Friday 28 February 2014

I'm one in a million!

Today marks Rare Disease Day, a day that didn't really mean anything to me last year, well why would it?, I wasn't aware that I was suffering from a rare disease, and didn't know of anyone who did; however this year it is different.

I wasn't planning to blog about this, however I feel compelled to help raise awareness of a condition that I am suffering with. Regular readers of my blog may recall that last year I underwent an MRI scan and knee operation.

Lottie and I after my knee opertaion

The results of my MRI scan identified that I was suffering from the early onset of something called Pigmented Villonodular Synovitis or PVNS for short. My consultant and I didn't really talk in too much detail about it, as we hoped that the operation he was going to perform would alleviate the severe pain that I was experiencing.

Well fast forward a few months after the operation, the pain has spread and I have undergone numerous blood tests to rule out other things, including my calcium levels in my bone profile; my calcium levels are decreasing month after month, and my Doctor believes that the PVNS is further advanced than we initially thought.

I am very blessed to have an amazing GP, but unfortunately she had never heard of PVNS, and why would she when only 1-2 people in every million have the condition; she has promised to research and become more aware in order to help me, and faxed my consultant last Friday asking for him to see me as a matter of urgency. On Monday to my surprise I received a phone call from my consultants secretary saying he will see me next Wednesday. I cant tell you how relieved I feel to have an appointment so soon, because it has started to grind me down.

I really don't like having to take strong painkillers to get through each day, but I have to if I am to continue with normal everyday life. I have quite a high pain threshold too as I gave birth to Lottie at home with less than 5 minutes of gas and air, but this knee pain is something different. Some days I struggle to get out of bed and some nights I go to bed in so much pain that I cry; I am fortunate that Hubbie is really understanding and helps me when I am struggling.

Inside of my knee

Many people have asked me what is PVNS, well its hard to describe but here is my simple definition. PVNS is where the fluid that surrounds the joints of a limb e.g. a knee or elbow etc, is full of lumps or non cancerous tumours that grow and cause pressure and pain on the joint; sometimes the lumps can grow into the bone. Obviously there's a lot more to it than what I have briefly tried to explain, but it all goes way over my head! I really hope it has not gone into my bones, but I wont know this until I have had another MRI scan.

There are a range of different treatment options, but each come with limited success; surgery seems to be the first option and trying to remove the lumps, however it is very difficult to tell if all of the lumps have been removed, and if a tiny bit is left it will regrow. Another option could be a full knee replacement if the lumps are in the bone, but yet again it requires that all cells are removed to ensure complete success so that they don't regrow. The third option, which might be offered, is radiotherapy, this aims to shrink and kill of the tumour cells; but then the side effects of radiotherapy are not great and there is still a chance at the end that cells still might be left.

I am not sure what the treatment options I will be given and what the plan of care is going to be, but I should know a lot more on Wednesday. I am determined that even though I have a rare condition I am not going to let it get to me too much. I would also like to apologise for the amount of time I sometimes spend moaning about being in pain, but I suppose it could be far worse! 

For more details on PVNS click here.


  1. Hi
    Thanks for helping raise awareness! I am hoping that your surgeon got everything out this time and you will be free of the beast forever. It is hard to feel that one surgery is the cure but it has happened for some.
    #RareDiseaseDay #PVNS
    Blessing to you

  2. That sounds really awful, so sorry to hear you are in so much pain, and that the treatment options are so limited. Great to hear you have a supportive husband.
    Thanks very much for sharing this. I have seen so many tweets from you about your pains that it's good to know a little bit about what is causing it (although it would be much better if you didn't have it, of course).

  3. Why do such things happen to such lovely people. You have no need to apologise at all - what you need to do is show the strength to reach out for all the support you can get. People who know you even a little will be happy to give it. I would be useless facing pain so I just hope that you can find a suitable treatment that eases things for you. Thinking of you

  4. I haven't heard of PVNS either but as a sufferer of Rheumatoid Arthritis for 30 years I understand your frustration and worry, hopefully you will get effective pain treatment. x

  5. Bless you lovely, well doen for being strong and writing this to help with awareness. I'll be praying for you, all the best tomorrow. Mich x

  6. Praying for you - it is horrid to be in pain and the pain you describe sounds horrendous. Really hope that everything falls into place and that there will be a suitable treatment plan for you. x

  7. My 15 year old son Jack one half of twin boys, has been diagnosed with PVNS. His op to remove his diffuse tumour is Monday 17th March. I wish you all the very best with this nasty condition, and hope that you get clear if it x

  8. What a horrible disease to have, it sounds as if there won't be a simple answer but hopefully they can make things easier for you to manage soon. Take care xx