Richard

Richard shares his experience of high-grade B-cell non-Hodgkin lymphoma with central nervous system involvement, also known as primary central nervous system lymphoma (PCNSL).

Richard with his bike

I hope my story may be useful to someone out there, patient, carer or medical professional. My illness is an exceedingly rare cancer and I understand that only 4 or 5 patients are diagnosed with it in the UK every year. I could certainly only find extremely limited material on my illness on cancer websites, so this may help to fill a gap to someone. Also, much of the story is of a generic nature so would be of interest to anybody involved with the long-term treatment of cancer and disorders of the central nervous system in the NHS and the trials and tribulations which that entails.

I was 53 when I became ill, 183 cm in height and 84 kg in weight.

It is difficult to know when it all started. I have always maintained a good standard of fitness and have always endeavoured to get to work no matter how bad I am feeling. At the time my illness developed I was training for a 300km cycling endurance event, the Rough Diamond Audax at Tewkesbury on the 11 August 2012. I would cycle to work from home in Malvern two or three times a week, which is a round trip of 50 miles, and go for a longer ride at the weekend with friends up to 70 miles, usually involving hills. Two of the main symptoms of cancer are sudden loss of weight and feeling fatigued for much of the time. This is also a symptom of long-distance cycling training so I suspected there was nothing untoward and continued training. I promised myself a rest in the week leading up to the event. However, I did not make it that far.

 
I certainly did not think that would be my last requirement for a haircut for over a year; the chemotherapy would take care of that.
Richard

On 3 August I returned home from work in my car and went for a haircut at the local barber shop. I can remember feeling exhausted as I sat in the barber’s chair and making a mental note to get an early night to maximise my sleep. I certainly did not think that would be my last requirement for a haircut for over a year; the chemotherapy would take care of that. The next morning, I joined my two cycling friends for a ride. I felt OK, apart from when we stopped for a cup of tea when sitting down for 20 minutes caused me to feel dreadful, or more specifically, nauseous and exhausted. In fact, if my wife had not been at work, I may have telephoned her and asked her to come and collect me and the bike. However, I had no other option but to climb back on my bike to complete the ride home. After about 15 minutes back in the saddle and warmed up again I actually felt OK and managed a steep hill with ease. I arrived home shortly afterwards and apart from the usual tiredness I felt OK.

On Sunday 4 August I cooked Sunday lunch for the family and my legs felt unusually weak and tired but nothing to complain about. I decided to stay off the bike all week so my legs would be well rested for the event on Saturday. On Monday 5 August I drove to work and noticed something strange about myself. Normally I am ravenous by lunch time at work, but for some reason I had lost my appetite, another classic cancer symptom. I had arranged to meet my Project Manager for lunch and really struggled to eat my lunch, which I did not manage to finish. This is such an unusual occurrence that I should really have realised that something was wrong. I came in to work the next day and started to experience severe headaches. I attended a meeting in the afternoon and found that sitting down was hurting my legs and I could not concentrate. I made my apologies to all my colleagues and left to go home. 

The next morning, I went to see my GP. He thought I had a virus and told me to take a week off to recover. At least he had more sympathy than my wife who had diagnosed me with ’man-flu’! The symptoms became worse and by 8 August my legs were so weak, I could hardly stand up, my headaches were persistent and now I could not even see clearly. My wife contacted NHS Direct on the telephone and they told me to get over to my local A&E immediately. So my wife took me straight to A&E and I was immediately triaged by a nurse then a doctor. It was during the triage that I discovered my poor vision was due to the fact I had double-vision and was seeing two fingers when the doctor held up one. This probably explained the headaches. My initial assessment had raised the alarm and various consultants were discussing the fact that I may well have a tumour or something similar inside my head causing these problems so off I went for scans. The A&E was particularly good. There was no waiting about at all. They realised the seriousness of my condition and acted on it straight away. Without a clear diagnosis of my illness I was admitted to the acute medical unit (AMU) for further medical investigation.

It was here that I first experienced a lumbar puncture which was not a great experience. I was told I would be injected with local anaesthetic in my lower back area and when it became numb, a needle would be inserted into my spine to extract cerebrospinal fluid for analysis in the lab. There was pain in my right leg during this process and the needle was withdrawn. The lumbar puncture was unsuccessful as not enough fluid was extracted. I was moved out of AMU after a day and found myself in the Infectious Diseases Unit or IDU as it is more commonly known.

To the IDU I was something of an enigma. My condition was worsening by the hour. Not only were my legs so immobile that I now needed a Zimmer frame to walk, but they were also extremely painful, particularly my feet.  At nights I needed to take a painkiller to suppress the incessant tingling and to allow me to get to sleep. I later found out that the tingling pain was nerve pain. Also, my face started to collapse on the right-hand side just like a stroke victim. The consultants and doctors would stand round my bed and discuss my case and ask me all sorts of random questions.

  • ‘Do you eat a lot of soft cheeses?’
  • ‘Do you keep animals?’
  • ‘Have you travelled to Africa recently?’

The random nature of their questioning made me realise that they did not know what was wrong with me. When I asked for a diagnosis, they told me that I might have tuberculosis, lupus, meningitis, encephalitis, listeria, Weils Disease and so on. To try and prevent my condition worsening further they gave me industrial doses of antibiotics intravenously and orally. At this stage, the neurologist was sent for to examine me. I can remember this day clearly, because my face had collapsed further and my appearance had become quite disturbing to my youngest daughter and a good friend who came to visit me. It was a relief for them to be sent out of my isolation ward while the neurologist examined me. After spending a long time bashing my legs with rubber hammers and testing my reflexes the neurologist admitted that he was baffled by my condition but was going to request that I was moved up to the Neurology Ward.

 
A huge amount of effort was going into my diagnosis behind the scenes as cultures were grown from my blood samples and the doctors were in constant contact with my own GP surgery to investigate my old medical records.
Richard

My family were starting to become very worried. I had now been in the IDU for over a week and the doctors were no nearer to diagnosing my condition, despite all the blood tests, MRI Scans, CT scans, and  a brain sensor test conducted by physiologists. My condition was deteriorating on a daily basis and now my speech was becoming incoherent as the muscles round my face failed to work properly. A huge amount of effort was going into my diagnosis behind the scenes as cultures were grown from my blood samples and the doctors were in constant contact with my own GP surgery to investigate my old medical records. It was starting to develop into an episode of ‘House’ where diagnosis evades Hugh Laurie until the last 5 minutes and there is usually a happy ending, although I was far from convinced this would be my outcome. Indeed, I was starting to believe that I would not survive and started to have some difficult conversations about the future with my wife and pragmatically, started to put things in order for this eventuality. My solicitor visited me one evening and updated my will.

It was decided that I would have a lumbar puncture followed the next morning by a PET scan. The lumbar puncture took a sample of cerebrospinal fluid from my central nervous system by inserting a large needle at the base of my spine and syringing out a sample. This was achieved eventually and the sample was sent away to the laboratory for testing. Meanwhile the senior consultant told me that she was investigating blood disorders as a possible cause of my illness.

The PET scan was to be carried out in another hospital, and on the morning of my PET scan I had to abstain from eating or taking oral medication in the morning which caused me considerable pain and I had to be given an injection of morphine for the ambulance journey. I arrived back that evening exhausted and immediately went to sleep. I was woken up late at night and told that I would be moving down to the haematology ward immediately and would receive my diagnosis the next morning from a haematology consultant. I did not know much about haematology apart from the fact it was something to do with the blood. I feared the worst but was grateful that I eventually had a diagnosis.

 
I feared the worst but was grateful that I eventually had a diagnosis.
Richard

Saturday 25 August was the Summer Bank Holiday weekend. I had asked my wife and two daughters to come into hospital so that we all received the diagnosis together as a family and could put any questions to the consultant. That was the first meeting with the consultant, and he was to stay with me for the next year and met with me at six-monthly intervals for follow-up appointments. I was incredibly lucky to have such a brilliant doctor assigned to my case. He was like a navigator for a giant container ship, slowly navigating me through the ups and downs of cancer treatment and care. He delivered my diagnosis in a very professional and direct manner, allowing time for my daughters to have their tears. He told me that I had been diagnosed with high-grade, B-cell non-Hodgkin lymphoma with central nervous system involvement. This was both progressive and aggressive. This was indicated by laboratory analysis of the cerebrospinal fluid from the lumbar puncture which had indicated a density of greater than 900 lymphocytes when there should be less than 5.  

 
I was to learn that having confidence in our well-trained, experienced NHS professionals goes a long way to keeping your stress levels down.
Richard

Due to the aggressive nature of the illness, treatment would start immediately with intrathecal chemotherapy consisting of cytarabine, methotrexate and hydrocortisone to attack the problem straight away. This process requires a large needle to be inserted into your spine and the chemotherapy being injected. I found this to be scary, but was heartened by the fact that an experienced, highly competent registrar administered the treatment. I was to learn that having confidence in our well-trained, experienced NHS professionals goes a long way to keeping your stress levels down and contributing to a faster recovery. There was some immediate improvement in neurological symptoms, my speech and walking improved. This was followed by an IDA-RAM chemotherapy regime. All weekend I was busy having a PICC line fitted, having X-ray scans and ultrasound scans to get my treatment underway. I was both surprised and delighted how many of the haematology doctors were on the ward on the bank holiday weekend providing treatment for me. I needed lumbar punctures to have the chemotherapy applied intrathecally into the area at the base of the spine. I had two difficult lumbar punctures in AMU and IDU and was amazed how painlessly and effectively this was administered in the haematology ward. I felt reassured to be in the haematology ward because the doctors really understood my illness and displayed a high level of practical ability, while the level of care displayed by the nurses was exemplary.  I was learning that a lot of supportive treatment was involved including regular use of eye drops and four different types of mouthwashes with regular anti-sickness tablets.

 
I felt reassured to be in the haematology ward because the doctors really understood my illness.
Richard

This was my introduction to cancer treatment, something I had not expected and was not prepared for.  My mind raced through all the normal questions:

  • Why me?
  • What have I done to deserve this?
  • Will I survive?
  • How will my family cope?

I decided that I was going to maintain a positive attitude throughout and somehow get through this. I asked her what the cause of my illness but to this day I am none the wiser.  

The first treatment of chemotherapy was difficult. I experienced a lot of pain in my lower jaw in combination with mucositis. I visited the hospital dentist and had a tooth removed from my lower jaw after the chemotherapy had exposed an abscess underneath it. I also went neutropenic for the first time and realised what it was like to lose your immune system and pick up a nasty infection. In this case, c-difficile.

I was discharged on 11 September 2012 to go home for a break at home before I was re-admitted on a week later for more chemotherapy. This time I was fitted with a Hickmann line into my chest following a minor operation administered by a team of anaesthetists. This line became a very close companion to me over the next 10 months.

I then embarked on the recommended protocol of chemotherapy treatment for my diagnosis, starting with R-CODOX-M, then R-IVAC, followed by another R-CODOX-M, then finishing with R-IVAC. I did find all these cycles of chemo very hard and did not feel that I coped too well with them. At the end of each cycle I would go neutropenic, lose my immune system, pick up an infection and become extremely ill. The doctors would then hit me with a strong dose of anti-biotics and I would recover enough to go home for a few days, put some weight on, ready to face the next cycle of chemo. Sometimes I would become ill at home while still neutropenic. On one occasion I became so ill that I could not even walk and an ambulance had to be called. The ambulance men had to put me in a stretcher to get me down my stairs into the ambulance and into A&E. I was probably down to 70 kg in weight at this stage and very weak. This time I was diagnosed with sepsis, which I later found out is a particularly nasty, and potentially life-threatening infection. 

One night in my isolation ward I listened on the radio to Andy Murray battling against Novak Djokovic to win the US Open Tennis Final, his first major win. That was inspirational for me. I was so impressed with Andy’s resilience in overcoming a formidable opponent and achieving a victory that I decided I would follow his example and overcome a formidable opponent in the form of my lymphoma and achieve my victory by getting into remission. Since then I have followed Andy Murray’s career closely and have become a huge fan of his.

The chemo treatment continued until 17 December 2012. By this stage the consultants were telling me that I was coping well with chemo and suggesting to me that if I went for a stem-cell transplant that would provide me with the best chance I could have of staying in remission. Initially I resisted this strategy because I did not think I had coped well with chemo so far and stem cell therapy chemo is a lot more severe. On the 24 December 2012 I was back in the haematology ward with a high temperature caused by two nasty infections. I spent the most miserable Christmas of my life in hospital fighting the infections and regretting the way I had messed things up for my family. When I became ill, I really did not want to go in to hospital but thankfully my wife persuaded me to. The consultant told me that if I had delayed coming to hospital for another few hours, I might not be alive! During this unplanned stay in hospital I had managed to canvass the opinions of as many doctors as I could about the merits of the stem-cell transplant and they were unanimous in their opinion that this would be the best thing for me. I therefore opted for this treatment. I then had a couple of meetings with the senior consultant who was initially reluctant to let me have this treatment because I had lost a lot of weight, I was weak and my mobility was poor due to my dropped foot and weak legs. However, I went home to recuperate and did lots of physio and ate lots. When I next saw the senior consultant, he approved my improved physical state and pencilled me in for a date in March 2013.  I continued to eat like a gannet and managed to get up to 80 kg in body weight.  

 
I had managed to canvass the opinions of as many doctors as I could about the merits of the stem-cell transplant and they were unanimous in their opinion that this would be the best thing for me. 
Richard

I also persuaded my wife to take me out for a few trips as I was going stir crazy at home. I had fallen ill during the Olympics so it was fitting that my wife took me to visit one of the gold-painted letter boxes dedicated to local Olympic Champions; Charlotte Dujardin from Newent in this case.

To have a successful stem cell transplant required the harvesting of my own stem cells to store and then put back into my system after the chemo to give my bone marrow a fighting chance of regenerating and producing the constituents of my blood again. The stem cell harvesting took place in the haematology clinic, following artificial stimulation of the bone marrow by multiple injections to the stomach. The nursing staff were excellent on the ward but I still needed a return visit to collect a satisfactory amount of stem cells. For the second attempt I had an injection of a drug called perlixoflor, which despite giving me diarrhoea for the next hour, seemed to boost the collection of stem cells. Now I was ready to go and was just waiting for a bed to become available.

I was admitted on 25 March 2013 for an autologous stem cell transplant, using my own stem cells, and started chemotherapy the next day. Instead of the normal BEAM cycle, I was given chemotherapy tailored to an involvement of the central nervous system and the regime consisted of first Carmustene, then Thio Tepa. To pass the time I searched the internet to read about the history of chemotherapy, which in hindsight was not a sensible thing to do while undergoing treatment. However, it did help me to understand some of the symptoms that I was experiencing. I also discovered that the hospital had a specialist team set up to deal with facial palsies and managed to persuade my ward doctors to get me a referral following my treatment. After 3 weeks I was discharged which was much better than the projected 4–6 weeks I was expecting. I had to return to hospital every Wednesday, to be weighed, have my blood analysed and then talk to the consultant. My blood levels were low and sometimes I would have to go for a blood or platelets transfusion.

I continued the long recovery from stem cell transplant at home while getting physiotherapy for my legs and foot and trying to build up my body by eating more and getting some quality sleep. I started to feel a bit better week by week but my blood levels were taking a long time to recover, even with the assistance of regular injections of GCSF. My wife and I felt confident enough to go for a five-day holiday in the Isle of Wight at the end of June which we both thoroughly enjoyed. I probably tried to do too much, and walked further than I should have done, which resulted in my blood levels plummeting. I was scheduled for a PET scan in July but unfortunately contracted an infection so had to cancel this. I eventually managed to reschedule my PET scan and waited to see my consultant to get my results. However, nothing ever goes according to plan and I was back into hospital as an in-patient with another infection. 

I finally met up with my consultant in late 2013 and he was able to tell me that I was now in full remission from the lymphoma and the PET scan had shown no signs of secondary cancer. Great news. So now I faced the next challenge…getting back to work and earning an income again to support my family. I was hoping to achieve some form of normality, whatever that now looked like.

 
So now I faced the next challenge...getting back to work.
Richard

My employer had been extremely understanding and told me that when I was ready, I could have a progressive return to work and start on one day a week. So, once I was able to drive again, I started back to work and built my time up to full-time.

By 2015 I was back working with my old clients again and doing some really good work. I surprised myself by getting promoted in 2016 to Senior Manager.

I was starting to realise, however, that following my many treatments I just did not seem to have the same energy levels at work as I used to have. I needed to embark on a campaign to strengthen my body and mind and reduce my hours at work. I realised that I could not work full-time anymore.

I did more physiotherapy, more exercise and went to bed earlier to boost my energy levels. I am lucky that my wife is an extremely good cook and made me some lovely nutritious meals with lots of fresh vegetables. I also drank much more water and less coffee during the day at the suggestion of my daughter. My physiotherapist suggested I joined a yoga class which has been extremely beneficial to my body and soul. Over the last couple of years, I have taken up golf and racketball for exercise and social purposes. I have also done an oil-painting course, a ceramics course, and a drawing course to enhance my creative side and have now been singing with my local choir for two years.

All of these activities have benefitted my holistic wellness and have provided me with stimulation, which is important. My consultant recently told me I have exceeded all expectations with my recovery, so I am doing something right.

I am grateful to my family, who have supported me magnificently through my illness and to the many NHS professionals that cared for me so wonderfully. When I was a hospital in-patient the news was dominated by the Mid-Staffs enquiry. I wanted to shout at the BBC that my NHS was doing a brilliant job looking after me and it was not fair that news coverage only covered the bad things and not the great work that most of the NHS was doing.  At least the COVID-19 crisis has now redressed the misperception of our amazing NHS, so that all the UK holds them in high esteem.

 
I am grateful to my family, who have supported me magnificently through my illness and to the many NHS professionals that cared for me so wonderfully.
Richard

I am now 61 and retired from work. I am in reasonable health for a man of my age, although my immune system is low and I have to be careful about infections, particularly in the winter. I have lost the use of some facial muscles and my non-verbal expression has suffered. I have a limp in my right leg and double vision, which my optometrist has treated with prescription spectacles. Apart from that I am OK and enjoy my retirement, living sensibly and responsibly. Even in lockdown when I wrote this piece!

So, what have I learned from this experience?

While being treated in hospital:

  • You should never give up on yourself. The NHS are amazing and will do their very best to help you.
  • Nothing ever goes to plan, that’s life. Your stay in hospital and probably afterwards, is all about how you deal with the inevitable setbacks that you will face. Accept that things will not always go to plan, be proactive and think about what you or someone else can do to help. I call this setback management.
  • Never feel sorry for yourself. It does not help you. Stay positive. This will help you, your family and friends, and the NHS staff trying to help you.

Following discharge from hospital:

  • Make a list of all the things that you want to do but have never had the time. In my case, examples were, visiting all the Norman Castles in Wales, visiting the Scottish Borders to see the autumn colours and indulging myself at the International Cheese Festival in Cheshire.
  • Also, make a list of things that you really enjoy and ensure you do these on a regular basis. Some examples were coffee and cake at nice cafes, cycling with friends, walking round National Trust properties and attending organ recitals at local cathedrals. All these things are guaranteed to put a smile on my face when the need arises. Even watching Countdown and competing with my wife to find the biggest words and solve the tea-time teasers is an easy thing to enjoy.  Unless she beats me!
 
You should never give up on yourself. The NHS are amazing and will do their very best to help you.
Richard

I conclude this piece by thanking my family and friends, the NHS, a local hospice and the Lymphoma Association before it became Lymphoma Action for all the help I received from them.

Most of all I would like to thank my dear wife Sharon. I would certainly not be around to write this article if it were not for her never-ending and unflinching support.