One story in a thousand

Introducing Adam Jones / WFH Contributing Author

In April 1972 I was born in the Sorrento Maternity Hospital, Moseley, Birmingham, UK. I am the youngest of five children: Angela, Sarah, Simon, David, and finally me – Adam.


At this time there was no suggestion that I could have hemophilia.  One of my cousins had been diagnosed with severe Christmas disease (now known as severe hemophilia B)  some years previous, but its heritable nature was not fully understood by my parents and extended family at the time, so everything seemed perfectly normal.  That is until at around six months of age I began to develop bruising around my ribcage, which raised the suspicions of our family doctor.

His immediate thoughts, however, were not to request the standard coagulation screens, but to suggest that the bruising was caused on purpose.  This was, of course, a horrifying thing to be suggested to my parents, especially as it was only a few years previous that my elder sister, Sarah, had died during surgery at the tender age of five months.  She had been diagnosed with Down syndrome, and also had a complication with her digestive tract which meant that each time she fed, she would regurgitate her food.  Thus she was slowly starving.  The operation was meant to rectify this and help her feed properly, but sadly she did not survive the procedure and passed away during the surgery.

With this in mind, and with emotions still raw, I cannot bare to think how my parents must have felt when a doctor was accusing them of physical abuse of their youngest child?  Whilst beginning to prepare the paper work for a referral to social services, the doctor then asked what he thought at the time was an unimportant question, but it was one which would have massive consequences on all our lives.  He asked if there was a family history of hemophilia.  My parents were somewhat surprised by this, but responded that the nephew of my mother had been diagnosed with Christmas disease a few years prior.  Upon hearing this, the doctor ripped up the paper work, and instead finally requested a coagulation screen.  When the results came back it was clear that I too had severe hemophilia B and the bruising was the result of normal handling of me by my loving parents.  And so my life, and the lives of my parents and siblings, would never be the same again.

I recall many occasions as a young child being with my parents and spending hour after hour waiting for the doctor on call at the hemophilia unit at Birmingham Children’s Hospital, often in great pain, and just as often bored stiff!  It was on one of these days that there was a new leaflet rack fitted to the wall in the waiting area with some new pamphlets neatly placed in it.  As I read the titles and looked at the pictures on the front covers, I noticed one which had the words Christmas disease emblazoned at the top of the page.  This caught my attention immediately as it was a label that I associated closely with, so I plucked if from the rack and began to flick through. Each time I returned to the unit I would head straight for that leaflet, and over time I learned more about Christmas disease and how it affects the ability of my blood to clot.

As a young child it always seemed harsh to me that my brothers were allowed to play football, rugby, and cricket, but I was not.  I yearned to understand why I had to have needles pushed into my veins, but they didn’t?  I did not have the knowledge and experience of my parents, so I could not see the ‘big picture’.  All I could see was that which was in front of me and feel the pain from the internal bleeding.  I was oblivious to the stress and worry my parents must have been feeling, having already had to bear the heartbreak of the death of one child with a lifelong condition, and now, here they are with another child who also has a lifelong and life threatening condition.  The fear and trepidation they must have felt is something that even today I cannot begin to fathom and only came to realise must have existed as I grew older.

As most of my formative years were in a constant state of flux between being in and out of hospital (which I’m certain many of my readers can relate to), I developed a deep rooted desire to understand why my blood was different to pretty much everyone else I knew (except for a few other people with hemophilia I would meet at the hospital from time to time). When new leaflets would arrive at the unit I would devour their contents, trying to increase my understanding of how the inner workings of my body differed to that of my brothers.  This desire to learn about my body ultimately led to me choosing to study physiology for my bachelor’s degree.  Fast forward to today, and I am a Senior Lecturer in physiology at the University of Sunderland in the UK – and I love it!  I have taught students of pharmacy, clinical physiology, chemical and pharmaceutical sciences, biopharmaceutical sciences, healthcare sciences, and biomedical sciences.  Of course my favourite lecture is the one I give to final year biomedical science and healthcare science students on the subject of thrombosis and hemostasis (with a heavy lean towards hemophilia)!

As an academic, and a person living with severe hemophilia, I have been able to develop an appreciation of bleeding disorders from these two, often polar perspectives.  Since my wife and I began our family, I now see it from a third perspective – that of a parent, as my daughter is a low level carrier.  Now I am beginning to gain an insight – albeit a glimpse – of how my parents must have felt as they did their best to guard and guide me through the early years of my life.  I don’t claim to be an expert on hemophilia – there is always so much more to learn as treatment and care is moving forwards at a tremendous pace.  I do, however, know how hemophilia has affected me and those around me over the course of my life.

Knowing how a clot is formed is one thing.  Knowing how it can go wrong and potentially be fixed is another.  However, no matter what you may know in relation to the science and treatment of this disorder, no one can ever truly understand how it impacts upon your life and the lives of those whom you love, except you.  Each person’s experience of living with hemophilia is different.  There is no ‘one size fits all’ perspective or path.  Thus how we come to terms with living with hemophilia, or any other bleeding disorder, is a personal journey; but a journey that we need not trek alone.  As the motivational speaker, Nido Qubein, once said “Your present circumstances don’t determine where you can go; they merely determine where you start”.  So, shall we start?