase 2 or 3, clinical trials (e.g. spk9001), and some which have obtained, or are in the process of obtaining, a license from the FDA or its European equivalent (e.g. Hemlibra).  I will post something more detailed about this in the not too distant future.

 

For now, I intend to focus on how I am beginning to feel that my self-imposed exile from the world of haemophilia advocacy has served its purpose and is coming to an end.

 

Here’s to the beginning of a new chapter in my life of learning to love living with severe haemophilia B.  There is still so much to learn!

Fall Down Seven Times, Get Up Eight. (Japanese Proverb).

I have an opinion (a shocking revelation, I know – sarcasm intended)!  It’s an opinion that will go against the grain of most, if not all clinicians.  An opinion that parents of children with haemophilia may be shocked at and feel that maybe I have lost my marbles (I remember exactly where I left them – in Never Land).  It’s an opinion that is open to criticism and debate, but for a moment, just hear me out – please?  It is, after all, only an opinion!

I have come to believe that every person with haemophilia should be allowed to study and practice a martial art.  Before I go on, I don’t mean full contact – that would be silly.  Even so, I can already hear the sharp intakes of breath; I can see in my mind’s eye people shaking their heads; and I can feel the gazes of horrified parents and clinic staff penetrating me.  I’m not crazy – honest!

Think about it though, it actually makes sense!  In any martial art the first thing you are taught (if your teacher is reputable), is to break-fall.  This means when you fall to the ground, you can land in such a way as to dissipate the energy of the fall in order to minimise any damage.  When this is practised, it is performed using crash mats so that those under tuition are not hurt.  Would this not be a useful skill for anyone, let alone people with bleeding disorders?  Learning how to fall properly would, in my opinion, prevent many bleeding episodes and reduce factor use.  Some may say that the practice itself is dangerous and could cause bleeding.  I dispute this, as the overwhelming majority of severe haemophiliacs in the UK (where I live) are on prophylaxis, which provides them with protection.  Mild and moderate haemophiliacs likely would not come to any harm at all performing a break-fall even without treatment.  Severe haemophiliacs should, in my opinion, have trough levels of 5% or over.

Next, martial arts classes always begin with a warm up and stretching which improve flexibility of the joints, strengthens the tendons and muscles, and means that there is less chance of injury.

The practice of a martial art would also be classified as regular, moderate exercise, keeping the practitioner relatively and generally healthy.

Martial arts are practised in a controlled environment and allow for mistakes in a safe and controlled fashion.  This is a major point.  I’m 46 years old, and I have been the victim of random attacks.  It’s a risk we all take when we step outside, but for someone with a bleeding disorder this is so much more serious.  If aimed at the right place and with enough force, even a simple single punch is enough to kill the average person.  If it doesn’t kill them, it will, at the very least, leave massive soft tissue damage.  So it makes sense then to learn how to dissipate the energy in an attackers blow by rolling with it; to be practised enough to see the attack coming (hopefully) and thereby have a split second more to either block, or evade the oncoming attack; and to be trained enough to know that Hollywood fight scenes are nothing like real life, so the avoidance of confrontation is paramount, and defence is only ever a last resort borne out of necessity.

I don’t know about you, but I think these are useful skills?

Notice that I’m not saying that all haemophiliacs should enter the Olympic Boxing, Judo or Taekwondo teams.  Neither am I saying that all haemophiliacs should become nunchaku swirling weapons specialists, or consent to being beaten to a pulp and thrown around a dojo on a weekly, or more regular basis.  I’m simply looking at the benefits that a martial art has to offer.  Things that aren’t taught by the physiotherapist, social worker, or other clinical staff at the local haemophilia unit.  Things that have a direct practical application to help minimise injury when danger presents – whether that danger be an icy path, an oily petrol station forecourt, or catching the last bus home from work or a social gathering and being mugged by a stranger.  It’s the techniques of damage limitation that I think are useful.  It just happens that they are taught very effectively in oriental martial arts.

Of course, I don’t think everyone should go out straight away and join a martial arts club.  People need to evaluate their personal circumstances.  Not everyone will be able to stand or walk, so is there a club with a teacher who would be willing to accommodate any special requirements, such as sitting, wearing shoes with supports, and so forth?  If necessary, can the person with haemophilia alter their prophylaxis date and time to accommodate their training?  Could a treatment plan be put in place in conjunction with the medical team at the comprehensive care centre?

All of this said, people will experience injuries whether or not they practice a martial art.  The more important question is whether the injuries that will inevitably be experienced would be less serious with martial art training than without.  Whether practice means only a bruised arm from falling on a concrete pavement instead of a broken arm, shoulder, or hip?  Whether training means you have a better sense of balance and only sprain a tendon in your ankle instead of snapping a cruciate ligament in your knee?  Or whether your familiarity with the signs of an impending attack give you a split second more to be able to react and deflect the blade of a knife, block a punch to your face, evade a kick, or escape completely instead of ending up unconscious and fighting for your life in a random street – or if you’re lucky enough – a hospital operating theatre where they may not know you have a bleeding disorder?

I don’t advocate martial arts to encourage people with haemophilia to engage in dangerous and risky activities.  What I do advocate is learning from people who can teach tried and tested techniques to help recognise threat, minimise physical damage, and mitigate unexpected danger.

At some point we all fall over and get hurt, but not everyone gets up afterwards.  If I fall down seven times, I’d like to be able to get up an eighth time, and a ninth…

What Doesn’t Kill You…

The purpose of this blog is, and has always been, to explore my life, thoughts, and experiences as a person living with severe haemophilia B – and everything else that comes with it.  Recent events with the Haemophilia Society have been harrowing for me, causing me to question much of what I was once certain of.  I have not yet reached a point where I have been able to accept, reconcile, or even understand my emotions about it.

 

I know what you’re thinking, “How can the simple process of a charity deciding to change its name have such an effect on someone?”.

 

Well, it hasn’t.  The rabbit hole goes deeper, much deeper, than that.

 

Yes, I have been upset at the prospect of the Haemophilia Society changing its name.  I have explained my position on that quite clearly in my previous post.  I still think the same, and I am also still no longer a member of the Haemophilia Society.  I acknowledge that my thoughts are not mirrored by the society, indeed the society still has not responded to any of the questions I posed in my resignation letter, except, that is, to say that they do not agree with my view.  That’s fine.  There are many others since who have expressed thoughts and feelings similar to mine, and many are also leaving the society.  But that’s still not it.  Really the name change is a superficial issue, one that I feel is designed to draw a line, distinguishing the Haemophilia Society as was from the society at present and in the future.  But why?

 

I am tempted to use a cliché, such as ‘there’s no smoke without fire’.

 

You see, it was when I read this article that I realised that I have been lied to over a great many years, and that I can no longer trust those in whom I had placed my trust.  I feel my sense of belonging to a community has been destroyed by these revelations.  I find it unconscionable that such betrayal could have been perpetrated, and unethical, immoral, and inconceivable that the society thinks it is able to independently represent those it has failed for funding, and also thinks it deserves a place on the inquiry panel when it will likely also be under investigation.

 

All this has left me in a state of limbo, no longer knowing where I belong, whom I can trust, or whether there is anyone that can, and will stand up for me and others in my position?

 

It appears that far more people have resigned their membership of the Haemophilia Society over this than over the proposed name change.  Frankly, I’m not surprised.  Had I not resigned my membership before, I certainly would have with this news.

 

There are those who are standing by the society, saying that it is a wholly different organisation than it was before.  That’s entirely their choice, and I respect them for it.  Though to me it does not mitigate the fact that the society knew all this and yet did nothing in respect of making it known or making reparations to its membership before now.  An apology would have been something, but I am not aware of any statements from the Haemophilia Society which goes anywhere near providing answers to the points made by Jason Evans.  There was one initial response on Facebook from the chairman of the society which states that they do not deny the points in Jason’s letter, and further stated that there would be an official response within the forthcoming week, but that deadline came, and went unanswered.  That was in the new year.

 

How do I feel about all this?  Devastated.

 

Has it affected me?  Yes.

 

What happens next?  I really don’t know?  Maybe I’ll leave it here, maybe I’ll join another membership organisation for haemophiliacs, like Haemophilia Scotland (if I can)?  Or, maybe I’ll start another organisation for haemophiliacs and people with other bleeding disorders?  Whichever option I ultimately choose it will mean that I will need to rediscover what living with haemophilia means to me.  This journey of self-discovery was always going to happen, I just never envisaged that this is the direction it would take me in.

 

There is still so much to be excited about in the world of haemophilia.  Every day there are reports of new and upcoming treatments, and I am grateful for that.  The future for people with haemophilia is still filled with hope, even if the past does keep resurfacing and reminding us of the scars we have collected along the way.  But then, scars are the proof that we survived another day.

 

So I’ll raise a figurative glass to tomorrow – in anticipation of the next scar.

Milestones & Turning points

The past few months have been quite a rollercoaster ride in many senses of the term.  Several things have happened which have somewhat caught me off guard, each a major milestone or turning point in my life.  A selected few of them follow in this post.

Recently I signed up to a Masters Degree in Clinical Trials.  This is a chief milestone in my life as I never thought I’d steer away from bench science toward experimental design, legislation, ethics, and statistical analysis.  I hated mathematics at school.  It took me 4 attempts to get GCSE mathematics at grade C, yet here I am doing Masters level statistics!  I must be mad… but I love it!  It keeps me focused and helps me to channel my energy productively.  It’s difficult, but it’s a challenge I enjoy as my mind is much more able and willing to be challenged than my body is!

Another milestone is the decision of the UK government to instigate a public inquiry into the contaminated blood scandal.  This came out of left field, as no one really believed that this would ever be something the government would agree to.  But it’s not without its twists and turns as at first the government want the Department of Health (DoH) to be in charge of the inquiry – a non-starter as far as the haemophilia community is concerned because it’s the DoH who are at the very centre of the scandal in the first place.  Then the appointment of the chair is dragging out, there are reasonable petitions for a committee to be instigated, but all of this is being roundly rejected by the government.  Presumably because this inquiry is going to be set up to be yet another whitewash!  We shall have to see how this turns out, but I don’t hold out much faith in the process.  It feels like haemophiliacs don’t matter and that we are somehow a burden to society, and an inconvenience in general to successive governments.  For example, in the tragedy and criminal affair that was the Hillsborough disaster there was a full public inquiry with wide remit to prosecute where criminality could be demonstrated, and people, including police officers, were prosecuted and convicted, with relatives and victims receiving due recompense.  In contrast to this the contaminated blood scandal has lasted longer, seen more people die as a direct consequence with more awaiting their inevitable fate, seen more families affected, and yet those so affected have not received one ounce of justice, or even an official apology.

Then, to add insult to injury, at their 2017 AGM at Heathrow airport, the Haemophilia Society voted to change its name to “Bleeding Disorders UK”.  It was said that this was something that was wanted by the membership of the Society, and was something which the executive and board of trustees wanted to happen so the society could have freedom to campaign more widely in behalf of bleeding disorders other than haemophilia.  Now, please don’t misunderstand me, I’m all for inclusivity, and have no objection to people with other bleeding disorders being part of the society and gaining therefrom.  But for a society that was set up with the constitution and articles of incorporation to specifically campaign for, and help people living with haemophilia to then turn its back on all those people who have built it up from its inception, raised funds specifically to fulfil its constitutional purpose, supported it, and given freely of their time and energy over many years, is simply betrayal.  Funds that were raised in good faith would now be used for purposes other than those originally intended by those generous enough to donate and those who sacrificed in fundraising efforts.  The timing is also very poor – I mean, why publicly change your trading name just when the public inquiry, which has been campaigned for all these years is finally beginning to materialise?  What message does this send to the politicians and the media?  What message does this send to all those who are suffering the effects of the contaminated blood scandal and the families of those who have already died?  It’s simple, the message is ‘we no longer care’, the message is ‘we no longer wish to be associated with you’, the message is ‘haemophilia no longer matters to us’.  The message rings loud and clear, and I find it distressingly awful.  When I heard this was happening, I made my opinion known to the society only to find that there were a great many members of the society who had not been balloted and were similarly outraged at the proposal (which, by the way, was passed at the AGM with 6% of the membership voting for it – hardly representative and painfully undemocratic).  I felt that a turning point had been reached where I could no longer support the society as a result and resigned my membership of both my local group and also the society.  My request was swiftly enacted by the society and I was also subsequently blocked from their Facebook discussion forums.  I received, about a week after my letter of resignation, an email from the CEO acknowledging receipt of my resignation and a statement that the board of trustees and the CEO would respond as soon as possible.  That was about two weeks ago and I have heard nothing from them since.  To say that I have lost faith and confidence in the society which for many years I felt represented me, is an understatement.  My letter of resignation follows this article – I think it sums my feelings up quite succinctly.

About 3 or 4 days after I resigned, the society decided to back track.  That is to say the board of trustees were informed how many people were complaining at not receiving a ballot that they issued a statement saying that the name change would not now go ahead as planned, but that after the conclusion of the public inquiry there would be another ballot of the membership to enact the proposed name change.  This, in my opinion, is nothing more than an exercise in temporarily saving face.  They still want to do it.  They still intend to do it.  So why wait?  Just get it over with already and let us haemophiliacs get to work forming a new charitable organisation that actually works for all haemophiliacs and people affected by all types of haemophilia in the UK!

Some people think I’m over-reacting, after all, what’s in a name?  Well, names depict who we are.  Names are things that identify us and our affiliations.  They are a source of rallying and community (how would you feel if your favourite team suddenly changed their name after years of support?).  Names bind us as families and extend a sense of pride and purpose, and belonging.  Names are important.

I still want to champion the cause of haemophilia.  I still want to advocate for change, better treatments, increased access to improved medications, increased access to medication in the third world, and so forth.  I can just no longer do that through the medium of the former ‘haemophilia society’.

Change is coming.  It always does.  But we can choose to act in order to determine the direction of that change, or we can stand idly by and be carried away in the acts of others.  In the words of Ghandi – “Be the change you want to see”.

Until next time…

Adam.

My letter of resignation from the haemophilia society:

17 November 2017

FAO: Liz Carroll & Board of Trustees

Dear Liz,

I have been a member of the society in one capacity or another for as long as I can remember.  I live with severe haemophilia B and have family members and friends who are also affected by this debilitating illness.  I have witnessed first-hand the contaminated blood disaster.  I have been infected with hepatitis B & hepatitis C.  I have received letters regarding potential contamination of blood products with vCJD.  I have lost friends to hepatitis and HIV infection.  I have represented and campaigned for the society and its members on many occasions.  I have spoken as a patient advocate at academic conferences around the world, and have been involved in various fundraising efforts.  It therefore pains me to admit that I have lost confidence in the Haemophilia Society (or whatever name it now goes by since the AGM on Saturday 18^th November 2017) and its leadership.  I no longer believe that the society represents haemophiliacs and their families, and is publicly moving to distance itself from haemophilia and the tireless efforts of those who have built the society from its humble beginnings.

The decision to change the name of the society is not indicative of a desire to build a community, but rather to facilitate haemophilia being pushed into the background and a destruction of the sense of community and fellowship that thrives amongst us.  I have no quarrel with people with other bleeding disorders, indeed I welcome them with open arms, however, it must be made perfectly clear that this society was set up, and built up, for and by haemophiliacs and their families.  Haemophilia is not just a genetic illness – it is an identity and a way of life, and this is being destroyed by the imposition of a name change of the society.

Indeed, this name change will invariably lead to various outcomes such as:

• Haemophilia, being a rare clotting disorder, will no longer be at the forefront of the society and other initiatives and disorders will take precedence irrespective of socioeconomic or physical impact.
• A lower media, public, and political profile of haemophilia and the plight of those so affected.
• As bleeding disorders such as Von Willerbrand’s disease (VWD) are much more common than haemophilia, there will undoubtedly be a drive to recruit more members in this category – meaning more money in the pockets of the society.  This demonstrates nothing more than a financial incentive (interestingly Type 3 VWD is also known as “pseudo haemophilia”).
• The public inquiry into contaminated blood products has not yet even begun.  For the society to even consider changing its name at this juncture will undoubtedly have an effect upon the public and political perception of this inquiry.  Why would the society that is supposed to champion those affected change its name at this point of the process?  This is simply a public declaration that the society is done with haemophilia, through with fighting the contaminated blood issue, and disavowing itself of its past associations.
• The sense of community, fellowship, and identity that exists amongst haemophiliacs will be destroyed.
• The funds donated by members and friends of the society will be used for the new organisation instead of the Haemophilia Society.

It has been said that the request for a name change came from within the membership and has been repeated over a number of years.  So I ask you, which members campaigned for this?  Are they members with, or without haemophilia?  Are they long standing members, or recent affiliates?  Who in the society championed this cause and raised it as an issue?  Was this course of action approved by the Board of Trustees?  If so, when did that happen?  Was it a unanimous decision?

Also, why, when such a contentious issue is being passed, is the AGM being held at Heathrow – a location notoriously difficult and expensive to access, and distant from much of the membership?  Some might conclude that the society seeks to avoid hearing disapproving voices, like mine?

You simply fail to recognise the distress all of this is causing.  To use a football analogy, this is like Manchester United saying to its fans “I’m sorry, but we have more fans in China than in Manchester.  We have had a ballot and as a result have decided to change the name of the club to ‘Beijing United’”.  Can you imagine the uproar and outrage amongst supporters?  They strongly and passionately identify with their club, for whatever reason.  Therefore, to do this, and then say “it’s okay, nothing is really going to change.  We’ll still be playing football”, is, quite frankly, a betrayal of loyalty.  Yes, betrayal.  There is no other word which adequately describes ones feelings on this issue.

As a result the society is obviously out of touch and no longer represents the interests of the members it was set up to assist and campaign for.  Therefore, I see no other option but for the society to step down from its membership of the European Haemophilia Consortium, the World Federation of Haemophilia (notice it’s not “The European Bleeding Disorders Consortium” or “The World Federation of Bleeding Disorders”), and any other group or body associated with haemophilia.

It is therefore with deep regret, and a heavy heart, that I am writing to you to request cancellation of my membership of the Haemophilia Society and to be removed from its records.  I cannot, in good conscience, continue to be part of an organisation which is participating (either actively or passively) in the demoralisation and fragmentation of the haemophilia community within the UK.

Yours sincerely,

Adam Jones.

Flying At Half Mast With A Heavy Heart…

If I had a flag and a flagpole, then today I would be flying it at half-mast.

 

It has been reported today that a patient with haemophilia A who was a volunteer involved in the phase 2 clinical trials of Fitusiran (designed to treat haemophilia A and B with, or without, inhibitors), a pipeline medication in the portfolio of RNAi specialist company Alnylam, has died from a thrombotic event.  It was first thought to be a subarachnoid haemorrhage, but on further examination of CT scans by 3 independent neurologists, it turned out to be due to a cerebral venous sinus thrombosis – or a blood clot in a vein that takes blood from the brain.  You can read the report on their website here.  This clot would have caused a blockage preventing blood escaping the vessels around the brain, but blood would still be entering these vessels, causing a back-pressure of fluid (oedema).  Seeing as the brain is encased in the skull, which is made of bone, there is no possibility of accommodating the increasing volume of fluid entering the vessels of the brain. This intracranial pressure from the fluid presses on the brain, and eventually causes death.

 

My heart goes out to the family of this young man.  Nothing I, or anyone else can say will be able to fill the emptiness of their hearts, or soothe their grief at this awful time.  But there is one thing I will say, the courage of this young man in coming forward as a volunteer for a phase 2 dosing trial of a new medication is testament to his selflessness and desire to bring respite into the lives of people living with haemophilia A and B everywhere.  His participation in this trial has already saved the lives of countless others.  No one wants to see volunteers die in any clinical trial – everyone involved in these trials only want to improve people’s lives, so you can be certain that everyone involved will be scouring the data to get to the crux of what happened.  And when they understand the issue, and they will get there, they will put things in place to ensure that this never happens again.

 

It is unfortunately impossible to eliminate all risk in experiments.  You can manage risk, but you cannot foresee every eventuality, so when something unexpected happens, you stop, study it, and figure out how to ensure it can be controlled for and effectively managed in future.  You can be certain that the investigators in this trial will want to make certain that the unfortunate and unexpected death of this volunteer is remembered and is not in vain.

 

This makes the relationships between patients, clinicians, and the pharmaceutical industry ever more important.  Each patient requires a personalised treatment plan  which considers an understanding of risk and benefit from all sides, but at the end of the day only one of those involved in this relationship will be having the needle inserted into their vein, so theirs is the most important perspective.  We need to strengthen ties and increase understanding and trust between all involved in this tripartite relationship.  The more we share, the more we learn, and the more we progress.

 

So, in closing, I want to dedicate this article to the young man who died, and to clinical trials volunteers everywhere who choose to undertake risk in behalf of the rest of us.  We are all blessed by your courage and selflessness.  Thank you, and may God bless you.

Don’t Get Left Behind,Get Involved!

Wow, where did summer go?  I was just getting settled in and… whoosh – it’s gone!

 

So many things are happening in the world of haemophilia that it’s almost impossible to keep up with the pace.  Here’s a taste:

 

• Biopharmaceutical companies are trialling different versions of extra-long half-life clotting factors such as polysialylated and PEGylated versions
• There are many companies looking at gene therapy for haemophilia B (generally utilising various serotypes of adeno-associated virus as vectors)
• Haemophilia A is getting in on the gene therapy action also – which is great news as it’s always been more difficult to find a suitable vector due to the larger size of the factor VIII gene
• RNAi technology is also on the scene with an upcoming new treatment for haemophilia patients with inhibitors (this is very exciting news as it targets the inhibitors at the level of genetic translation of the antibody protein)
• Also, with the advent of CRISPR/Cas9 technology (also known as gene editing) there is talk afoot about the possibility of editing the foetal genome, or even more radically, editing the germline (I always knew that this would be inevitable, though I think it might be a while yet before it becomes a reality as the uninformed and tabloid media will have a field day, just like they did with mitochondrial replacement therapy, such as pronuclear transfer)
• The UK government have caved to public opinion and announced a public inquiry in to the contaminated blood scandal – however this isn’t to be taken at face value as they are still doing everything within their power to obstruct it by putting the Department of Health as the Lead in an inquiry against itself (most other organisations would consider this to be a conflict of interest), they are still ignoring the calls and wishes of all those who have been affected, and key dates for things like determining the terms and scope are being put back again and again and again. But then, are we really to have expected anything different?

 

With all this coming through there is an even greater impetus for patients, carers, and healthcare professionals, to be more engaged and educated with regard to these new treatments, their actions, limitations, proper use, and policy.  What will they mean for prophylaxis regimens?  Will the prospect of adverse reactions be more, or less likely?  Will the cost of these new treatments be a stumbling block when attempting to pass the desks of the commissioners and be funded by a future, ever decreasing, NHS budget associated with ever increasing red-tape and fiscal justifications?

 

This may seem onerous for many, and it may well be easier for most to think that we should “leave it to those in charge of these things”, but at the end of the day it’s our veins that the needle is being stuck in to, not the far-removed politician or civil servant who looks at me, and the cost of my treatments, as numbers on a spreadsheet.  This means that if we truly want the best out of our medical care, we need to get more involved in the understanding, education, and regulation of it as progress happens and advancements are made.  And it seems that advances are being made at a startling rate… so hold on to your seat, or you’ll be left behind!

Nine Dots & Logical Fallacies.

Nine dots.

Just nine simple dots, no more, no less.

Arrange them in a square of dimensions 3×3.  Once you have done this (I assume you have drawn the configuration) and using only four consecutive, connected, straight lines, and without removing your pen from the paper (or from altering the paper in any way), you must connect all nine dots by ensuring each dot has one of the lines going through its centre.

Go on, try it.  I’ll wait…

Waiting…

Still waiting…

Done it yet?  No?  I’ll wait a little longer then…

Keep trying.

Go on!

Almost there!

Any luck?  No?

Don’t quit on me, you can do this!

Frustrating, isn’t it?  I mean, how difficult can it be, right?

If you followed my instructions correctly, then you would have started out with something like this image below (ignore the square around it):

 

 

You then just need to join all the dots with four straight, consecutive lines.

It’s not a trick question and there is no hidden meaning or cunning deception.  You just need to complete the task as instructed.  It is perfectly possible!

Shall Put you out of your misery?  Okay, one of the possible answers look like this:

 

 

I know what you’re thinking.  You think I cheated, right?  But the truth is, I didn’t.  If you look back over my instructions you will find that they have been followed strictly to the letter.  At no point were any other conditions or restrictions placed upon you that would have prevented you from achieving this same outcome.  Indeed, the only restrictions that did prevent you from achieving this are those which you unwittingly set for yourself through assumption, conditioning, and the self-imposing of limitations, or boundaries to your task.  Just because the dots were laid out in the form of a square does not mean you need to restrict your attempts to solve this problem by limiting your efforts to the same perceived, yet non-existent form.  You will notice that the solution requires that, on more than one occasion, you must travel beyond the limits of the problem that has been presented to you.  In other words, in order to find an appropriate solution, you need to understand and view the problem in the wider context of its environment, and not simply see the problem as a single isolated entity.  Stephen R. Covey said it thus:

“The way you see the problem is the problem”!

We all go through life armed with what we think we know.  This is not only obvious (I can hear you saying “Duh… y’think?”), but also dangerous as knowledge is often perceived as being a weapon to wield to demonstrate greater mental prowess over someone whom we perceive to have less knowledge than ourselves.  But the greatest knowledge that is to be had is found in the one who recognises that all that they know prompts yet further questions, and thus leaves them understanding that actually, they know nothing.  This then, in my opinion at least, is the early buds of wisdom, and the start of a journey towards the realisation that not everything is as cut and dry as we might first condition ourselves to think, even though we might want it to be so through unintended confirmation bias.

This is true for much more than knowledge alone, and amongst these things it is true of living with a long-term condition, such as haemophilia.  Boundaries and limitations are placed upon you before you can even walk or talk, and it is expected that you conform irrespective of whether-or-not you want to.  After a while these expectations become engrained into your own psyche and they then find their way into your habits, lifestyle, and character.  From this point on your path is now at least partially predetermined as these parts of your character become self-limiting, helping to develop you into a self-fulfilling prophecy, hesitant to ever tread beyond the boundaries which others fashioned for you long ago.

In some respects, this is no bad thing.  For example, if you have internal bleeding it is expected that you inject clotting factor to stop the bleeding.  This is perfectly understandable and sensible.  However, in other respects it acts as an unintentional, but well-meaning hindrance to being allowed and able to live, enjoy, and love life in the same way as everyone else.  For example, whilst an undergraduate I joined the university SCUBA diving society (SCUBA is an acronym for Self-Contained Underwater Breathing Apparatus).  We would have weekly sessions in the university pool and receive training in the theory, practice, and safety issues involved in SCUBA diving.

I enjoyed immensely everything to do with SCUBA diving and felt I was progressing well.  Then it came to applying for my open water license.  At that time the society was affiliated to the British Sub-Aqua Club (BSAC) and it was through them that my application was to be made.  I had already taken and passed the theory exam and just needed to gain a license for my first open water dive.  To do this I required a medical examination and certificate of health.  My medical was great – this was the period of my life when I was most physically fit (all things considered), so I submitted my forms.  It wasn’t long before I received a response telling me that the diving doctor for the United Kingdom Sports Diving Medical Committee (UKSDMC) had refused my application on grounds that I have haemophilia.  This seemed incredibly odd to me as my medical was performed by my haemophilia consultant and I had letters of support from 2 haemophilia consultants (one whose care I was under whilst at university, and one from my home comprehensive care centre in Birmingham), letters of support from the Haemophilia Society and the World Federation of Hemophilia (WFH), and guidelines for the management of haemophilia.  It seems that all this was not sufficient for this doctor and despite my protests, multiple appeals, and support from the university society and my healthcare professionals, he refused point blank saying that I am a liability to everyone else underwater.  This was enforced despite the simple fact that appropriate training and a license is not required for someone to purchase and use SCUBA equipment.  So essentially I was being refused the opportunity to receive proper training to be able to safely SCUBA dive.  Tempting though it was to go out and buy my own gear anyway, as a physiology student I understood all too well the dangers of doing so.  So, as I was no longer allowed by BSAC to even remain a member of the society (the society were threatened with BSAC removing their affiliation if I were to continue to train with them), I gave up and discontinued pursuit of my interest, and I have never again attempted to try and push this externally imposed boundary, regardless of whether, or not, I believe it to be informed, correct, or just.  This, I feel, was a great shame, as I believe that this would have helped my physical fitness, and would also have been a great asset to my academic curriculum vitae (the physiology of extreme environments is both fascinating and understood less than we would like).

You will not be surprised that I believe that this situation was a direct result of confirmation bias on the part of the UKSDMC diving doctor.  It was his opinion that my having haemophilia was a hazard which precluded my participation in this sport, even though there were numerous potential solutions presented and expert medical opinion to the contrary.  I am certain his intentions were honourable and that his decision was not laced with malice, however the way that this situation was viewed by this doctor was the real issue.  Had he been open to other ways of managing my haemophilia whilst SCUBA diving, then this could have worked out so differently.  This is the strength of other people’s confirmation bias.

 

Those of us living with long-term health conditions often feel that we must appear, on the outside, in the way that others expect to see us, because if we don’t there are those who would doubt our situation, question our integrity, or invoke guilt for happening to be having a better day than yesterday.  The British comedian, Michael McIntyre, has a good stage routine which addresses this and lays bare it’s absurdity; because after all, it is the way we, as individuals and as a society, see problems/illnesses/disabilities which is the real problem.  Therefore let us not exert the limitations of our own bias on others, for life is to be lived and loved by all – not just those in good health.

A Continuing Injustice. Will it Ever End?

The past few weeks has seen haemophilia going through a surge in the popular consciousness.  The news seems to be littered with articles about the contaminated blood scandal, with focal points such as:

 

• The final ministerial statement by former MP and now Mayor of Greater Manchester, Andy Burnham, in the House of Commons
• The revelations in the Daily Mail that haemophiliacs were being administered blood products that were known for 5+ years to be infecting them with hepatitis and HIV
• The further revelations that this was happening without the knowledge of the patients and their families, but full knowledge (and it seems maybe even approval) of the government and medical/scientific personnel (see previous link)
• The fact that every major national political party in The United Kingdom (with the noticeable exception of the Conservative Party) had in their manifesto for the recent snap general election, a pledge to hold a full public inquiry in to the contaminated blood scandal and to finally obtain justice for all those who have suffered from the effects of this terrible atrocity (if you’re wondering why certain parties don’t feature in this brief list, then that is because I do not consider them to be major national parties on the basis that they have extremely few, or no MPs, or that they are parties with a specific remit for a devolved regional government and therefore not a national party per se)
  1. Labour Party Manifesto
  2. Liberal Democrat Party Manifesto

• The most recent point of order in the House of Commons given by Diana Johnson MP (who also happens to be the chair of the All Party Parliamentary Group on contaminated blood)

 

I won’t pretend that this isn’t having a negative effect on me, as it seems to pervade every sphere of influence I can imagine – television, newspapers, radio, Facebook, Twitter, and so forth.  It is bringing to the surface memories and emotions of personal experiences from long ago, heartache for friends and relatives who are still affected, and grief for those who did not, and may not survive.  That said, this issue needs to be resolved to the satisfaction of those who are and have been directly or indirectly affected, irrespective of the desire and intentions of certain politicians to continue to avoid doing so by making all but meaningless token gestures, setting up inquiries without proper authority to determine whether or where blame lies, demand answers and documentation from all involved sources, and ultimately to prosecute if and where it is appropriate and legal to do so.

 

Please do not misunderstand me, it is great that this is receiving more attention as it remains a modern-day atrocity which is yet to be remedied and proportionate justice secured.  But important as this is, I often think that on a personal level I’d much rather focus on the positive steps forward that have been made and that are being researched and developed.  It’s not that I wish to brush this under a carpet, or avoid the issue – not at all – I just feel that the past holds many negative and difficult experiences and emotions which have their place in my life, but I don’t necessarily wish to be reminded of them all at once (if you are curious as to what some of these experiences are, then please see some of my previous, and no doubt future, blog posts).  That said, for those haemophiliacs and their families who live each day wondering if it may be their last, or how they are going to support their family because they have not been able to work due to their deteriorating health caused by contaminated blood products, this is something that I am certain is a constant mental, emotional, and physical companion for which they require justice, settlement, apology, peace of mind, and from which they undoubtedly long to be estranged.

 

The facts are irrefutable.  For many the pain and suffering remains and is greater now than it ever was.  Greater even than most people would or could bare.

 

Over 2,000 have died from causes directly related to this, with their families and friends left to grieve a life cut short far too early.  Governments may attempt to placate or dodge the issue, but it will not go away until the victims are satiated.  This has already gone on far too long.  Surely it is time for the government to swallow this difficult pill and allow everyone, including them, to be able to finally put this behind them and move forward with hope and confidence in the new generation of treatments which can and will aim to increasingly ease our symptoms, control our pain, and ensure that a disaster such as this will never happen again.  There are people, some whom I know personally, that are diligently and tirelessly working towards this very aim.  They are giving their all.  It is time that those responsible for this situation did likewise, and it is time that the rest of our society, including those with the legal authority to do so, held them to account.

Personal Evolution: Moving From ‘Creature’ to ‘Creator’.

One of the many people I admire is the world-renowned business leader, educator, author, and motivational speaker, Dr Stephen R. Covey (1932-2012).  My admiration for him is not based in his economic success, but his disciplined focus on principled leadership and his drive to help others to help themselves.  His book ‘The 7 Habits of Highly Effective People’ is a seminal work of deserved renown.  Within its pages are a great many insightful observations, recommendations, and challenges for behavioural change.  I would highly recommend it above all other books within its genre.  Though I could write much concerning this book, it is not the focus I want to have in this article.  Instead I want to draw attention to one of Dr Covey’s quotes which reads thus:

 

“The key is not to prioritise what’s on your schedule, but to schedule your priorities”.

 

This is a pivotal insight that I continually strive to perfect my understanding of, and then refine its implementation in my life.  One issue with this philosophy is the conflicting priorities of others, and how they impact upon our priorities.  Unless these external influences are also among our own priorities, it is often difficult to reconcile the competing demands.  It is this conflict that I would like to spend time talking about, though not in the context of business or self-improvement, but in the context of living with a long-term health condition, such as haemophilia.

 

Everyone has a schedule that they fulfil each day.  This is true whether or not you happen to be the master of that schedule, or even if you do nothing.  Your schedule is the manifestation of the activities you intend to achieve.  These activities may be productive or unproductive, labour intensive or labour free, mentally engaging or a complete distraction.  Whichever category they fit, they are the result of an active choice that you have made at some previous point in time.  Some people, such as managers (I will use the term ‘manager’ for anyone with any real or perceived authority over us, whether legal, contractual, voluntary, familial, or otherwise) have priorities which will in some way directly impact upon us.  Therefore the decision to be made is whether these imposed priorities are inferior to, equal to, or supersede our own?  The wise will first determine which of these imposed priorities are relevant to them and their position/situation, and then work to find some way of integrating these with their own priorities.  Sounds simple enough, right?  Well, maybe, but is it really as simple as that?  I don’t think it is.

 

Speaking from my own experience, there may be many externally imposed priorities which are both relevant and pressing to me and therefore find a place on my schedule, however they may not be one of my priorities at that time.  This means there is a conflict between that which is important for my manager, and that which is important to me.  Let me clarify, I am not referring to flights of fancy or rebellion against people in positions of power, I am instead referring to times when my health is suffering but the external expectations remain relentless.  In this instance my priority is to treat and recover, but the work/deadline/expectation from my manager is unyielding.  Some may scoff at this as surely all I have to do is speak to my manager and they will understand my plight?  Well, yes, but not every situation is quite so simple.  When you have a chronic illness things like this happen so often that you may perceive that your requests are too frequent and you may fear being labelled as a work-shy slacker, or even a burden to those around you who depend upon you and upon whom you depend.  When this happens you know what your priorities should be, but you may feel obliged to ignore them and persist in your attempts to complete those which are externally imposed – even though it is highly likely that you are not capable of working to your best ability.  This is often referred to as ‘soldiering on’ and is nothing less than a destructive, self-defeating behaviour.  I say this unashamedly, yet I am acutely aware that I am guilty of it.  There have been countless times in my life when I have felt pressured (irrespective of whether that pressure is externally or internally imposed) to do something or be somewhere when the wisest decision would have been to take greater care of my immediate health concerns and needs, and defer or delegate the responsibility to another time, place, or person.  This is a classic example of prioritising my schedule instead of scheduling my priorities.  Anyone can fill their schedule, busying themselves, and be effective in those tasks, but not everybody can control their schedule and busy themselves with things which are effective uses of their time.  Taking time to heal and recover, being honest and open with those around you regarding your health needs, and prioritising this over all else, is the only way of ensuring that you are able to use your time effectively now and in the future.  It means that when you are performing, you are performing at 100% capacity, and not struggling along at some diminished level of competence.  It means that your relationship with your manager can develop into one of mutual trust, so that when you are there, they know you are 100% there.  It means that you also allow yourself to not feel guilty for being ill.  After all, when living with a long-term illness it is inevitable that you are going to be ill at some point, so why make it arduous and burdensome when you are?

 

The essence of having a schedule is that those things that fill it are to some degree moveable.  A schedule is not something that must be adhered to at all costs, because the cost might just be more than you can bear – either physically, emotionally, mentally, or economically.  The most effective schedules should have a certain amount of inbuilt flexibility, thereby providing room for dealing with unforeseen events; but most importantly of all, you must not be frightened to utilise that flexibility when your circumstances require.

 

This, in the context of self-management of personal health and wellbeing, is my interpretation of Dr Covey’s quote.  Here it is again:

 

“The key is not to prioritise what’s on your schedule, but to schedule your priorities”.

 

I sincerely hope that all who read this post find a way to schedule their health priorities effectively amongst every other competing influence with which they must contend.  By so doing they will hopefully develop the confidence to evolve from being creatures of their own circumstance to being creators of their own circumstance.

A Right Royal Pain in the..?

One thing that people living with one or more long-term health conditions have in common is pain.  In my experience pain takes many forms, and affects me in different ways at different times.  Sometimes it is generalized pain, sometimes it is very specific, and sometimes it is a complex mixture of these two categories.  I find pain difficult to quantify.  Some people use nominal classifications, such as “severe”, “moderate”, or “mild” to describe their pain, whilst others use various systems which are similar to the ‘Borg Scale’, where the severity of the pain is determined by the sufferer on a numerical scale, often 0-10, 0-13, or 0-20, and so forth.  As useful as these classifications might be, I personally feel that they are intrinsically limited and thus do not tell the whole story.  Indeed, something as subjective as pain is, in my opinion, almost – if not entirely – impossible to compare between individuals, and therefore impossible for one person to know and understand how another will feel, interpret, and be (un)able to respond to their pain.

 

Aside from my experiences with acute bleeding episodes, I have, for a great many years, been in a state of constant pain.  The arthritis in my ankles is probably the most debilitating, mentally and physically.  I do take painkillers and anti-inflammatories (diclofenac sodium 75mg BD), but this merely manages my pain, it does not rid me of it.  Though I often yearn to be free from pain, I do think it also has a useful purpose and with care and wisdom, can be employed to great benefit.  I have told people this before and almost invariably they think me to be mad. How can pain possibly be employed in a useful way?  How can pain be classified as “good”? Surely this is foolish talk?  Well, I don’t think so.  I think it has its place amongst all of the other coping mechanisms that people who live with pain need to develop if they are to regain any amount of self-efficacy over their life.  For me, as well as pain being a signal conveyed from peripheral and visceral nociceptors to be received and interpreted by the central nervous system and the somatosensory cortex, it is also very much a useful internal calibration tool (or you may prefer the term “measuring stick”).  If I know how much pain I can be in, and still be able to utilise other coping techniques in order to perform to a certain level, then I am in a much better position to understand the limitations of my body in relation to my present or intended actions.  This provides me with the ability to find a pace that is suited to the task(s) ahead of me, or alternatively it helps me to know when I am doing too much and need to stop.

 

It’s not a perfect system by any stretch of the imagination.  There are times when I still do too much and end up paying the price later, and there are times when I don’t want to continue the task that I am doing at that moment but circumstances dictate that it must be done, and I therefore have little or no choice in the matter but to proceed knowing that I am going to be in a world of pain later.  The thing that is crucial to understanding and effectively using this technique is balance.  In an ideal world one should be able to self-manage and avoid unnecessary pain.  However, the reality of modern life doesn’t work in an ideal manner.  Just because a doctor or other health professional wants you to take control in a certain way does not mean that you are capable of doing so from minute to minute, hour by hour, day to day, week by week.  There are other pressures that bind your activities, such as personal hygiene, work, education, chores, shopping, cooking, and so forth.  These are all essential activities which require attention and effort.  Most of the time you may able to self-manage how and when you achieve them, but often one or more will demand attention beyond your usual and known limitations.  This is the point at which frustration begins to build, yet this frustration serves only to make the activity and the pain more arduous and all encompassing.  If left unchecked, it can become consuming.  Life, it seems, does not follow an ideal path – at least not the sort of ideal that some people outside of your situation and looking in would have you believe, as on paper, everything seems so simple and logical.

 

In recent years there has been only one time when I felt completely pain free in my right ankle.  This was when I had my first intra-articular steroid injection.  It was weird.  Very weird.  I must admit that it was wonderful to feel no pain, however it was also rather worrying as I knew that the injection merely removed my pain, it had not improved the articular surfaces of my ankle at all, so there was a real danger that I would do too much too quickly and risk a bleed that I would have no way of noticing until it was too late.  So as much as being pain free is something that I long for, it needs to be taken in context of the wider picture of my health.  Hence pain management is a more realistic goal to help me manage my life effectively and find some sort of balance, but as much as I and others would like it to be, it will never be perfect.  As such I can expect a future with pain as an ever-present companion, with the severity in constant flux as it is now.  But with careful planning and listening to my body, it can be managed to yield the best possible outcomes that I can realistically hope to achieve.  Having said that, it is inevitable that as I age my joints will deteriorate and my pain will increase, so I will have no choice but to continually adjust and develop my coping mechanisms accordingly.  And that’s the point, isn’t it?  Though you may be constantly in pain, the pain you are in is not constant, but dynamic.  As such a ‘one-size-fits-all‘ approach to pain management, even for one individual, is not an effective long-term approach, but more of a temporary stop-gap along the way to achieving long-term pain management.

 

Over the years I have tried many different coping mechanisms to help manage my pain, ranging from medication, exercise, and surgical interventions, to cognitive-behavioural techniques such as visualisation, distraction, guided imagery, and so forth.  Each of these tools have their value and limitations, but nothing that has a sound evidence base should be off limits to achieving a sustainable level of pain management.  I said ‘nothing’ there, didn’t I?  Well, I personally do draw a line at interventions which, in my opinion, have no sound, independent, peer-reviewed evidence base, such as homeopathy, reiki, and other such practices.  Don’t get me wrong, if it works for you, great, go ahead and knock yourself out, but I see no value in pursuing these types of alternative therapies (or as I prefer to call them – ‘expensive placaebo’s‘).

 

There is still much to learn about pain.  We have valuable insight in to receptors, neural transmission pathways and somatosensory perception, but because it is so individual and dynamic, we don’t really possess a complete understanding of it – but we are making steady progress.  Who knows, maybe the advent of 3D tissue engineering mixed with effective pain management may be able to deliver a future where not only will it be possible to be completely pain free, but also ensure that this is permanent by providing remodelled articular joint surfaces?  Then, rather than having to perform highly complex and risky prosthetic joint replacement operations, less traumatic articular surface autotransplantation procedures may be able to fully restore joint function, reduce/eliminate pain, and provide better long term outcomes?  Personally, I think that this day is not that far away.  Watch this space as I know there are research teams already working on this very possibility!  Until then, however, where did I put my diclofenac..?

Humility, Horror, and Gratitude.

I find it amazing how, when you think you’re on an even keel and the wind is in your sails, life has the uncanny ability to ground you back in harsh reality.  At this point, and depending on the stimulus, one of two things happen, you either:

 

1. Consider the past experiences of your life and how they, and the decisions you have made, led you to the point that now faces you, and how you intend to move forward to improve your situation.
2. Consider the past experiences of your life and how they, and the decisions you have made, led you to the point that now faces you, and how thankful you are that you were able to move forward to improve your situation.

 

Of course, there will be those amongst us that might add a third option – to hide from our situation and pretend it doesn’t exist.  Though I am certain there is much mileage in writing about this option, I cannot see that it leads to anywhere productive, either for any given situation in which we find ourselves, or for our feelings of self-worth and self-efficacy – so I won’t waste time and effort extolling the virtues of being an emotional ostrich.  Instead I will continue with the two original statements above, because you see, today, I experienced both.  One I experienced actively, and the other I experienced vicariously.  Intrigued?  I hope so, because I certainly have been!

 

It all started early this morning when I briefly checked my Facebook feed.  Last night I had been involved in a conversation with another haemophiliac who had been experiencing some difficulties and I just wanted to check to see if there had been an update on how he was doing?  As I was engaged in this, I was drawn to a post that was placed by the Haemophilia Society.  It stated that there was another haemophiliac in need of advice as they had been recently told that they required a radioactive synovectomy, but they nor their doctor knew much about the procedure, and so if anyone had any experience of it, would they mind getting in contact for a chat?  Well, today is day 17 post synovectomy for me, so I replied saying that I would be more than happy to chat and explain the procedure and my experience of it.  It wasn’t long before I had the contact details of this gentleman and I could speak to him by telephone.

 

At this point I should explain that I have no intention of breaching this persons’ confidence.  I shall not use their real name, and neither shall I disclose their whereabouts.  But what I shall do is attempt to paint a picture in your mind with words, though I am no Leonardo Da-Vinci (but possibly leaning more toward a Lowry/Dali/pre-school toddler hybrid).

 

When the telephone was answered, I heard the voice of a senior gentleman, and when I say gentleman, I really do mean it in all the possible connotations that the word has to offer.  He spoke softly, politely, calmly, with an air of intrigue that leads you to believe that the person to whom you are talking has experienced more in their lifetime than you could ever hope to fit into three equivalent time spans.  There was a comforting, soothing, and effortless rhythm to his speech that made me want to listen and not talk (for those of you who know me personally, you will realise just how novel, and frankly ridiculous, the notion of me being voluntarily quiet actually is).  I found myself listening to this gentleman regale me with just a few of his own personal experiences of living with haemophilia over the many years of his life and being fascinated at how, in some respects, our lives were quite similar. That is until he began to recount some of his more recent experiences which were directly related to the purpose of our conversation.

 

Recently this gentleman had been experiencing some quite severe bleeds into his last fully functioning joint – his elbow.  Normally this would be a “so what?” moment, but I was quickly taken a-back by his explanation of the events that led him to this point.  He is not a very mobile person, suffering the effects of haemophilic arthropathy and living in an area where there is no local haemophilia comprehensive care centre, or even a haematologist of any sort.  I thought that this was bad, but then he explained that recently he had been experiencing these quite bad elbow bleeds (again… “so what?” springs to mind).  As there is no haemophilia centre nearby he has to attend the Accident and Emergency department of his local hospital to obtain treatment, however on two successive occasions he was refused treatment as the doctor who examined him felt that the elbow is a non-essential joint, and as clotting factor is expensive, he did not see a good case for providing treatment.  This meant that the elbow bleed got much, much worse.  In fact it got so bad that this gentleman spent the next 3-4 weeks on morphine for the pain.  It eventually healed, and not long after another bleed ensued.  He attended A&E once more, this time he saw a different doctor.  However, this doctor did not know what to do, and saw in the medical notes of this patient that the previous doctor had refused treatment for the elbow bleed.  Therefore on the basis of clinical precedent, this doctor also refused treatment, and another 3-4 weeks of intense pain and daily morphine ensued.  Did this gentleman complain?  No, he didn’t.  He got on with it all and took it in his stride.  On a subsequent visit to his general practitioner (GP) these treatment refusals were noticed, and his GP was not happy with the level of care that had been proffered.  This prompted the GP to contact a distant haemophilia comprehensive care centre to speak with one of the consultants.  When the consultant heard what had happened he was left speechless that this level of care (or rather lack of care) was still in existence in the health service of a first world country in 2017.  As I listened to his story I was gripped with a sense of morbid awe, for what I was hearing was horrifying, but at the same time the apprehension and quasi-medieval nature of his story was both gripping and unbelievable.  I could not find words to adequately express my thoughts and feelings.  Indeed, any rendition of something like “I know how you must have felt” would have simply been an outright lie, for even though I have suffered many an excruciatingly painful bleed, I have always received treatment in the most timely manner for the current medical understanding of the day.  I have never been left to suffer the agony of active bleeding without treatment for such a long period, and I hope I never will be!

 

The remainder of our conversation went as planned and we discussed a radioactive synovectomy and my personal experience of having one performed – for which he was extremely grateful.  After this he thanked me and politely hung up the telephone.  My mind was racing, although I didn’t quite know, or understand, which direction it was racing, or indeed where it was racing to?  My thoughts seemed somewhat conflicting and my emotions tended to flick between sympathy, horror, disgust, and gratitude.

 

Sympathy; because I understand all too well how painful a bleed can be and how desperate you can feel when that pain becomes all-consuming.

Horror; because it is simply inhumane to expect, and wilfully allow, anyone to experience such agony without providing the very treatment that would be the healing balm which would alleviate their suffering.

 

Disgust; that someone in such a privileged position of trust over the health, wellbeing, and care of the most vulnerable members of our society should knowingly betray that trust, and coldly violate their duty of care.

 

Gratitude; because I am thankful that I have never been on the receiving end of such wilful neglect.  Also, gratitude because I had the privilege to converse with someone who had gone through such an experience, yet harboured no animosity toward his oppressors.

 

Today I have been truly humbled.  I have been made to think of all my past experiences of living with severe haemophilia B, and to give thanks for being privileged to have had the treatment and professional, heart-felt care that has been freely administered to me.  I have also been made to think of how this gentle-giant of a man has been humble enough to not dwell on the effects of lack-lustre standards of care, but to look forward to, and actively seek out, a path to a future potentially free of pain.  Thus, I have experienced point 1 vicariously through the experiences of this wonderful gentleman, and point 2 I have experienced as an active participant.

 

I sincerely hope that one day I can look back over my life with the same sense of acceptance, and look forward with the same sense of gratitude, as was evident in the voice of and conversation of this rarest of men today.  This is truly an aspiration worthy of the closest attention and deepest dedication.

 

I truly hope that a theatre slot is found for him at the earliest opportunity, and that the procedure will be successful in every possible way!

Spinning the Wheel of Fortune.

I’m now 16 days into the postoperative period of my radioactive synovectomy.  I am acutely aware of my lack of updates, but it is for a good reason – the risk of internal bleeding is much increased during the 2 week period immediately after an invasive procedure.  Hence I have been trying to rest my elbow as much as possible during this time.

 

My synovectomy was quite a last-minute affair.  Initially I had been told to prepare myself for the 10^th March as there were other haemophiliacs in the same position as me who were having their procedures done on that day and the rhenium was already on order.  It was only a short while after that where I was informed that the staff who are in charge of theatre bookings had turned down my consultant’s request because the theatres were fully booked that day.  Thus, I was left in a state of limbo, awaiting a phone call from the haemophilia unit on an unknown day and at an unspecified time, to inform me of when the possible date of the procedure might be?  So I went about my usual routine, but with the apprehension in the back of my mind that if it didn’t happen soon, I may be taken off IDELVION and put back on to Benefix as the problems with my elbow were causing me to use more IDELVION and it may turn out that the new treatment may not prove to be cost neutral.  A quick intervention is what I was desperately in need of, but the decision of the theatre booking staff had the effect of turning my silver lining into a high stakes spin of a roulette wheel.

 

Then, ironically on the morning of the very day that I was originally told to prepare for, I had just dropped my eldest son at his secondary school and was driving in to work, when I received a call from an unknown number.  I pulled over to the side of the road and answered.  It was one of the Clinical Nurse Specialists from the haemophilia unit at the RVI.  I was informed that one of the haemophiliacs who were to have their synovectomies that day had backed out of the procedure at the last minute and, if I was able and willing, I was being offered their theatre slot.  I jumped at the chance and said yes, although I would have to clear it with my line manager and go home to pack an overnight bag first.  Luckily I had not eaten breakfast at this point, so I had to continue my unplanned fast until after the procedure, but that was a small price to pay in return for the prospect of fewer bleeds and slowing the pace of arthritis in that joint.

 

After meeting with my line manager to confirm that I was okay to go ahead with the synovectomy, a few phone calls, and readying my overnight bag, I headed to the RVI.  I was to report to ward 42 where all my preoperative checks would take place and I would be given factor cover to ensure I didn’t bleed and to assist my healing.  I arrived at the ward at around 11.00 AM, just in time to settle down and watch all the other inpatients be given their lunch.  It smelled quite inviting, but when I looked at it I was reminded of the slop that was fed to Neo on the ship Nebuchadnezzar in the film The Matrix.  I didn’t particularly want to know what it tasted like, and there was no woman in a red dress to distract my attention!  As much as I love and respect the NHS, the food that is served to patients is sub-standard in terms of both calorific and nutritious content.  Personally, I would have to be anaesthetised and on some serious painkillers that dulled my gustatory senses before I ate that poor excuse for what I guess you would have to call, in the loosest possible terms, a ‘meal’?

 

I finally was called to theatre at about 2.00 PM.  As is usually the case I started to get nervous, and when that happens I start wittering on about random nonsense and making dire attempts at ‘dad humour’.  My fears were somewhat quenched though when I saw a familiar face – one of the theatre porters that came for me used to be a receptionist at the haemophilia unit.  She had seen my name listed in theatre and remembered my NHS number (I should get frequent flyer points if I’m that well known), so she decided that she would take me to theatre to help me with my nerves and for a bit of a catch up.  It was a great idea.  Immediately I began to feel much more relaxed and comfortable. After having my factor cover, we proceeded to head to theatre.

 

There were quite a few staff in theatre.  All of them not only dressed in their theatre scrubs, but also wearing lead-lined aprons to protect them from the radiation given off by all the rhenium they were handling that day and the X-Ray’s from the machine they use to guide the needles into the joints.  I also spied a Geiger counter placed near to the trolley on which I was laying down.  A necessary precaution, but a little reminiscent of a Hollywood B-Movie where I was the unwitting patient involved in some unlicensed experimental procedure.  This was my very own Dr David Banner (“The Hulk”) moment… but my clothes didn’t fit because of the hideous and loosely tied hospital gown that I was wearing, not because I was hoping that a side effect of the procedure would be to develop super-human muscles!

 

The theatre staff were extremely professional, but I was rambling non-stop.  One of the nurses was laughing at me whilst I was recounting my previous experience of having steroid injections in my ankles, and how loudly I screamed.  I warned them to cover their ears before they started, and as I was saying this I felt a needle enter my elbow.  It was nothing special.  It just felt like having an injection into an area of skin that I hadn’t used for a while.  It was obvious that this must be the local anaesthetic, and the rhenium needle was to follow shortly afterwards – or so I thought.  I heard one of the radiologists say “it’s in position”, then within a split second I felt the inside of my elbow ballooning from the inside and the joint space filling with something.  This was not the anaesthetic.  It had to be the rhenium!  It was a little uncomfortable, but nothing unmanageable.  Then, after a minute or two, the needle was removed, a dressing placed over it, and my arm was swiftly immobilised with a plaster of paris back-slab and a sling.  It was all over.  I was shocked at how painless the procedure had been, but even more shocked at the speed from start to finish.  I had only been in the actual theatre for about 10 minutes before I was being wheeled back up to the ward.  I got back to my bed at about 2.45 PM and I changed out of my theatre gown and into some pyjamas.  I sat there for a while in wonder.  I couldn’t quite grasp the events of the day – it all seemed to be such a blur that I hadn’t yet had a chance to sit and think about it, but now that it was over I had plenty of time for that!

 

After a punctuated night of what I suppose barely passes for sleep (my arm was fine and I don’t begrudge it at all, as there were several serious events that took place during the night involving a couple of the other inpatients and the ‘crash team’ which woke up most of the ward) I was seen by John Hanley, Consultant Haematologist and haemophilia specialist.  John and I discussed the procedure and my treatment plan.  Everything had gone smoothly and I just needed to rest up my arm for a few days, attend the haemophilia unit for reviews with the team, be assessed by the physiotherapist, and have some trough FIX levels taken, before going back to work the following Thursday.

 

And so here I am today.  My arm is still a little uncomfortable, but less so than on the day of the procedure, and I am taking things easy before ramping up the rehabilitation.  Time will tell if the synovectomy has had the effect that we were all hoping to achieve, and to be honest, I am excited to find out as the results so far have been very promising for I have not yet had any further bleeds in that elbow.

 

As for my silver lining turning into a spin of a roulette wheel, I guess lady-luck must be on my side after all?

Caught in the Trap…

One of the things that has plagued me over recent years is the vicious cycle of repeated bleeding into my joints (mainly ankles and elbows) that leads to detraining, decreased fitness, increased weight gain, and decreased self-esteem, which leads to decreased energy, motivation, and physical ability (see figure 1).  It has got to the point now where I have gained excessive weight which I fear is affecting both my health and ability to self-manage effectively.  Thus I have made the decision to attempt to do something about it and break the cycle.

For a great many years I was as physically active as could reasonably be expected.  I would exercise regularly each week to build my muscular and cardiovascular fitness, but as I have aged, my joints have become increasingly arthritic, limiting movement, and increasing pain levels.  It reached a point some years ago when this cycle, mixed with other extraneous factors, led to a time of worsening physical health, deepening depression, and as a result my motivation to continue regular exercise evaporated.

 

Since that time I have, on a number of occasions, attempted to re-engage in exercise, but each time it has ended in a failure to re-ignite the flame of enduring motivation.  Often I had to stop for a while due to a bleed, or a surgical intervention that prevented me from exercising.  Unfortunately, by the time I recovered my determination had gone, and I would return to a state of inactivity.

 

Over the years this has led to increasing weight gain.  This is not healthy for a great many reasons.  There are the usual cardiovascular events that I am at increased risk of developing, type 2 diabetes, stroke, fatty liver, etc., but there is also the issue of what the weight is doing to my joints – especially my lower limbs.  I already have arthritis, but the extra weight I am carrying around will restrict my movement, put pressure on my ankles, knees, hips, and lower back, leading to increased pain and joint damage.  These are just a few of the reasons why I need to become more pro-active in my self-management.

 

As you know, recently I moved over to IDELVION, the new EHL FIX by CSL Behring, and from my last post, you will also be aware that there is the potential for me to be taken off this product and returned to Benefix, which is much less effective.  I don’t want this to happen.  I have finally reached a point in my life where my concordance with prophylactic treatment is excellent and my trough FIX levels are good, which is protecting me from spontaneous and minor traumatic bleeds.  I am already awaiting a rhenium synovectomy for my left elbow, my right elbow is severely restricted in its movement, both my ankles are severely arthritic having already had keyhole surgery and steroid injections with the future certainty of arthrodesis (joint fusion)… can I really afford to compound these issues through carrying excess weight?  The answer is simple.  No, I can’t.

 

I am under no illusion.  I know that this is going to be a long and difficult road.  I understand that I will face issues with motivation and the inevitable pain of DOMS (if you’re not familiar with that term it is an acronym for “Delayed Onset Muscle Stiffness”) that comes the day after exercising.  I understand that I need a paradigm shift in the balance of my energy input vs. energy output, and that this paradigm shift will not happen overnight, but you never get anywhere if you don’t take the first step.  After that, it’s all about persistence, tenacity, and mental endurance.

 

I have learned through my education and profession how marvellous and adaptable the human body is, and also how when things go wrong, they can really go wrong.  But I have also learned that really it is mental strength and focus that are the only things that separate the good from the great.  It is the ability to persistently dig down deep within oneself and push through, regardless of the challenges and obstacles that arise along the way that ensures long-term success and self-efficacy.  I have also learned that to achieve this takes long-term objective planning, and short term incentives to build, and keep, momentum.

 

So, to bring back a theme of my blog which has been missing for some time (inspiring and motivational quotes), it was Nido Qubein who said “Your present circumstances don’t determine where you can go; they merely determine where you start”.  Bearing this in mind, here I stand and here I am determined not to stay.  Today now marks the point where I am choosing to break the cycle, leading to a new start to a healthier, fitter, happier me.  Will I succeed?  Time will tell, but I believe in the words of Henry Ford when he said “Whether you believe you can or whether you believe you cannot, you are right”.  I believe I can do this, therefore I will!

All Change… Maybe..?

I had an out patient appointment with one of the consultants at the haemophilia unit this week.  It should have been my consultant, Dr Talks, but she was away, so instead I got Dr Hanley.  It’s been quite some time since I had a clinic with John.  He’s an incredibly pragmatic person, but above all else – he gets my humour (and I believe he appreciates it, though he may be loath to admit it)!  The general up-shot of the appointment was that my appointment for the 10^th March to have the radiosynovectomy may not be going ahead as the theatre space is fully booked.  This presents a problem, though it may not be what you first think.

 

NHS England approved the use of EHL FIX products, as you know from my previous posts, but only as long as they are cost neutral to Benefix.  This is the problem.  You see before I transferred to IDELVION I had to sign an agreement which states that if my factor use is not cost neutral after 3 months I must change back on to Benefix.  This is the case irrespective of whether I am achieving better outcomes on the new product.  Shocking, but when the cost of IDELVION is almost twice the old price of Benefix, it is somewhat understandable.  So the issue is that I have been getting all these elbow bleeds due to the synovitis.  Hence, if the radiosynovectomy doesn’t happen soon it is inevitable that I will have more bleeds, which means I will use more IDELVION, which means the costs go up, and I may be taken off the new product in early April.  That sucks, because IDELVION is, in my opinion, far superior to Benefix in every way except cost (and packaging – there is far too much packaging with IDELVION).  So John is going to, in his own words, “continue negotiating” with the theatre booking staff as there is a strong financial and clinical case to be made to get me in as quickly as possible for the procedure.  This may, or may not work, as the theatre booking staff have their own objectives to achieve, such as reducing average waiting times all round, not just for one patient.  It’s crazy that different departments have competing pressures.  It’s almost like the political establishment wants the NHS to fail by placing it under as much internal, external, and financial stress as possible (I’ll not comment on that any further, but rather allow you to draw your own conclusions).

 

So, the outcome of the clinic appointment was that I’m essentially waiting for a call from the haemophilia unit, which could be as close as the day before, to confirm that the rhenium synovectomy on the 10^th March is going ahead.  If I don’t get that call, then that means the negotiations broke down and who knows when the appointment will be?  I’ll just have to wait for a letter to arrive in the post – but by then I may have been taken off IDELVION, which is not a position I want to find myself in.  Keep your fingers crossed for me… and your toes, your legs, your eyes, and, if possible, your ears!

Get Up and Glow…

So I had my appointment with my haemophilia consultant, clinical nurse specialists, physiotherapist, and orthopaedic elbow surgeon at the RVI in Newcastle yesterday.  There were a whole bunch more people there than that, but they were primarily medical, nursing, or physiotherapy students.

 

When I got to the haemophilia unit there was no time wasted in sending me straight to X-Ray to have my left elbow imaged.  I was a little disappointed as the radiographer wouldn’t let me view my elbow before I got to talk to the surgeon.  But never mind, on with the story…

 

The clinic was held in the depths of the RVI – a place I hadn’t visited before, but this was all part of the mysterious atmosphere surrounding what was going to be decided.  As you will recall from my previous posts, I was of the opinion that there are only 2 viable options for me, radiosynovectomy or arthroscopy and debridement, and my money was on the arthroscopy and debridement!

 

Mr Williams (the orthopaedic surgeon) welcomed me in, invited me to sit, and then proceeded to examine my elbow and its range of movement.  He had my X-Ray on the screen in front of him, but never turned the screen to allow me to sneek a peek!  After some questions and answers, he said words to this effect:

 

“Adam,I think a relatively good case can be made for a Rhenium synovectomy.  If you’re happy with that we’ll get the paperwork sorted out and be in touch”.

 

He’s a man of few words, but they are concise and poignant.  I managed to ask a few more simple questions such as when the procedure might take place and the length of the waiting list for the procedure?  His reply took me back somewhat as he looked at me and said:

 

“It all depends on when we can get the rhenium from the reactor.  We have to book a slot for it to be extracted, but we are already waiting for a delivery, so sometime around March 10^th I presume”?

 

So… 3 weeks!  Here I was expecting to have to wait somewhere in the region of 3-6 months, and I’m being tentatively offered a slot in 3 weeks.  This is awesome!  It will mean an overnight stay in hospital, and I think my arm may need to be immobilised for 24-48 hours thereafter, but as long as it goes well (which I have no reason to suspect otherwise) for all intents and purposes I should be having fewer problems with my elbow in the future!

 

From what I understand the rhenium has a half-life of around 3.8 days and emits mainly beta radiation and a little gamma radiation, though the penetration through human tissues is only 3-4 mm, so I won’t pose a risk to anyone else in close vicinity.  I’m sorry to have to disappoint all of you who thought I may be glowing like I’d eaten a bowl of Ready Brek ™ for breakfast, but I won’t be turning in to the Incredible Hulk anytime soon.  Some of my colleagues at work have suggested that it would be humorous for me to have a radiation sticker attached to my elbow for a few days – though I’m not convinced our health and safety officer would appreciate the jest?

 

Apparently the positive effects of a rhenium synovectomy aren’t really felt until 2-3 weeks post procedure, and the week immediately after the procedure may be somewhat uncomfortable due to the colloidal consistency of the treatment injected directly in to the articular space.  Still, it’s worth it if it manages to prevent further bleeding and slow down the arthritic changes going on in my elbow!

 

Am I nervous?  Yes.  Who in their right mind would want a needle stuck into the joint of their elbow?

 

Am I worried? No.  I have complete trust and faith in my medical team to be able to perform this procedure safely and professionally.

 

How do I feel about it?  Relieved, and quite excited at the prospect of potentially slowing down the rate of arthritic deterioration in my elbow.

 

That reminds me, I need my prophylaxis for this week (I’ve managed to get a whole week without a bleed).  Better go get my infusion kit and go say ‘Hi’ to Sammy Snake!…

Never Tickle A Sleeping Dragon

Okay, I admit it.  I am a Harry Potter fan.  There, I said it.  It’s not just me, it’s my wife and all of my children.  We are a family of “Potterers” (is that a word?  Did I just invent a collective noun for Harry Potter fans?).  We have visited the Warner Brothers Studio in Leavesdon near Watford several times and thoroughly enjoyed every moment.  We have opened the doors to the great hall of Hogwarts School of Witchcraft and Wizardry, we have had rides on broomsticks and in Mr Weasley’s enchanted car.  We have walked the cobbled street of Diagon Alley and gazed in through the window of Olivander’s wand shop.  We have even visited the very spot on the beach in South Wales (I’ll not reveal its location at this point) where Harry Potter buries Dobbie the (house) Elf!  I will also admit that I do tend to dabble in making toy wands for my children (see image) to play with, because, well, have you seen how much the replica’s cost?  It’s quite extortionate!

 

The Harry Potter books and films depict a young boy born to loving and protective parents.  His personal circumstances are beyond his control.  He is immediately judged by all who meet him.  Some base their judgement on his being a wizard, and others base their judgements on his history and the circumstances leading to his visible and ever present scar.  But deep down, he is a boy who wishes for one thing – acceptance.

 

He struggles with the expectations and impositions of others.  He fears for his future, but is willing to sacrifice it for those whom he loves and cares for.  He is challenged and rebuked by people with their own personal agenda to pursue.  But through it all, he finds his voice and makes a stand for the values he holds dear, and the people he has learned to love.  He rises to the challenges, suffering heartache and regret – even melancholy – along the way; and there are times when he would have given up and handed victory to his oppressors.  Yet still he manages to take these emotions and use them to motivate him to action.  He knows full well the losses that may occur, but he has also a keen understanding of the consequences of inaction through passive participation in the events in which he finds himself caught up.

 

It is this that makes Harry Potter the man that he becomes.  He learns to recognise his mistakes, and ensures that he learns from them.

 

Harry is not unlike the rest of us.  We each have the same decisions to make and the same challenges to face (well, we don’t have to face Voldemort and the Death Eaters, but I’ve met some people who could easily be mistaken for dementors as they have the same uncanny ability to suck all the happiness out of you as they enter the room), and the same choice of passive or active engagement.

 

The story of Harry Potter could be the story of any of us.  Instead of a wizard, he could be asthmatic, paralysed, have mental illness such as depression or anxiety.  He could have HIV, autism, ADHD, or any other disorder – even haemophilia.  I think this is why this realm of witches and wizards created by J.K. Rowling resonates so much with the readership, because Harry’s story could be our story.  Indeed we can each recognise elements of our life in his.  Our potential to excel therefore is laid hidden in the Latin motto of Hogwarts School of Witchcraft and Wizardry “Draco Dormiens Nunquam Titilandus”, or to put it in to English “Never Tickle a Sleeping Dragon”.  Harry was the sleeping dragon, until ‘tickled’ by Voldemort and his followers, in which case he arose from his slumber and became a fierce adversary.  Thus the decision each of us must make in our lives is between maintaining our slumber, or rising up as a fierce dragon in the face of our trials and tribulations; to be a passive bystander, or an active participant in the ups and downs of our lives.

 

Sometimes life sucks, but it is ultimately our reaction(s) which depicts the outcome.  So, whether you are a Hungarian Horntail, a Welsh Green, or a Chinese Fireball, embrace your inner dragon and face your adversity with gusto, which is how I have now resolved to face the forthcoming surgical interventions on my left elbow.

 

This dragon just got tickled..!

Hand Me the Chisel…

The past few weeks have so far been somewhat of a rollercoaster ride.  The success of the EHL FIX IDELVION is rather overwhelming and I couldn’t be happier.  On the other hand, my left elbow has not yet got 7 days without a bleed.  Though somewhat disconcerting, it is not the result of a failure of IDELVION.  I have been to see my physiotherapist, and he has scanned my elbow.  The scans quite clearly show synovitis and arthritic joint surfaces.  But more than this, there are osteophytes (abnormal bony outcrops) which are most likely to be the culprits causing the internal damage.  There may also be some loose bodies – small osteophytes which have broken off and are floating free within the joint – but this has yet to be verified.  Still, the grinding and crunching noises when I pronate and supinate are fairly reliable indicators that all is not well with my elbow.

 

It may seem a minor issue compared to most other things I have had to deal with during the course of my life, however to me, at this time, it is not.  It seems that the simplest of things is causing pain and internal bleeding.  This means that my elbow is swift becoming a new trigger joint, which will only serve to perpetuate the cycle of pain, synovitis, Internal bleeding, and arthritic changes.  Just the other day I was putting together a toy pram for my daughter to play with and the simple action of screwing it together left me in pain, discomfort, and unable to use my arm for any useful purpose (such as feeding myself, lifting a glass to my mouth to drink from, etc.) for several hours thereafter.

 

The problem is not so much the pain, neither the internal bleeding, for I am used to all of these things.  It is the overwhelming feeling of helplessness.  It is having no choice but to be the passive observer of the decline of my ability to remain independent and self-sufficient.

 

Some might say that it is pride, and that I should be willing to allow others to help and assist me.  They are probably correct, at least to some degree, but not completely.  It is the independent use of agency for the purpose of self-sufficiency that endows one with the hope and drive sufficient to endure the troubles and trials of life.  When you are faced with the possibility that this fundamental principle of self-directed action is in decline, with the distinct possibility that it may at some point vanish for ever, hope begins to fade and your ability to mentally and physically endure begins to falter.  It is then that the constant pain begins to become more burdensome, and the future seems all the more uncertain as a direct result.

 

I have an appointment with my haemophilia team and an orthopaedic surgeon specialising in elbows later this week.  I only see two options ahead for the short term.

 

1. Radioactive synovectomy
2. Keyhole arthroscopic surgery with debridement and washout

 

If I’m honest, I think the keyhole surgery is most likely in the short term.  Mainly because I think that there is far too much happening structurally to the articular surfaces of my elbow for a simple synovectomy to be able to correct.  But then even though hope is diminishing and my ability to endure is waning, they are not gone completely; and as long as they remain there is sufficient reason to continue on.  I may be many things, and have many failings, but I have never yet quit at anything.

 

So, fearful though I may be, my future is not yet written in stone.  I still have some time to chisel it out!

Trust Personified

I recently attended another musculoskeletal outpatients appointment at the Freeman Hospital in Newcastle.  Normally it would be under Mr Briggs’ clinic, but he has recently retired.  As such, there is a new consultant in town, Mr Torres!  I thought I might see Mr Torres in person, as this was my first appointment since he has taken over the reigns, but alas it was not to be.  Instead I got to see Dr Mangos, the Specialist Registrar in foot and ankle orthopaedic surgery.

 

Dr Mangos is a friendly face to me.  I have been under his care many times before, and he has assisted Mr Briggs in all the procedures that have been performed on my ankles, except for the most recent steroid injections.  Dr Mangos is from South Africa.  At least that’s what I think? He has that distinctive Afrikaans inflection, when he speaks, although it seems to be fading somewhat due to the length of time he has spent in the UK.  He also has a great sense of humour.  It’s quite a dry, some might say arid, sense of humour.  Even so, I appreciate it greatly!  For in the midst of my body falling apart, and knowing that various joints and surfaces are going to be ground, hacked, hammered, sawn, pinned, chemically numbed, etc., there is the glimmer of hope that comes from the blackest of comedy that can only be shared between patient and surgeon.  It is a bond like no other.  Both of you know intimately the pain, the treatments, the relapse, the deterioration, and the finality of what must be done, and the decisions and actions to be taken are not made in isolation, but in unison.  One cannot progress without the others consent and encouragement, and through all the pain, setbacks and anxiety, therein lies the beauty.

 

From my experience of his medical knowledge and surgical skill, I have come to trust Dr Mangos.  I cannot yet say the same thing about Mr Torres.  Though I am certain Mr Torres is a wonderful surgeon, I have no experience of him, nor he of me.  There must be a time for us to generate that trust which is so important between a surgeon and his patient, as without that trust I have no intention of letting anyone take a scalpel to my skin or a chisel to my bone.

 

On that note Dr Mangos and I conversed, in a matter of fact way, about the state of my ankles and the options that lie before us, of which there are but few.  I believe the technical term for the physical state of my ankles is obliterated.  Knackered would be a good synonym, as would mashed, hammered, jiggered, and screwed, but I think I’ll stick with obliterated for the time being – it is a word which has the cold air of finality that so befits the pathology.  It is possible for me to continue to have steroid injections, though these will have a diminishing return, much like that which was demonstrated with the last set I had done.  Another option is to do nothing, and put up with the pain.  The only other option is arthrodesis, or joint fusion.  I am beginning to reconcile myself to the fact that this latter option is not far distant.  My ankles are getting more painful by the day, even though I take my diclofenac and paracetamol.  That said, I don’t think that I’m quite ready for it psychologically.  I need to be certain that there are no other viable alternatives, and that I can no longer put up with the level of pain that I experience day to day.  It is already at the point where I often find myself seeking a mental escape from the pain, or at the very least – a distraction.  I think, and I discussed this with Dr Mangos, that I am likely to go ahead with a fusion sometime in the next 3-5 years.  I feel that I have put it off for as long as is feasibly possible, and to keep going any longer than that is not an option for me.

 

I was quite surprised by the response of Dr Mangos.  He told me that he would be happy to do whatever I wanted, whenever I wanted.  If I want a fusion, that’s fine, he’ll do it.  If I want another steroid injection, that too is fine (although it would be best to wait a while to get the best effects), and keeping the status quo is also fine as he understands the psychological impact and preparation required for such a procedure as ankle arthrodesis.

 

So, that’s where I currently am in relation to my bilateral ankle arthropathy.  Not quite in limbo, but neither quite moving forward.  At least I have hope, and the opportunity of another clinical examination filled with black humour, and, most importantly – trust.