Rare Diseases Day 2021

Today is Rare Diseases Day. MDS or to give it its full title myelo dysplastic syndrome is rare enough. Within that group diagnosed with MDS there are many subgroups. Mine is RCMD which stands for refractory cytopenia of multi lineage dysplasia. On top of that I have hypo cellular bone marrow and an autoimmune component to my illness. If you are already a rare group the subsets are going to be even rarer. This can bring about many challenges. We are all unique and respond differently but sometimes safety in numbers has its advantages. We only need to look at the current pandemic to see that in action. In order to get clinical trials through and to get good quality data you need as many participants as possible. With Covid19 you can get 100s of people every day. This has enabled the science to move on in leaps and bounds. That's not the case with rare diseases. A GP would be lucky to see just one case of MDS during their career. Local hospitals may only have a few patients. Thats why it's so important to have an addition opinion from a Centre of Excellence where they see most patients. I'm really fortunate to be looked after by Kings in London.

I've been dancing with my cancer for 21 years now. I had a stem cell transplant in 2016. I'm still living with the complications of the cancer and its treatment. I say I dance with my cancer because I don't like the battle terminology. When people say so and so lost their battle with cancer, it makes me shudder. People don't lose their battles. My experience is they struggle on right up to the end giving it every bit of strength and courage they have. If you go into remission, it doesn't mean you've won and those that don't win have lost or not fought hard enough. It just means the cancer was overwhelming. Cancer is an awful thing and very unpredictable for some. The dancing imagery suits me better. Sometimes I'm dancing so fast that I can hardly breathe and after that particular music stops I collapse into a chair exhausted. This might be dealing with yet another infection that has led to sepsis, which I've had many times. Other times there might be months go by where all I can do is be held up by the love and support of who is dancing with me and slowly shuffle my feet. Sometimes I waltz with my medics going this way and that as we try different medications and treatments to help my quality of life. I hope that helps make sense of how language can be challenging when talking about illness and cancer.

My latest news is mixed. I have hands that are like sandpaper both sides. They are also hot which is highly unusual for me as I'm normally so cold that I joke with people that I'm part lizard. On top of that my fingers are stiff and I'm not as dexterous. I struggle to pick up individual tablets. My nails keep peeling and splitting too. Strange that so many things are going on in this one small area.

The rest of my skin feels irritated and slightly burning like I've been in the sun. It's also very dry and sadly still itchy. It doesn't feel like sandpaper, as my hands do, but it feels quite taut. I've always had very soft feet. I never go barefoot and I never wear uncomfortable shoes. I'm mainly in trainers or ankle boots with a good sole. If I was to wear high heels, I would walk like a parody of a man in drag. I just can't do it. I also don't wear sandals or flip flops. Despite all of this I now have hard dry skin, the kind that people who are on their feet for ages or don't wear shoes get. It's weird.

My mouth is also really giving me grief. I keep getting blood blisters and ulcers and a sore tongue. I've also lost two teeth in the last year, both of which have snapped. On doing some research it seems this may be linked to the high dose steroids that I've taken on and off since 2014. 

my lovely consultant, Victoria, has managed to secure permission for me to have a new medication that may help with some of these challenging symptoms. I've been taking them for two weeks and not had any side effects. They should start reaching the efficacy point after 4 weeks. I'm keeping everything crossed that this is the case.

My legs are challenging me these days too. It seems if I'm standing, like doing the ironing or washing up, my muscles go tense and feel really tired. I was cleaning a chair the other day in the garden and it made my stomach muscles feel like I'd completed 50 sit ups. How can that be? It's all perplexing. My muscles have been really weak for ages and I tremble when I hold the kettle or try to pour a jug of water into the steam iron but this seems to have gone up a level.

One good thing to report is that since taking the new medication for the gastroparesis, I've had less reflux. It still happens but it's not several times every night like it was. It's a horrible feeling waking up choking with a burning throat. Let's hope it stays calmer.

Regular readers will remember that I had this weird episode where I partially lost vision in my one good eye for a very short time. This has happened twice since with both occasions happening in the last few weeks. This has baffled the team. At the time I was checked by Moorefields Eye Hospital, who confirmed cataracts but no major problems. My team at Kings decided to check the blood vessels in my neck with a special scan. I also had a 24hr ECG and this week will have an echo cardiogram. I'm glad I can't travel on public transport. With all the pipes and wires hanging out of me for the duration of that test I would have generated fear and panic in London!

Alongside dealing with all of this, the next biggest issue on my horizon is when can I come out of lockdown? When will immunocompromised patients have hope because vaccines can be ineffective in many of us? I'm doing ok so far but the thought of indefinitely being stuck in my house is almost too much to contemplate. I try not to let my thoughts run away with me on this subject. I know the medics and the scientists are working really hard on making it safe for people to rejoin society. I just hope those that will be left behind don't have to wait too long to catch up.

It can be tough keeping your inner resilience. Sometimes it can feel like you're going to buckle under the weight of it all. When the young students used to talk to me about their problems when I was their school chaplain, I would tell them they had the power to change the world. They would look at me as if to say "Yeah right" but I would follow it up by saying "to the world you are just one person, but to one person you might be the world. All you need to do to change the world is start with the person next to you. If you notice they haven't got a smile, give them one of yours, you have an unending supply" They always left me walking slightly taller and with a smile on their face. Well I need to practice what I preach and make sure I keep giving the love away. As long as I've continued to give and receive love then I've done alright. One of my favourite funeral poems ends "love doesn't die, people do. When all that's left of me is love, give me away"

World Cancer Day 2021

 Today is World Cancer Day. It seems appropriate to do a blog today and give voice to the impact that cancer can have not only on the sufferer but also on all those that love and care for that person. 

My rare blood cancer, Myelo Dysplastic Syndrome was officially diagnosed and confirmed in 2000. That’s not where the story started though. I had my first strange blood tests in the 80’s when I was in my early 20s. My GP discovered I had very large red cells and was a bit anaemic. She asked me if I was drinking too much and gave me some iron and left it at that. This continued to come up as an issue every now and again for the next few years. One day my GP phoned me at work and asked me if I was lactating. I started to laugh and said had she rung the right patient? She said that my prolactin was sky high. She repeated the test a couple of times and got the same high reading. Also my cortisol was high too. On top of this some of my thyroid tests were a bit off as was my calcium but not all of them were off. She eventually referred me to a specialist. I was given a brain scan as they thought I might have a pituitary tumour. This was negative. I was prodded and poked many times and by a variety of specialists. Lots of things were just slightly off but nothing conclusive was discovered. After a couple of years I was sent to a local haematologist as the one consistent was the large red cells and the low grade anaemia. They did some tests and said that it could possibly be MDS. So my diagnosis became ?MDS. Eventually in 2000 I was sent to Kings who did a bone marrow biopsy. This was when I got to meet the amazing Professor Mufti. He told me it was definitely MDS and put me on what was called “watch and wait” It’s a horrible term because it’s like you are waiting for something to happen. It’s like the sword of Damocles hanging over your head and it can fall at any time. Active monitoring is a much more positive way of putting it. 

As the years rolled by the appointments got closer together as my illness progressed. I’ve had sepsis around 14 times. I’ve had serious line infections from having a hickman line inserted into my chest which would sit just above my heart. Last year I had 2 types of flu and have had flu every year since I had my transplant as well as a few times before. I’ve had NG and NJ tubes and still have a PEG J inserted in my tummy due to long periods of not being able to eat. 

When all this started I was a young fit football player. I never walked any where, instead I used to run. I rode a big motorbike. I worked full time, sang in a choir, volunteered with St John Ambulance, was a Venture Scout Leader as well as playing and training hard with my football. I played for Millwall Lionesses and went on to play for Charlton Ladies. 

After all these years of cancer being my constant companion officially for just over 20 years but as you can tell early signs were there over 30 years ago, I now live a very different life. 

I had a stem cell transplant in Oct 2016. My younger sister was my donor and my life was saved by the amazing team of haematologists and allied health care professionals at Kings many times over. My life has definitely been saved but at what cost. My quality of life is much less than what it was. I now walk very short distances with a stick or use a mobility scooter. I have serious hearing loss and wear bilateral hearing aids. This is due to damage from the high dose chemo and antibiotics. I have myopathy which is muscle weakness from all the steroids I’ve needed. My lung function is not what it used to be and I get breathless very easily on exertion or when singing. My skin feels like it constantly has ants crawling all over it. I have awful reflux and wake up choking in the night. I can’t lie down at night because of this and that has an impact in my neck and shoulders. I get ulcers and lesions in my mouth and several of my teeth have cracked and snapped off. My muscles and joints throb, burn and ache. I haven’t been able to work since my transplant and this has been a big blow. The list feels endless and there’s often a new issue to add. 

Despite all of that, I’m glad I’m still here. I’ve seen my daughter grow up into a wonderful woman and capable mother. I’ve loved and nurtured my 4 beautiful grandchildren and miss hugging them terribly during this awful pandemic. I’m still here and in love with my long suffering partner. When someone in the family has cancer the whole family has cancer. 

Cancer has taught me to live deep within my soul. It’s taught me what’s important in life and not to sweat the small stuff. It’s made me even more resilient than my unusual childhood had already made me. It’s introduced me to some fantastic people among the patients, doctors, scientists, advocates, allied professionals, admin teams, cleaners, patients support groups personnel and charity workers. 

I’ve also met two amazing sisters and a brother and their wonderful families who I had never met. My cancer diagnosis spurred me on to keep looking for them. I’m so glad I did.  

I am also blessed to live in a country with a first rate health service.  I have the most supportive and loving family around me. Not everyone is so fortunate. I also have some very clever and creative consultants and medics who I trust and who always have my best interests at heart even in these strange covid times we are living through. 

My job is to keep on top of it all and not curse the darkness but just keep lighting candles. 

I saw Victoria, my dedicated consultant on Tuesday. I have IVIG once a month. This is a drip and stands for Intra Venus Immuno Globulin. It gives me some antibodies to try and help me to have an immune response. Victoria told me one of the things to be looked at is adding covid antibodies into the mix to see if that helps people like me who probably won’t respond fully to the vaccine. Scientists and doctors ans Pharma companies are so clever. Let’s hope they find a way for it to work. 

Victoria also said that she would look into a new drug that may help me. She was going to discuss it with her colleagues and then see if I would be appropriate for funding. It’s so good that the team behind me are still committed to making me as well as possible. They haven’t given up on me once. I’m am touched by their commitment and faith in me. I’m also willing to give anything a go that may help. Advances are being made all the time. What I have to do is when I get to the end of my rope, I need to tie a knot in it and hang on. 

Tomorrow I have my covid vaccine. As said, no guarantee it will work but even if it gives me a tiny bit of immunity it’s worth it. I’ve been told very clearly I need to continue shielding for the foreseeable future. Regular readers of mine will know this was hard news to swallow and made me feel down for a few days. I’ve bounced back as I usually do because I have to choose to live in hope. 

To all of you at different stages of your own story, I write this to encourage you. Sometimes the only thing you can do is grit your teeth and breathe through it. There will be days though when the smile of a grandchild lifts your day or a cuppa with a friend eases the burdens or a hug from the partner reminds you there’s more to you than cancer. I try not to be defined solely by my illness. There is so much more to me than cancer and hospitals and medication. 

I’m a partner, mother, grandmother, auntie, sister, cousin, niece, priest, friend and neighbour. Cancer can’t rob me of that and I choose to keep moving forward and step out in the hope and light of each new day.