I had my post op appointment today.

I travelled to London for a check-up after my operation and to find out what it was that they were removing. Turns out, it was actually cancer, the same type I had in my arm, MPNST, but it is all gone. I’m clear. They have discharged me for my lung now, they aren’t worried at all but I will still be going to check-ups for the next 9ish years. 

Seeing the x-ray of what my lung now looks like is so weird. You can actually see how a section of it is missing and that the diaphragm has moved to accommodate it. It’s crazy.

But I’m happy that’s all over now. Finally. 

U of M researchers develop model for better testing, targeting of MPNST

Researchers from the Masonic Cancer Center, University of Minnesota, and the University’s Brain Tumor Program, have developed a new mouse model of malignant peripheral nerve sheath tumors (MPNST) that allow them to discover new genes and gene pathways driving this type of cancer.

The research was published this week in the journal Nature Genetics.

Utilizing the Sleeping Beauty transposon method, researchers in the lab of David Largaespada, Ph.D., professor in the Medical School and College of Biological Sciences, were able to use an unbiased approach to generate mouse models of MPNST development that lead to the identification of genes related to this tumor’s development.

Funding:  The National Institutes of Health (NIH) National Institute of Neurological Disorders and Stroke (NINDS) grant P50 N5057531, the Margaret Harvey Schering Trust and the Zachary Neurofibromatosis Research Fund, and the Jacqueline Dunlap Neurofibromatosis Research Fund. Additional resources for contributing authors includes the Children’s Tumor Foundation Young Investigator’s Award 2011-01-018.

Read more >

I don’t think I ever mentioned the kind of cancer John had, so I thought I’d share that and a picture or two of of it today.

John developed a tumor called a “Malignant Peripheral Nerve Sheath Tumor”.

Here is just a bit of info on this type of tumor.

>>Malignant peripheral nerve sheath tumor (MPNST) is a rare variety of soft tissue sarcoma of ectomesenchymal origin [1,2]. World Health organization (WHO) coined the term MPNST replacing previous heterogeneous and often confusing terminology, such as malignant schwannoma, malignant neurilemmoma, and neurofibrosarcoma, for tumors of neurogenic origin and similar biological behavior [3,4]. These tumors often create diagnostic problems because of their cellular origin and histopathological similarities with other spindle cell sarcomas like monophasic synovial sarcoma, leiomyosarcoma and fibrosarcoma [5]. They arise from a major or minor peripheral nerve branches [6] or sheath of peripheral nerve fibers [7,8]. These tumors may arise spontaneously in adult patients, although 5% to 42% of MPNST have an association with multiple neurofibromatosis Type-I [9-12]. Surgery is the main stay of treatment of this tumor though they are biologically aggressive in nature [5,12,13].

John did not have the genetic disorder associated with the majority of cases of this cancer. He may have gotten it spontaneously, but more than likely it was a result of his being irradiated during treatment for Hodgkins Lymphoma 12 years prior (at age 18). The location of his tumor was very rare and also pointed to it likely being a result of radiation he received. The fact that he developed this type of tumor at all is very rare. It would not have been the more likely secondary cancers you see after radiation treatment.

We probably will never know how much more time John might’ve gotten had this been caught by one of the 2 Dr’s he was seeing for treatment of what we were TOLD was a pinched ulnar nerve but we do know he would have been eligible for quite a few different types of treatment that couldve extended his life for some time. I cant speak much more on that for the time being.

Anyway, by the time he became paralyzed, the larger part of the tumor was about 2x3" in size as it had been growing for at least a 6 month period (more likely for about a 9 month period, but again, we will probably never either way for sure), on his spine and into his brachial plexus bundle, and a portion of it (not a metastasis, but rather just a growth of the original tumor through the foramen of the spine and into the spinal canal- it used the nerve sheath, as its a nerve sheath tumor) was at that point in his spinal canal and had severely compressed it.

The pictures above are from different scans. The top one shows the tumor on the outside of the spine- its outlined in red and as you can see, quite large in size. The second picture is of the portion of the tumor INSIDE the spinal canal. The whole of the canal (running up and down in this picture) should show up black in this scan. I have circled the quite large" lima bean looking" white tumor that was heavily compressing his spinal cord- hence the paralysis.

Sometimes I look at these pictures and it amazes me that he was essentially so seemingly healthy before the surgery. It was a rapid downhill progression after the surgery on his arm. But considering what he had growing in there…how he managed to work, and parent our kids, and help out with stuff around the house is beyond me. Towards the end (and by that I mean prior to the surgery on his arm for the misdiagnosed “pinched ulnar nerve”, not “the end” as in when he passed) he was in SO much pain even those things were very difficult for him to do and sometimes he couldnt. I know he hated that.

But we had no idea that we were about to be hit by a freight train that would change the lives of many people, forever.

This just happened.

[3:57:36 PM] Freya James: No, I’ve got better things to do.

[3:58:37 PM] Mark Traveltv: really, hope they pay well

[3:59:26 PM] Freya James: Well, not particularly, but personally, I think recovering from cancer is a little more important than working

[3:59:58 PM] Mark Traveltv: you dont have cancer

[4:00:03 PM] Freya James: Yes, I do.

[4:00:08 PM] Mark Traveltv: since when

[4:00:28 PM] Freya James: Technically for three years but was diagnosed in January

[4:00:33 PM] Freya James: the 17th to be exact

[4:00:54 PM] Mark Traveltv: really and what was the treatment

[4:03:26 PM] Freya James: Radiotherapy

[4:03:34 PM] Freya James: And an operation

[4:03:34 PM] Mark Traveltv: full of shit

[4:03:59 PM] Freya James: And possibly having chemo later if the radiotherapy doesn’t get rid of it all

[4:05:29 PM] Mark Traveltv: if you think joking about stuff like that is funny

[4:05:39 PM] Mark Traveltv: there something wrong

[4:05:44 PM] Freya James: I am not joking.

[4:05:45 PM] Mark Traveltv: #with your head

This twat started talking to me on Skype and kept going on and on about me not having a job. After he said “Have you been looking? Do you have better things to do?” this happened. 

He has really pissed me off. So much.

Anyway, he has been deleted and all that and I don’t plan on talking to him ever again.

So yeah, I should probably update you guys.

I wasn’t called in on Monday after my scan. So that means that it is very unlikely that there will be anything wrong with my chest. Obviously, the possibility is still in the back of my head but I am not going to dwell on it now. If anything was serious then they would have called me in on Monday, I would imagine. 

But they have also decided that all my follow up appointments will be at just one hospital now instead of them being in two different ones like they have been since the start of all this, so that’s a good thing. 

Cancer really isn't fun.

I know a lot of people feel a ‘connection’ with people who have cancer if they do too but I don’t really have that. I mean, I quite like talking to survivors and all that and I like to talk to people going through the same thing as me, I just don’t really feel a connection, maybe I would if I had ever met someone who had/has the same type as me but I haven’t. 

I also don’t like it when people tell you that you’re going to be okay and don’t give you room to voice any worries you might have.

Yesterday was the six month mark since the start of this all.

On the 4th of January this year, I was sent to see a new consultant and it was he that set up me getting a biopsy and a bunch of different scans. It was because I saw him, I was diagnosed with cancer. Yesterday, I told Luxford, my best friend, that it was exactly six months since the start of this all. She replied “It seems so much longer.” And she’s right, it does.

It has been an absolutely CRAZY six months. I have been under general anaesthetic twice. I have been to the hospital more times than I can count. I have sat two exams and got the results to those exams, I turned 18, I have been diagnosed with cancer, had a confirmation diagnosis of neurofibromatosis (nf), learnt more about cancer and nf. I have also realised how amazing people can be and how much the step up and I have also started radiotherapy, which I am now close to finishing. There is so much more that has happened to me in these six months but just looking back on it all, it’s been a crazy half a year.

I never thought something like this would ever happen to me. But it has. I remember saying to Luxford once that I am happy it is me that is going through this all and not someone I care for. I wouldn’t cope having to watch one of my friends or family go through all this. I would hate having to get the text or the phone call or the message online. As much as I hate all this, as much as I hate having to go through all this, I would rather it’s me than anyone else. 

It feels like this has been going on for so long but it has just been six months and hopefully, when I am declared ‘cancer-free’ it will only be six months plus a week and a half. Although, I say it feels like this has been going on a lot longer than six months, it also feels as though there has been one hell of a lot crammed into this amount of time.

But yeah, here’s to the six month mark. 

Okay, so I know I said, ages ago, that I had news I would share the next day.

I never got round to it, sorry. 

Anyway, I’m getting to it now. 

So a couple of weeks back, I went to the hospital to get the results of a scan which was to, effectively, tell me whether or not my treatment was successful which, thankfully, it was for the cancer I had in my arm. Which is amazing news, there’s no doubt about that. 

However, during the scan they found something in my lung. It’s only 6mm which means it’s too small for them to know what it is. It could be anything from scar tissue, to another tumor or just a benign fibroma lump. The only thing is, due to where my tumour was before, I am at an increased risk of lung cancer, despite the fact I don’t even smoke. 

I have to go back to the hospital in September, the 16th to be exact, for another scan which should tell us what it is, if the mass they have spotted grows in that time, then it is likely it is cancer again. However, my doctor has said she isn’t worried about it and for now, I’m going to try and not worry. But as much as I say I’m not worried, there is always going to be something nagging me in the back of my head; I’ve already spent the first half of this year dealing with my health, being in and out of hospital and now I just want to get on with it. I want to go to university in September, I want to start that new chapter in my life, I don’t want everything to be put on hold again.

Anyway, I’m going to keep as positive as I can. 

The diagnosis

So, about 3 years ago, this lump started growing in my arm, it grew pretty quickly and was really painful. I was worried about what it was so I went to the doctors and they told me to go to A&E, so I did and was told that it was only a bite. I was pretty sure it wasn’t so went back to the doctors, they said it was a lump and got referred. The man I got referred to, I later found out, was a plastic surgeon and he was going to operate to remove it. Then, he told me about this PDT treatment (Photodynamic therapy) which is a sort of laser treatment and her referred me to the man who does it, in London. Anyway, eventually I got this PDT done. It involved an injection that causes every single cell in your body to become light sensitive to the extent that you can’t go outside and have to stay in a room with only one 40 Watt bulb. Yeah, and it lasts 4 weeks, but the procedure it alright. They put me to sleep then put these needles in my arm and shoot the lasers through the needles. It was supposed to burn away the cells of the lumps and make it go. After the treatment, the lump started to shrink and I was really happy but after the initial shrinking, it grew again so we went back to the doctor who did the PDT and told him and he scheduled me in to have PDT again. So I did, again is started to shrink and then started to grow again. Each time the lump grew back bigger than it was before and more painful. Anyway, I went back to the doctor and had to have an MRI scan and supposedly the scan showed that the reason the lump was growing again is due to water in the tissue. Basically, it was just swelling like your ankle swells if you sprain it.

I was absolutely convinced that it wasn’t that so told the doctor and he referred me to see someone else. I went and saw him in London and he didn’t really talk all that much, he kind of just looked at me, spoke to a collegue, went outside, looked at me again, spoke to me a bit, went out again and all that. Anyway, he sorted out for me to go and see Professor Ferner and neurofibromatosis sepcialist, which is a condition I have and the condition that caused the lump the initially grow. And I had a PET scan, a couple of blood tests, a CT scan, an MRI scan and a biopsy on the lump in my arm. I had all that done in three days and had to go to three different hospitals all in London. We were told that we would get all the in two weeks and ended up being asked to come down the next Monday (the biopsy was on Wednesday, that was the result we were waiting for) and I was a bit anxious because it happened so quickly. Anyway, I went to go and see a doctor called Mr Pollock, he told me that the lump that has been growing in my arm for three years was cancerous.

I cried. A lot. My mum cried, my dad cried but the doctor was so reassuring. He was the surgeon that removed the lump in my arm and when he was talking to me about it, he made sure that I knew everything he was planning on doing. He really made me feel a lot more comfortable and got me some better painkillers. He told me more about the type of cancer I have and all that.

I genuinely could not and still can not believe that the lump that was growing in my arm for THREE FUCKING YEARS was cancerous.

My Dream.

Right at the beginning of all this, I was introduced to Suzie Holmes, my social worker. She helped me to get grants, get a place to stay during treatment and she has done so many other things, I really couldn’t thank her enough. However, one of the things that she told me about was that I could get ‘a dream’ from this company called 'Dreams Come True’. I didn’t really know what I wanted to do and since they mainly work with children 17 and under, I had to get a 'dream’ in pretty quickly. 

Basically, I had absolutely NO idea what I wanted to do, I thought about a holiday and then realised I wouldn’t be able to pick just one place that I wouldn’t really have the time for the next 3 years so I chose something a little more simple. I decided on seeing 'Wicked’ (which I had seen before but we were sitting in the worst seats ever and could hardly see a thing) and I also, just because I could, asked to meet the cast. Now, I have been told that I might not be able to meet the cast but what they are doing for me is: paying for travel, getting me a free meal, getting me tickets to see the show and getting me a hotel for the night. All that and there is STILL the possibility of meeting the cast. 

That is going to be happening in August some time and I am so freaking excited!!! 

I am fucking terrified.

So, a week ago I was called into hospital to have a scan on my chest, they said that the scan was requested as ‘urgent’ but wouldn’t tell me anything else, so I went into London, last minute, to have the CT scan, which wasn’t too bad; out of all the scans I have ever had, they are definitely one of the easiest. I wasn’t expecting to hear anything for a week or so because I know that the team I have working on my case have their meetings every Friday. That means they had their meeting yesterday. That also means I will hear from them either over the weekend or on Monday if there is something wrong. I haven’t heard from them yet and I am hoping I won’t hear from them until my next appointment. 

I remember, back in January, after my scan, which was on the Wednesday, I heard from them the Friday after and they asked me to go in on the Monday after. 

So yeah, I am really fucking scared that I am going to get a call asking me to go in on Monday because if they do, there is something wrong. But that would also mean there is something wrong with my chest, not my arm any more. If I get told that it’s spread to my chest, I don’t know what I am going to do.


True love is (or alternatively you know you are a pathologist when..) when you can get into a lengthy discussion with your husband about whether or not the clinical presentation of the tumor in 50/50 is accurate and agreeing that using an antiquated term for a MPNST (malignant peripheral nerve sheath tumor) is annoying. <3