Same journey, different path

As a parent, as a mum, I’m going to try and articulate what my daughter Buffy’s diagnosis of Aplastic Anaemia means to me. Surprisingly I have found writing this very difficult - I normally have no issue talking about it - but writing my thoughts down is like baring my soul and forcing me to acknowledge and confront my feelings which isn’t easy – so doing this blog will also be cathartic.

My personal experience has been in dealing and coping with the descent into a world of horror and fear with my daughter staring death in the face but also the fantastic and the brilliant that has saved her life and in learning and trying to do something positive out of this dreadful situation

My beautiful, funny, clever, gregarious girl was enjoying life to the full, achieving everything she wanted and then found herself stunned into a life of impossible decisions, terrible statistics and choices a 21 year old should never have to make. This awful, devastating disease came completely out of left field and with very little warning but was life changing and life threatening; Buffy was extremely ill.

I’ve included some points that are poignant for me and I appreciate that everyone’s journey is unique; for me as a person, I have to know the detail, to know what I’m dealing with; forewarned is forearmed!

I should add that Buffy’s dad and I are divorced but both of our girls remained a priority so we quickly fell into a support routine for Buffy covering ‘shifts’ whilst also supporting each other and we never let Buffy out of our sight; we were probably being over –protective but we’re supposed to protect our daughter and we couldn’t - things were out of our hands and control for the most part.

Coming to terms with the diagnosis

From being admitted to hospital on 7thNovember 2017 until the official diagnosis of Aplastic Anaemia (AA) on 20thNovember 2017 the discussions had been around Leukaemia; however after listening and trying to understand what AA was and what the treatment entailed, I was initially relieved that it wasn’t Leukaemia but that was short-lived as we were told that as a disease AA is as serious as Leukaemia but more difficult to treat. Where do you go with that?

We’d never heard of AA and nobody else we knew had either and getting information was pretty difficult plus ‘anaemia’ sounds pretty innocuous so it doesn’t convey the seriousness of it.  We received very little in terms of information from the hospital and the Aplastic Anaemia Trust at the time could only offer very limited support.

I went straight into practical coping mode and my world closed in as my priority was in dealing with the situation we were in and caring for Buffy; I can’t really convey the reality of the day to day – the sheer stress and exhaustion and trying  to juggle full time work. I never allowed myself to cry and 15 months later I still haven’t – I used to feel that if I started I wouldn’t stop and because treatment is on-going I can’t let my guard down

What our new world looked like

There was no time to let things sink in as Buffy needed treatment immediately (she was diagnosed with Very Severe Aplastic Anaemia). We were suddenly caught up in a new world of blood tests, transfusions, Hickman lines and appointments for stem cell transplants and fertility preservation. It’s not my place to go into detail here but this was a vitally important and upsetting issue for Buffy: the effect her treatment would have. We are so grateful that she had the option of accessing this pioneering work which gave her hope for the future.

We started a diary initially so we could keep track of everything and now that diary is quite powerful as we can now see how far she has progressed.

The new terminology!

A reminder for my family & friends - in brief, we learned that ‘Idiopathic, Acquired, Aplastic Anaemia’ is a rare and serious blood disorder - an autoimmune disease whereby the stems cells in the bone marrow are destroyed thus affecting people’s ability to function and is quickly fatal without the correct treatment.

There are 2 possible definitive treatment options: the first choice was Stem Cell Transplant (Bone Marrow) which offers the only potential cure or secondly, immunosuppressant therapy - ATG & Cyclosporine. My eldest daughter was not a donor match unfortunately but the Freeman Hospital in Newcastle  did identify an unrelated donor. However due to the treatment pathway and how poorly she was, Buffy’s consultant at Sunderland Royal Hospital recommended her for ATG & Cyclosporine. Now the scary statistics: a transplant came with a 1 in 2 chance of survival and ATG with a 30% chance of success – not brilliant and we were torn. But we had to make a decision and of course we were guided by Dr Marshall whom we trust implicitly. We knew this wasn’t a cure.  Buffy started her treatment on 14thFebruary 2018 - a different kind of love for Valentine’s Day!

The rollercoaster 

As you can see there was so much going on and every day was different and a rollercoaster of emotions. The decline of her health was quick and her neutrophil count by now was zero and couldn’t be replaced with daily transfusions; we were constantly checking her temperature and worrying about her catching an infection and hospitals are the worst place to be!

In January 2018 a simple mouth ulcer developed into jaw infection or neutropenic sepsis led to 2 weeks in hospital. The first round of granulocytes - always a 5 day course given Tuesday to Saturday and with a 12 hour life span, often given at 11:30pm after being motor-biked from Manchester every evening – how incredible! A massive thank you to blood and platelet donors.

In the midst of this harrowing situation you see the wonderful - for me it was a juxtaposition between the worst and the best and I don’t know if I’m conveying the emotional aspects of that.

2 weeks later Buffy  was admitted to hospital with an abscess and bleeding in the bowels and the pain was unbearable: Buffy is normally very stoic, practical and level-headed but she was literally screaming in pain. This was utterly heart breaking and so distressing – I cannot put into words how that felt. It was at this point and for the next 9 weeks while she was in hospital that I stayed with Buffy every night – I couldn’t leave her and Buffy needed and wanted that support as she was very frightened. We have since talked about the fact that she thought she might die and was preparing for that and that was crushing to hear.

Buffy was very poorly again with neutropenic sepsis and as a last resort she received her 2nddose of granulocytes and the pain team from St Benedict’s Hospice managed this aspect of her care working alongside the haematology team. Of course there was the initial fear of the word hospice and although it’s because acute pain management is their expertise, it was also another sign of the seriousness of the situation.

It was during this period that there were 2 poignant moments for me: firstly when the registrar said that if the granulocytes don’t work then ‘we are struggling to treat this’ and secondly when the palliative nurse said to me:

I needed to remove all distractions including work because Buffy is very poorly, time is precious and you don’t want to have any regrets

That was shocking: I ended up taking 4 months off work to allow me to concentrate on caring for Buffy.

The reality now

So 6 months later she went into remission and treatment is on-going. However a couple of weeks ago she had a set back with a new anaemia and folate deficiency diagnosed (those old feelings re-surfaced) which will need treating before any wean off Cyclosporine can be considered. This means at least another 18 months of treatment - so disappointing and frustrating because she was starting to live life as a 22 year old should – with restrictions and limitations of course, and this is a reminder of how fragile she is.

It’s been very, very difficult emotionally for Buffy but my girl is gradually re-emerging. 

Life has been gradually getting back to normal (apart from the recent set back) and Buffy has had to re-think her career in the Police which is devastating enough, but she has returned to education and is working towards a Master’s degree in Counter Terrorism. She has passed her driving test and bought her first car and has the wonderful support of her boyfriend Jordan. I have absolute admiration for Jordan because he chose to stand by her knowing the difficulties she has and will face, and I am so proud of Buffy and she continues to amaze me for overcoming but still living with this awful disease.

What's helping me

This has changed me too - it’s been very traumatic and although I am recovering I’ve just had to lock stuff away in my brain otherwise I will go mad. There is no point worrying about what may never happen, but it lurks there in my mind and I try very hard to remain positive. AA will never go away so it’s a case of learning to live with it, respecting it but not being defined by it.

Work helps me focus and is a welcome distraction. It’s hard but nothing in life can be worse than what we’ve been through. I often sit and reflect and the situation is what it is – we’ve had no option but to face this head on but my emotions still seesaw daily and I can’t fully imagine how terrifying it must be to actually have AA.  People say I don’t know how you got through it but you have no choice. I have a wonderful support network as and when I need it and my colleagues at work have been kind and generous - it’s been humbling

I feel as if I have neglected my eldest daughter Kathryn. She wasn’t a stem cell donor match and this upset her and she would often get upset during conversations about Buffy. She lives 300 miles away which didn’t help in terms of being able to see her as often as I wanted to, plus she had personal issues to deal with as well so she ended up ‘retreating’ and keeping that to herself and importantly we have changed that.

The care Buffy has received has been utterly fantastic and is testament to the collaborative work of the NHS and I will be eternally grateful to the Haematology team at Sunderland Royal Hospital – they saved her life.

Grazina [our previous CEO] and the AAT has been a lifeline for me and as a result both Buffy and I have become involved in working on some of the initiatives in place, for example in trying to establish a pilot ‘support and outreach group’ here in the North East but it’s easier said than done and could easily be demoralising so a new approach and tactic is being developed by Grazina but it’s important to me to remain involved.

Grazina has transformed the AAT during 2018 making it accessible, relevant and professional: the support offered has vastly improved and is so much more informative. Grazina’s relentless pursuit of raising awareness and funds as well as expanding the network of expertise is vital in ensuring the charity remains sustainable.

The challenges we all face

The treatment for AA is very similar to that of Leukaemia and is equally as devastating but patients with AA do not have access to the same level of co-ordinated care, dynamic and relevant information, support resources and access to peer to peer support networks as cancer patients and we are trying to address that inequality – 14 months later Buffy has still never met anyone with AA – but the closed face book group provides invaluable support.

Government funding for research and emotional/psycho-social support for patients as well as information about rare bone marrow failures is extremely limited and existing NHS resources are targeted mostly at clinical treatment.

Grazina has kindly invited me to attend a reception in Westminster on 27thFebruary with her in recognition of Rare Disease Day so it will be a good opportunity to speak to MPs, and my local MP is attending.

Oh and myself and 3 friends are running in the GNR in September 2019 – my first time ever – inspired to raise funds and awareness for the AAT.

Blog by Tracey