Part II: The saga continues...

How would you like your disease? Rare? Would you like some embarrassing symptoms on the side with that?

So here we go, Part II: how I finally got from being diagnosed with Cushing's Syndrome to Cushing's Disease. If you missed out on Part I, you can find it here. But let me start by reminding you that I was the one to bring up the suggestion of Cushing's to the head psychiatrist conducting my assessment at the Eating Disorders clinic. When  I had mentioned my concerns to my GP, she had simply told me that I needed to diet or asked if I wanted to go on anti-depressants!

So the way it works in England is that when you go for any type of test, they only get back to you if the results are abnormal and this is usually done by post. But the letter you receive does not contain any more information apart from: We have received the results of your [...] test and they are abnormal. You do not need to worry however please book an appointment to see your doctor as soon as possible. Or something along those lines.

Now at this point I'm sort of panicking. I mean, I asked for the test and I read up all about Cushing's but I guess part of me still thought that because it was a rare disease, it was probably just me wanting to be able to blame something else but that I didn't have it. In fact I was so convinced it was probably a false alarm that I hadn't really even told my family about the test. Then I remembered that abnormal didn't necessarily mean anything. Depending on the tests sometimes they use that term when the results are inconclusive so you have to test again. Anyway all I could do now was book an appointment and wait to see what my doctor was going to tell me. As usual, I couldn't get an appointment until the following Friday which meant over a week of waiting! Oh the waiting..... It is horrible. All I could think about was: What does this mean? Do I have it? What's going to happen to me? And I was just frantically searching all over the Internet about anything and everything I could find out about it. Let me just say, there's some very scary things out there on the WWW. You have to be very careful about what information you read and how reliable the sources you use are...

So I found out that Cushing's syndrome, which is the diagnosis for elevated levels of cortisol can be due to several things though the most common is a benign pituitary tumour also referred to as an adenoma. When this is the case, you are diagnosed with Cushing's Disease. However, there is a small percentage of cases where the Cushing's is due to a tumour located somewhere else in the body like the adrenal glands or even the lungs and other places. These cases are rarer and also more dangerous because these tumours can sometimes be cancerous. (Though they've located my pituitary adenoma, there is still a small chance that I have a tumour somewhere else but I'll get to that later)

So it's Friday and it's finally time for my appointment. I come in and the doctor says to me:

"OK so I have your results and it appears that you have extremely high levels of cortisol in your body which suggests you have Cushing's syndrome. Now I don't normally deliver a diagnosis on a Friday because it's the weekend afterwards and if patients want to speak to me they have to wait till Monday but I think you'll be fine! Here's a leaflet with more information about the condition. I have to say, I don't know why I hadn't thought of it..."

OH MY F****** GOD!!!! Is this really happening? I could not believe my ears! Aside from the actual shock of the diagnosis itself, I was just left speechless by her nonchalance. When I think back on it now, it still makes me angry. "I don't know why I hadn't thought of it" EXACTLY!!!! WHY HADN'T YOU THOUGHT OF IT? How could all these doctors and people I'd spoken to over so many years have ignored all the signs that were there? Even my supervisor at university told me that when she first met me, she wondered if I was suffering from something because she recognised the "moon face". But I try to remind myself that it is a rare disease, only about 5 new cases a year in the whole of the UK, and that the symptoms really are very vague. But I have to be honest, I still get annoyed sometimes, especially when I'm waiting for a test or results because I think to myself, haven't I waited long enough already? Don't I deserve a bit of a break? I've been suffering for years from all these inexplicable symptoms and more importantly, I've spent years blaming myself, thinking I was a worthless person. There were times when I was so depressed that I had contemplated suicide. What was strange about it however, is that in those moments when I was thinking about taking my own life, I realised that it wasn't because I wanted to die to end my suffering, it was because in a way I already felt as if I was dead! Now this probably sounds completely morbid and absurd to many of you but it makes complete sense to me. I would be lying there in bed thinking: Why aren't you living your life to the full? Why can't you just stick to your diet, exercise and be happy with yourself. If this is what it's going to be like for the rest of your life you may as well be dead.
Very harsh and unforgiving words I think you'll agree!

But back to that fateful Friday. So we finish our appointment with her telling me that I will be referred to the endocrinology department at University College Hospital where they will take over my case and probably want to conduct more tests so all that's left for me to do is wait for the appointment letter from UCH to come through the post. So more waiting.... In the meantime, I'm starting my final year of university. It's getting tough, the lectures, readings and dissertation are demanding and with my symptoms worsening I'm really starting to question whether I'll be able to make it through the year.
I get the letter about a week later and it says that my appointment isn't till December! 3 Months? I couldn't handle it. The thought of having to wait 3 months just for a 1st consultation pushed me to get pro-active. I started emailing anyone I thought might be able to help and even considered going private however I was told that this was a bad idea because Cushing's required long-term care and going private was really expensive and probably not worth it. Turns out that when it comes to rare diseases you're better off being treated by NHS doctors because teaching hospitals receive funding and government support therefore they have the centers of expertise. Luckily I got in touch with a lady from the London branch of the Pituitary Foundation, told her my story and she very kindly emailed the consultant at UCH who is also a medical advisor for the Foundation asking if there was anyway she could see me sooner. Turns out there was an earlier appointment available!

16th of November, I arrive for my appointment. I'm nervous but excited at the prospect of finding out where I go from here. You can imagine my disappointment when I am seen not by my consultant but by a registrar (something which I will come to realise will be a frequent occurrence) and the appointment is spent taking my medical history. He explains to me that Cushing's in complicated and long to diagnose, blah blah blah... Not very encouraging! But the first step is to confirm that the high cortisol levels of my 1st urine test weren't a one off so I now have to do 3 more 24hr urine tests and some blood tests. I of course rush to sort everything out as soon as possible but don't hear anything for a whole month. And even then, all I receive is a letter which summarises our appointment and states that they will see me in a few months time to review everything. By this point it was Christmas break and I was going to France, so I didn't push anything because I had enough on my plate already. But as soon as I returned in January I phoned UCH to say that I wanted to do all the other tests now and didn't want to wait months to review the results when I knew they had them already. I really don't want to go into all the details because they are long and infuriating but basically it was as if luck was not on my side!! They would take weeks to get my results, then they would get sent to the wrong person, then the blood tests would be done wrong or the MRI machine was down, and so on and so forth... So over 6 months I had a total of 6 blood tests, 2 CT scan, 3 MRIs and only 2 appointments at the endocrinology clinic where I was seen by a different person each time. When I look at these numbers, they don't look too bad and I think to myself, why do I get so worked up about it? Well it's because that's 6 separate occasions when I had to fast for 24hrs and get my bloods drawn at 8am, each time more painful than the next because my veins are notoriously hard to find and Cushing's makes that worse. Cushing's also makes you bruise easily so for weeks after these blood drawing sessions I would have bruises on my arms and hands (because they'd usually attempt several areas)  which made me look like a battered woman!

My latest hand bruise though this one is actually not so bad compared to some I've had before! 

The MRIs weren't painful but the experience is very unsettling. The machine is noisy and makes you feel like you're being buried alive. You have to lie there for around 20 minutes and you cannot move. Oh and did I mention that because of the incompetence of someone somewhere, when I went for one of the MRIs they hadn't received my blood results yet so couldn't do the full scan. Which is why I ended up having another one!

So I think that by now you're probably starting to get a sense of why I was getting so frustrated! Well not to sound like I'm trying to lay it on thick, but what was really tough about all this was that I was really struggling coping with this and university. How could I be expected to write a 12 000 word dissertation, revise for 3 exams, chase up after incompetent and completely unsympathetic NHS staff, and take care of myself and my flat? All this with only a handful of friends whom I could share my problems with and the closest family member over 200 miles away in Paris.
I would come home after a day full of lectures and would have to muster up enough energy to cook dinner, do household chores and then if at all possible, revise for the exams. I had no life whatsoever! The only time I went out was for uni or to do the shopping because I couldn't and didn't want to live off take aways! But again, even the few activities I did have (working and cleaning) were becoming more and more difficult. My concentration and memory were deteriorating, I suffered terrible headaches and backaches and even though I was exhausted all the time, I was suffering from insomnia. I think the only thing that kept me going was the encouragement and support of a few key people and me reminding myself that if I had managed to come this far, I could push a little more. To be fair, I also have myself to blame because no one forced me to do anything. My parents never put any pressure on me and it's not as if the world would have changed if I didn't graduate this year or graduated with a lower class degree than I had anticipated. But I think for me this wasn't just about that! This was about proving to myself that I wasn't a useless/worthless person....

So I finished my last exam on the 11th of May, convinced I had failed but deciding that there was nothing more I could do now. I then had just a few days to finish off my dissertation for which I had been given an extension due to my mitigating circumstances. At first, I was even reluctant to ask but I realised that I wasn't superwoman and sometimes being strong is about asking for help!
I handed in my dissertation on the 21st of May and by the 24th I started my summer job. Yes folks, I'm a bit of a sucker for punishment it seems as I accepted a job over the summer. But the reason I did that instead of going to France and spending some quality time with family and friends is because I was still waiting for the results of my last MRI and therefore had to be in England!! And actually I just couldn't turn down this job offer. I was contacted by my old boss at the charity for genetic diseases where I had done a work placement the year before. It was 8 weeks of paid work, doing research into undiagnosed families. I would be employed freelance so could work from home if need be and because I was starting end of May and the report didn't need to be submitted until end of August, I was free to work whenever I wanted and felt up to it. How could I say no? Working with all my old colleagues whom I really liked, doing an interesting piece of research which could contribute to improving the lives of families who are in dire need of help and support, being able to work around my own schedule AND get paid for it all. This was perfect! Not to mention a huge ego boost because she had contacted me.

June went by relatively smoothly but July was rough. On the 8th of July, my grandfather passed away. I was close to my grandfather because he loved singing too. In fact, many of my relatives think that he's who I inherited my voice from because neither my mum or dad are very good singers. But I'm not the only one who took it hard. My grandfather was a real patriarch, the true definition of a strong old fashioned father figure and family man. He always made sure that every summer we would all get together as a family to celebrate something, either a birthday or a wedding anniversary or anything really. And we all loved him for it! The proof? He was able to unite a family of over 60 every single year. That's right, over 60 people. My mum is one of 8, and of her 7 brothers and sisters only 1 never had children. Most of them have 3. And of course those children (my cousins) also now have children of their own. So we're talking 4 generations here!

So I rush over to France for the funeral, knowing that at my return, I have an appointment scheduled at UCH and that they should have the results of my MRI by now...

15th of July 2010, the day I am told that they have located the tumour on my pituitary gland and I am being referred to the National Hospital for Neurology and Neurosurgery. Nothing more, nothing less! I guess it's slightly my fault, maybe I should have asked more questions I don't know, but I left feeling like... well I don't know really, feeling numb I guess! But then I remember that of all the possible sources of Cushing's, this one is the least worse (pardon the terrible grammar but there's no other way to put it!).

I should probably mention at this point that I had applied and been accepted onto a Masters course at the University of Hong Kong which I assumed would start end of September, as they do here in London. I was wrong, my course started end of August, and without an operation date fixed yet I was starting to panic wondering how I was suppose to pack all my stuff up for the year and be ready to go. So when my parents came over for my graduation ceremony, we had a long discussion and decided it would be wiser for me to defer. Initially I wanted to defer only until January 2011 but it turns out that if I did that I would miss out on certain optional modules which I really wanted to take and were the main reason for me applying in HK as opposed to London. So I had to defer until September 2011. But it's a good thing that I did...

I had my graduation ceremony on the 23rd of July where I received my BSc (Hons) in Psychology and Social Anthropology with the degree class of 2:1! And the following day, mum, dad and I went to France for 3 weeks of holidays. It was a well deserved break for me though not quite as relaxing as I would have liked because there were 3 big family functions (birthday, baptism and wedding) and in the back of my mind was the nagging thought of When am I having my operation?

On my return I receive a call from the National Hospital (NHNN) and they ask me if I still want my appointment.
What do you mean do I still want my appointment? Of course I want an appointment, when was I not clear about that?
Apparently they had misunderstood and thought that I had decided not to go through with the surgery. To this day I still have NO IDEA where they got that from because if anything I couldn't get the surgery soon enough! I was pushing and pushing... Like I said before, I have just had the worse luck so far. Either that or the NHS REALLY sucks!!!

So I go to my appointment and to my surprise EVERYONE is there! My consultant whom I had not met till now, the neurosurgeon Mr.P (whose name had been mentioned before but remained a mystery to me) and even 3 consulting endocrinologists and specialist doctors. Basically, I was at the big multidisciplinary meeting! Now this is a BIG deal folks!!! I even got to see my MRI and they actually showed me where the tumour was. FINALLY they were acknowledging me! I even mentioned that when I was younger I was told that I had only one sinus and wasn't sure if that would be relevant with regards to the operation, and they promptly wrote a referral for a CT scan.
As the meeting draws to a close, all the doctors are in agreement that the transsphenoidal surgery is the best option though they remind me that there is still a very small possibility that I may also have a tumour somewhere else which is causing my Cushing's. Nevertheless, the first step is to operate on the pituitary and take it from there. At this point, Mr P. picks up his diary and says: I can do the 13th or 20th of September though I would prefer the 20th, which is good for you? I was slightly in shock, never had anything been so quick or easy to organise! I guess neurosurgeons like to get straight down to business. So I shyly utter: The 20th is fine for me.
Great! Here's all the forms you need, go down to admissions and pre-admission and I'll see you on the 20th.
I go to all the offices, sort all the paperwork out and head home with an appointment for Friday 3rd of September for a pre-admission check. I was feeling so good after that that I immediately emailed everyone who knew about my disease that I'd finally gotten the op. date. That day I also decided to register for the Adidas Women's Challenge 5K run in Hyde Park. I wanted to raise money for the Pituitary Foundation who'd provided me with a lot of information along the way and most of all, I wanted to use the opportunity to start a web page announcing to everyone that I was suffering from a rare disease and having surgery soon. I also started my blog! Why all this at once? Well because I had only actually told a select number of people and even they weren't fully aware of how severe my disease was/is. Not because I didn't want to but because ever since I found out about it in Sept.2009 because I was still technically testing and the diagnosis wasn't 100% I just never really found the right time to tell everyone. Also it's not really something you just bring up in conversation:
Hi, how've you been? Oh good, what about you? Well actually I have a tumour in my brain which is why I've gotten so fat and depressed but don't worry I'm having it removed soon!
So the Adidas challenge and blog were the perfect opportunity!

As I mentioned in my previous posts, I really hesitated for a long time about how much I actually wanted to share on my blog but I finally decided that it would only be worthwhile if I was completely honest and not afraid to "put myself out there". And boy am I glad I did. My mum forwarded the web addresses to my blog and justgiving charity page to all of her friends and the support (and donations) I have received is incredible! Just look at some of the comments that have been left. I have never felt more loved or supported in my life and it is a great feeling, especially in these tough times.

Friday's appointment went fairly well except my blood pressure is still too high so I've had to up my medication to resolve it before surgery. Oh yeah and my CT which was due to happen on the same day was cancelled and rescheduled to yesterday because the machine broke down. Like I said, I've been having the worse luck....
But you know what? All these little things haven't really bothered me. Sunday's race was great and I've been extremely positive lately.
I've been having concerns about the surgery and how long it will take me to recover after but I use a Cushing's help and support discussion forum and my online Cushie friends have been very encouraging!
Only down point is that my subconscious is obviously not as positive as I am because I haven't been able to sleep properly lately (insomnia has always been a problem) but what's been troublesome is that I've been having terrible nightmares about my health. Dreaming I was misdiagnosed and I actually have something worse than Cushing's or other things like that...
Oh well, I'm staying strong!