Where angels fear to tread – by Kes Grant

In my last update I explained that I’d been an in-patient and they were treating me for pneumonia. It seems I have a fungal lung infection that then caused a bacterial infection. I was put on some strong antibiotics as well as a hefty dose of antifungals.

I got out of the hospital Sunday night which gave me a day to prepare for my friends funeral that I was taking. I felt pretty awful. I’m normally very quick in writing liturgies and personal eulogies. I’m lucky that I have this ability to sit and let it all come out in one stream. What I found was that I kept losing my concentration. The words would also start to swim in front of me on the screen. It was a strange experience. I kept at it but it took me twice as long.

The other thing I noticed was I was shaking. My hands had an obvious tremor. Also my legs were uncoordinated. If I had to step over something, I’d really wobble. On top of this when I was in the car, I’d look up and not recognise where I was despite it being a familiar road. In what is highly unusual for me, I asked my other half to drive. This went on for a few days. I felt like I was dying. I was uncoordinated and clumsy. I couldn’t concentrate and I was very shaky.

Despite all of this I was determined to take Eileen’s funeral. It was at a nice time later in the afternoon so I didn’t have to get it together too early. My lovely Maggie drove me to the crematorium and went for a wander in the grounds while I took the service.

When I write these liturgies I always print them in large font. This makes it easier to look up and connect with the people at the service and navigate your way back to where you’ve left off. This was working fine until we came to the hymn.

The family were keen to have a hymn because their mum loved music and singing and used to be on the rota to play the recorded accompaniment during our church devices. I told them all they needed to sing. The only problem was the backing track was a choral version that was set too high for congregational singing. Normally I would belt it out and try to encourage others to join in. Sadly though my lungs were not up to singing. The effort it took started making me shake from head to foot. The small print of the hymn words meant the words were swimming before my eyes and I couldn’t find my place. I had to grab the chair beside me at one point and I also gave up on attempting to sing.

It’s never happened to me before. I felt vulnerable in that moment and just decided to concentrate on my breathing. Fortunately I was able to dig deep into my soul and find the resilience and resourcefulness I needed to get through the rest of the service. I was so pleased to have pulled it off without incident or anyone noticing. I hope I did Eileen proud. May she rest in peace.

The next day I took it really easy and recharged the batteries. Then I had to go for a check up to the hospital. I explained how I was feeling and that I don’t remember ever feeling so frail and vulnerable. Normally I sleep like a log for 10-11 hours a night. I hadn’t slept for 3 nights more than a brief doze and this was also making me feel very spaced out. I told the doctor and it seems that the strong antibiotics were the culprits. Also the antifungals were causing problems so they changed both of them and I slowly started feeling normal again. It’s not until you’re really sick that you realise the huge impact that some of these life saving drugs have on the rest of your body.

The current additional meds will need to be taken for around 3 months. Healthy adults do not get fungal lung infections. It’s something that only happens to immunocompromised people like me. Fingers crossed there’s not too much lung damage.

I’m feeling slightly sad just now at the impact all of this has had on my voice. I had to give up so much over the years. Football was a hard one to let go of. Hill walking and circuit training and bike riding all have gone by the wayside. What I’ve held onto was my singing. The last couple of years with covid put pay to communal singing for me. The hard thing for me now is that I just don’t have enough puff. I’ve been struggling to get to the end of the line of music before taking a breath for a while. If you sing with others you can hide that you a breathing in the wrong places. These days though I can’t push enough air out of my lungs with my diaphragm to make a good quality sound. Music is the language of the emotions and it’s speaks for us when we can’t put into words how we feel. It’s also good for you mentally and physically. I really miss it and don’t feel I’m ever going to get back to be able to sing in cathedrals when my old choir covers the cathedral choirs for absence.

On this rare diseases day I wanted to share with you how debilitating it is to have a rare blood cancer. Then after transplant to have rare side effects like losing my hearing and the ongoing battle with GvHD (graft versus host disease) and now lung damage due to a fungal infection. All these things are rare and people like me are beginning this journey every year. The big cancers of breast, lung, bowel and prostate take the lion share of cancer funding. The rest of us have to fight our corners to shine a light on to the issues.

Please help us share our stories of living with rare diseases so that funding is given equally and also so that people begin to recognise the early symptoms of blood cancer. In case you don’t know what they are its:

• fatigue
• breathlessness
• bruising
• night sweats
• prone to infections

If you are experiencing any of the above, please see your doctor and ask for a blood test. This will be enough to show if there’s a problem that needs further investigation.

Thank you to everyone who has helped me with living with my rare blood cancer. My family and friends are an amazing bunch. I’ve also met many dedicated medics. I’m really proud to be part of MDS UK which does so much to demystify this appalling illness and bring comfort and support to so many. On this Rare Diseases Day 2022 I dedicate this blog to Sophie and all the staff and volunteers at MDS UK. You will never know the difference you make to so many. Keep going all of you, we need you and the world needs more people like you too.

In June 2015 I was diagnosed with MDS RCMD, it was something I had never heard of.

The only symptoms I had had up to the diagnosis was bad mouth ulcers, really big ones. This led my GP to run some blood tests which showed that my platelets and neutrophils were low.

I was referred to the Northern Cancer Care Centre at the Freeman hospital. It was there that I had the first of many bone marrow biopsies over the years. The first bone marrow biopsy confirmed that I had MDS RCMD.

My blood counts slowly dropped over the following years and my fatigue and infections increased. As my neutrophils fell below an acceptable level in 2019 I began a course of regular GCSF Injections. These gave my neutrophils a boost.

However, during August and September 2020 my white cells started to increase really quickly, a bone marrow biopsy confirmed that the MDS had transposed into acute myeloid leukaemia.

On the 5th October 2020, after becoming really unwell & losing a lot of weight, I was admitted to ward 33 of the Freeman hospital at 8am. I was fitted with a Hickman in theatre at 1pm. I had my first dose of CPX chemotherapy at 7pm that evening, it would prove to be a very tough 5 1/2 weeks.

I had intensive CPX chemotherapy doses but I didn’t lose my hair- this time. There were a few ups but mainly downs such as infections, Neutropenic sepsis, joint pain, nausea , severe mouth ulcers, rashes &, shall we say, the unmentionable ‘toilet issues’.

Due to infections I had to have x-rays, scans & ultrasounds to check where these infections were. It was also the only time I was allowed out of the ward.

My blood counts were basically obliterated by the chemotherapy so the leukaemia cells could be killed off. Blood & platelet transfusions were a regular, almost daily thing. Throw in some intravenous antibiotics & fluid drips plus regular obs. It felt like I was always attached to a machine.

Covid:"Nurses, Drs, radiographers and staff on the ward were amazing, always there for you"

Covid brought its own set of challenges. A couple of times I had a cough and was almost immediately popped into an isolation cubicle until the Covid test came back, negative luckily. With these spells in isolation & visitor restrictions & it got to be a lonely place at times.

However I always felt cared for, I was surrounded with love, affection & care from my family & friends through cards, messages and social media. The nurses, Drs, radiographers & staff on the ward were amazing, always there for you no matter what was happening.

Also the BMS lab staff were always there to process samples, bloods & the regular covid tests.

I found I could cope with the physical side of being very ill, however the mental side was much harder. It was made harder still with covid restrictions on visitors, but at least I could have one nominated person, the hardest part was speaking to my daughter and son in law via the phone, through the window, whilst they stood in the rain on the car park. That really crushed my soul and spirit. However the nurses and Drs were always there with their unending support. I love them from the bottom of my heart. I cannot thank them enough.

"My preparations to have my stem cell transplant"

I came out of hospital on November 10th 2020. On the 16th November 2020 we met with the transplant team at the Freeman to discuss my preparation to have my stem cell transplant. During my stay in October we had spoken about my transplant and possible donors, they’d got several 9 out of 10 donors but had asked if my daughter, Aimée, could be tested to see if she could be a HAPLO match. I also signed the treatment consent forms and what seemed like a contract that I would finish the treatment.

The following day I had another bone marrow biopsy to ensure the CPX chemotherapy had killed off all the leukaemia cells.

On the 19th November Aimée had a medical to see if she was able to donate.

On the 25th November we attended the Freeman for my “work up” chat. I had lots of bloods taken approx 14 vials, then spoke to the radiologist to talk through the risks of the total body irradiation, TBI (Editor's Note: TBI is a type of radiation therapy, given to the entire body. TBI is often used with high-dose anticancer drugs to help prepare a patient for a stem cell transplant) and to sign the consent forms.

Then it was the heart unit for an X-ray of my chest, next up was a lung function test, then across to another department for an ECG. After that it was off to the radiology department to be measured up for the TBI,they tattooed a small dot on my chest ready for when I would have the TBI. It was good to have my wife with me, it helped to process all the information we were being given.

On 27th November I had a new Hickman line fitted at the Royal Victoria Infirmary, RVI in Newcastle. All went to plan, it was a worry having it fitted but I knew it would be a blessing when I was going to have my treatment.

On the 28th November - 1st December Aimée had to go to the Freeman for GCSF injections to boost her cell counts and on the 2nd of December Aimée donated her cells, what a special thing to do. Aimée was very brave to go through the donation process.

I was re- admitted to ward 33 on the 4th December, this time to an isolation cubicle, cubicle 2. That evening I was given my first dose of chemotherapy, fludarabine and cyclophosphamide.

The following day, 5th December, I had my 2nd dose of chemotherapy, however this time I had a reaction to the cyclophosphamide so they slowed down the drip rate and gave me an antihistamine.

I had my 3rd dose on the 6th December, this time just the fludarabine. The following day, 7th December I had my 4th dose of fluradarabine chemotherapy, I was now starting to feel nauseous, however I hadn’t been sick.

The next day I had my 5th dose of fluradarabine chemotherapy, I was starting to feel quite unwell.

On the following day, 9th December, I was taken down for the total body irradiation, 8am sharp. I was placed on the top of what appeared to be a bunk bed. I then had extremely cold Vaseline packs packed around me. I was irradiated on one side then they turned the bunk bed around and they did my other side.

When I got back to the ward I was completely shattered, I slept all the rest of the day and night.

10th December and it was transplant day, finally I got to receive the cells Aimée donated, well some of them anyway. Some are kept aside in case I need a top up.

The transplant went ok, it was just like having a blood transfusion. The CNS transplant nurse was with me, also a chap from the labs from the RVi who had brought over Aimee’s cells in the deep freeze pod unit.

The following two days I was completely wiped out, I was hardly able to get out of bed.

During the following weeks, I also got a small bleed behind my eye, I had to go to the eye department at the Freeman. The bleed was due to my platelets being so low, at one point they were just 12. I needed quite a few platelet transfusions to ensure my platelets were kept at 50.

I also lost my hair due to the chemotherapy, for some strange reason it didn’t bother me. I got further infections but the staff were onto them very quickly with intravenous antibiotics. The staff were absolutely wonderful, always there for me, their professionalism was first class. I don’t know where I would have been without them.

On the 13th & 14th December I had a further two doses of cyclophosphamide chemotherapy. During this period I managed to get an E. coli infection and my temperature spiked to 38.5. I felt terrible.

On the 15th I started on the anti rejection drugs, Tacrolimus.

On Christmas Eve morning I dressed up as Santa, stepped out of my cubicle and just said ho, ho, ho, it really brought a smile to all the nurses at the nurses station. It was shift change over, so they were all there. I felt awful really unwell but I was determined to do it. Just for them.

I was in isolation for approx 5 weeks, I spent both Christmas and New Year in cubicle 2. I came out of hospital On the 6th January 2021, it was such a relief to come out but there was still a long journey ahead. That’s for another time.

I’d kept a journal of my time in hospital and my continuing journey afterwards, it often saved my sanity to be able to download all my feelings onto paper. I could cope with the physical side of being ill, however the mental health side is just as difficult to get through, perhaps even harder.

Today marks 65 days since my husband Neil lost his battle with this challenging disease, a disease where every single case is different, making it very hard to fully diagnose and treat. It took us weeks to get our heads around it (never mind learn how to pronounce its full moniker, Myelodysplastic Syndrome, correctly — Neil spent the first couple of weeks referring to it as ‘Melody’s Plastic’).

After months of debilitating fatigue and being unable to shake off a nasty covid-like virus (contracted in October 2019, months before covid was reported to have hit the UK), Neil was diagnosed in February 2020, a few weeks before lockdown one.

The diagnosis came as a body-blow to him, to both of us — after a short spell in hospital and a course of antibiotics he’d been feeling well again, and had thought the clinical haematology appointment was a post-discharge formality.

The worst thing he expected was a flea in his ear about leaving it so long before seeing the GP about his fatigue, persistent cough, putty-coloured pallor and unexplained weight loss. To be told he had a rare form of blood cancer came from nowhere, and hit us like a bag of wet cement.

So began a year of fortnightly blood tests, blood transfusions and further bouts of overwhelming fatigue. Neil’s consultant referred him to UCLH for a possible stem cell transplant immediately after diagnosis, but partly due to covid, the referral wasn’t picked up until the October. By then Neil had undergone two courses of azacytidine, which he’d tolerated well. Two 100%-match donors were identified just before Christmas last year, and his transplant was scheduled for early in the New Year. Then the first donor dropped out, possibly got cold feet about attending a hospital in the midst of a pandemic. The second donor stepped up, and the transplant was rescheduled for early March. Then UCLH checked his ferritin levels — after a year of iron-rich blood transfusions these were found to be stratospheric, so the SCT was put on the back burner in order for these levels to be brought under control. Sadly, this wasn’t a treatment routinely offered by our local NHS Trust due to funding issues. (If you’re having regular transfusions your iron levels should be monitored — Neil’s weren’t. If yours aren’t, talk to MDS UK).

In late February Neil went up to London for a ferriscan, a type of MRI designed to identify the amount of iron being stored in the major organs. After the scan — which left him feeling a bit sick and as if he’d been ‘pummelled’, he went to the main hospital for a blood test and platelet transfusion, but began to feel ill on the way home. A few days later he was admitted to our local hospital with suspected neutropenic sepsis. He came home after a fortnight, the transplant was rescheduled for April, and the iron chelation started in earnest. However, his MDS began to progress and his counts plummeted. He began to receive blood and platelet transfusions more frequently, meaning regular trips to and from London, often by public transport (during a pandemic!). He kept getting infections, spending up to five weeks at a time in hospital, fifty miles away from home, when, thanks to covid he could only have short visits from me once or twice a week. These hospital stays often left him depressed, due to lack of outside contact, sleep deprivation and the hospital food (which was, frankly, nothing short of disgraceful — paltry, cold, dry, of little nutritional value and often inedible).

To cut a very long story short, Neil’s transplant was postponed four times. He died five days before what should have been ‘day zero’. The gods had more in store for us — a few hours after Neil passed, in intensive care in UCLH, my mother died too — 82 years old, she’d been suffering from a bad chest infection and the news about Neil proved too much. She was over 300 miles away in the North-East. Weirdly, she, too, had been diagnosed a few years ago with low risk MDS, which was managed successfully with EPO injections that she administered herself every two weeks. It wasn’t a factor in her passing, but a cruel twist of fate that the same rare blood cancer affected two members of our small family. Maybe it’s not as rare as we think — all the more reason to raise awareness and campaign for further research.

Neil’s case has turned out to be complex, and there is to be a full inquest into the sequence of events before his passing, to take place in February 2022. However, before contracting what turned out to be a final, fatal bout of neutropenic sepsis, Neil began to respond really well to venetoclax and aza — his counts improved dramatically and he had more energy than he’d had for months.

Just a couple of weeks before he passed he actually began to feel like himself again — it is thought his sudden downturn may have been due to something else that hadn’t been picked up. There truly is hope to be had in the new therapies coming along the pipeline. If your local NHS Trust doesn’t appear proactive or cooperative when it comes to new drugs, treatments or clinical trials (ours wasn’t, at least not for MDS) get yourself referred to a centre of excellence or research hospital, and ask about shared care. MDS UK are there to support you, should you meet any resistance.

We asked for donations to MDS UK at Neil’s and my mum, Joan’s, funerals, which raised over £900 for this amazing charity. I cannot overstate how much a difference it made to us to have the support and friendship of the MDS UK community during Neil’s journey, I will be eternally grateful for it, and as a family we will continue to support their work. It may be too late for Neil, but we hope that the detailed examination of his case may throw some unforeseen light onto lesser-known elements of the disease, and how it progresses, or impacts other underlying or undiagnosed health conditions.

May we send our love and strength to every one of you touched by this disease, whether you’re the patient or a carer or a loved one of someone going through it. I resisted posting in the MDS groups about Neil’s passing, as I know how soul-destroying such stories can be to read. But every single case is different, and one person’s story isn’t going to be the same as another’s.

Neil thought he only had weeks left when he was diagnosed, but it was nearly two years from when he first became noticeably ill to when we lost him, and we had many good times in those two years. He met some incredible people on his journey, and developed new levels of patience, resilience and humility along the way. If he’d managed to stay infection-free for just a couple more weeks he would have had his transplant — the donor cells were there, ready, on ice — and he could have been living a normal life again by Christmas. The new drugs he’d started were working, they just took longer to kick in for Neil than he’d needed.

Don’t ever lose hope!

"My path to an MDS diagnosis is perhaps unusual. In 2016 I noticed that could not feel the top of my left foot. A visit to the GP plus a blood test resulted in an appointment at the local hospital."

After further tests, Alastair was referred to King's College Hospital where he was diagnosed. He is currently in a low-risk category, on watch and wait, with monthly blood tests and regular checks at King's.

A passion for running

I have always tried to keep fit with running, cycling, sailing and hiking.

In 2018, two years after my diagnosis, I celebrated forty years of running with a worldwide non-competitive social running club called Hash House Harriers having nearly reached a total of two hundred Parkruns. On that year I also run my very first marathon.

Since many patients are far worse off than me, I started raising more money to add to the charity funds, increase awareness and support sufferers of MDS.

In 2020, despite the pandemic, Alastair smashed his 2020 running target with one day to spare. In fact, after surpassing 2500km, he thought he could reach 4750km. Ultimately, he ran an incredible 4759km!

My MDS story began just under two years ago. I was a normally healthy 71 year-old (or so I thought): I had no symptoms and I was still jogging a couple of times a week. I just happened to have a random blood test which showed that I had a low white blood cell count.

My doctor son saw the results and strongly advised me to consult a haematologist. That led in turn to bone marrow tests, which showed that I had high-risk MDS (MDS-EB2).

After a stem cell transplant in May 2021: "I’ve been very fortunate"

The pandemic delayed some of my treatment, but eventually, after five cycles of Azacitidine (plus Venetoclax), I had a bone marrow transplant (from an unrelated donor) at King’s in May 2021.
 
Since being discharged nearly three months ago, I’ve had a couple of weeks back in hospital with infections, but generally I’ve been very fortunate, avoiding the worst of graft versus host disease (GvHD).
 
My post-transplant blood and bone marrow test results have been positive and, all being well, I am looking forward to getting my new set of Covid jabs within the next few weeks.

Sam Astley opted to donate his stem cells instead of attending the England football match!

Sam decided to register as a stem cell donor, thank to our long term MDS UK Patient Support Group member Simon Wilkes, who was diagnosed with the rare blood cancer Myelodysplastic Syndromes several years ago.

I'm Kate, 48yrs from Yorkshire, recently diagnosed MDS RA-RS in July 2020 - found out by complete accident as I had no symptoms.

January 2020 - went to the Dr as my chiropractor asked me to get a blood test to check for inflammatory markers - I had an ongoing severe muscle spasm in my shoulder. I think she was thinking rheumatoid arthritis or some other issue - I have arthritis (shoulders/hips) but that's mainly wear and tear and have other joint issues as I was a jump-jockey in my younger years - fell off a lot at speed! Self inflicted torture? depends how you look at it 😜 .

Anyway - the bloods came back anaemic but with higher iron than is normal - so by March 2020 I was having another blood test - I wasn't worried - didn't have any symptoms and was just a bit tired sometimes. I am currently a Pilates Instructor/Personal Trainer so I am fit and healthy! Well... so I thought!

I haven't let Covid stop me working and I am now teaching online (ZOOM) pilates to a few small groups - I am happiest when I am helping others. 

I am a lower back pain focused teacher and have several clients who have survived breast cancer and some who have managed to reverse their osteoporosis.

Read an article on iron chelation therapy, written by Bergit Kuhle on our MDS Patient Support Group Newsletter May 2018