Bad Friend

One of the biggest worries I have had since starting this blog was letting my friends read it, there are things that I have written and will write that they know nothing about and in all honestly I am fairly scared about their reactions if and when they read them.

I have an amazing group of friends and I would jump in front of car for any of them, well most of them, some of them might need some a bit of knocked into them. The way I see it, it is not a reflection of them or our friendship that they don’t know things about me that I have hidden from everybody apart from the other people who live in my head. (Just in case you wondered I only have one voice in my head.)

But, this whole experience has made me very insular, I am an extremely social person and love to “work a room” but beyond being friendly I keep my cards very close to my chest as to what is really happening in my head. I prensent a bravado that really isn’t me, I imagine it is a protection mechanism, for me and for my friends; I don’t want them to associate me with anything to do with my illness. I just want them to be friends with ‘me’, the one that a lot of them knew before my illness, but I guess I am starting to learn that this is a part of me and for them to know who I am it is important that I don’t bricked them out of a part of my life.

I would definitely say I have lived behind a facade for probably the full 10 or so years, which is pretty scary to realise. I have been to and still go to pretty dark places in my head. My dark place and I get on pretty well these days, I try to give it a few hours at a time convenient to me every week, and as a result I rarely get dragged into a slump as frequently as I use to. I have always been able to paint a rose picture for observers but I have never taken time to stop and think if people are recognising my strange depressive behaviour and let me get on with it or just let me get on with it.

My head has been fully loopy ever since my diagnosis but it got worse when I realised that I was going loopy and not talking to anyone. You become more insular as you come to terms with just how cuckoo you have become, well I did anyway.

Oh and yeah and …………… I have heard the one about the dark space in your head being because something is missing.


Operations, more operations

After my second operation I was given time to heal, I had a year away from the table to allow hooks and clips to dissolve and to let my face reattached to my skull, before it got ripped off again.

So …..March 2007 was the date of my 3rd operation and this time it would be both the previous operations in one go, it surprisingly only took 8 hrs and again I was home within 48 hours of having my face sewn, glued and stapled back together, no mean feat. This time I was sent home with drains in again, these are particularly horrible contraptions, a tube comes from the bottom of the incision out to a deflated pump balloon which fills up with blood and fluid until the wound has healed. I was making frequent trips back to the dressings nurse again but this was  due to the number of incisions and not an infection so I wasn’t that bothered. The stitches around my ears were the first to come out and then after another two and a half weeks out came the staples. Now this is when you think I am a real freak. Having staples removed is one of the strangest pleasure/pain sensation I have ever experienced. The area is still reasonably numb, and some of the stables are scabbed over and can be hard to remove but all in all the sensation as the staple is pull from your wound is kinda nice. There I said it, don’t look at me differently now.

After this operation I again put everything on hold to let my face recover and thought I should probably finish my degree without any interruptions, so any more operations could wait.

Time to get in the DeLorean, we are heading to 2009. (if you don’t understand this reference, please stop reading – only joking, but seriously Marty McFly is the bees knees)

I have finished University and decided this would be a good time to have my next operation ahead of joining the voyage on a ship by the name “Career”. I saw my Surgeon in August, I was on the list, but he was busy at the time and would get me in as soon as possible. I was given a date in September for a pre-operative assessment, which went wonderfully. Then the wait, a long wait.

Throughout December and January I was trying to contact the NHS to find out if I had a date for my surgery yet. I didn’t hear back and by the time February had arrived I was more than concerned that I was putting my life on hold for something that was not even scheduled. By March, I resorted to making a private appointment with my surgeon to discuss if that route would speed things up. Low and behold my surgery was actually scheduled with the NHS 6 days after my private appointment, I just wasn’t told. To rub salt in the wound, I was meant to be on holiday.

My surgeon kindly said that I should go on holiday and he would sort it all out for me to have the surgery in April. He was an absolute superstar creating space for me after the administration of Chelsea and Westminster had royally fudged up. My surgeon has always tried to make my surgeries as convenient as possible for me, for my first 3 operations we got our diaries out and scheduled them around my university holiday and his kids’ school holidays.

So April came and it was time for my 4th operation. This time I was having the scars around my ears removed and a bit of a tweak around my eyes. The surgery was a lighteningly quick 4 hours and after another breezy 48 hours on the ward I was being driven across London to nestle on a sofa for a few weeks.

As with previous surgeries I had to sleep upright again with my 3rd and 4th. This is one of the hardest parts of the whole experience, you don’t feel as though you are recovering properly when you can’t have a bit of quality shut eye. My recover took about 4 weeks this time with the stitches coming out after 2. I think my head is getting used to inflating to the size of Hey Arnold’s.

Well that is all of my surgeries covered now, but fear not, I am awaiting a date for my next and we have all the joys of the investigations and more presentations.

The investigation goes…. well nowhere

After the inspiring update from last week, let’s get back to the history and try and get up to date before my next appointment.

After having the first two surgeries, my face was given a year off, to settle. This was when things started to go less than brilliantly service wise with Chelsea and Westminster hospital. I was referred to the dermatology department for them to carry out some studies into the disease. This was excellent news; one of the best things about being a special case is that you normally get the attention of the doctors as they like to play with weird stuff, that’s me. Apparently not so in 2006/2007 at Chelsea and Westminster.

I saw a professor of dermatology who specialised in connective tissue disorders; he was going to conduct research in to everything about me. This sort of happened, but I was never given the interim findings and there were no further studies beyond those findings. I had a medical history done and a skin biopsy, then nothing.  My case and I were shown at the Royal Society of Medicine which was a strange event, I sat topless in a cubical as about two to three hundred doctors walked past me staring, some even did me the courtesy of pointing or poking me, this was a pretty horrible experience, but not the worst of this time.

During my skin biopsy I was asked if the students could look at my case photos. To start with I have students in lots of my appointments, Chelsea and Westminster is Imperial College’s teaching hospital and I’m all for helping the kids learns. I’m also vain so think they were going to see a set of portrait shots I have posed for in the photography studio at the hospital which would give them a good look of my cool hairstyles over past two years. Now if you have a choice between looking at photos of yourself or someone cut into your hand down to the bone, on most occasions you who be watching the slideshow, after about 6 photos I saw a bloody mess. Ahhh they were showing photos of me with face cut off, if you have seen face-off, it is accurate. Now I had never had a problem with gore but my own gore that is disturbing and since that day I have been unbelievably squeamish, I hide my eyes during Holby City. My skin biopsy results were pretty normal to the disappointment of the doctors and this is when the department’s interest in me tailed off, the interim findings suggest I had six more investigation to find out more about the condition, I had none of these, in fact I had no further appointments with the dermatology department until December 2011, when I took the initiative to approach the department again and tell them to sort their act out. Now I can’t fault the new team looking after me, they seem to be good geeky doctors who find me extremely interesting. So project me continues.


I’m a Mutant

This one is coming out of sync, but I’ve had some pretty fandabbydosey news. I had an appointment with my dermatologist on Monday and was told not only has she found me a dermatological disease specialist to take over my case and give me the attention I deserve, but medical research has located the mutant gene that causes my disease. I don’t think I have ever been happier to find out that I’m a mutant; you can officially call me the x-man from now on, or the hero turtle. But to be serious again this is amazing news, I don’t actually know how this news will initially affect me, it will probably mean more tests. The good news is that when I decide to have kids they won’t have it, gene selection already happens, so whoop whoop I am a very happy bunny.


My First Two Surgeries

We now arrive in 2004, time for my A Levels. As I was meant to be concentrating on my education the medical stuff took a back seat, I had a couple of dermatology appointments at the end of 2003 and then in the spring in 2004 I met my FANTASTIC surgeon for the first time. I’m going to throw it out there; the man is pretty big time in the NHS maxillo-facial plastic surgery world. We scheduled my first surgery for the summer of 2005 and then put everything on the back burner, screw the A levels I had a gap year to go on.

So we jump to the summer of 2005, back from 6 months in Mexico, with tragically long hair and lots of bracelets, I had around 6 weeks to prepare to have my face pulled from my skull, yeah I went there. At the time I didn’t really pay attention to the play by play, I have seen face-off and pretty much knew the drill, by complete mishap I have seen the pictures from the surgery and the film is pretty accurate.

The first surgery was a good 7 hours, I slept straight through, so I can’t complain. The incision was from ear to ear across the top of my head, I had over 20 clips drilled into my skull and which would hold the skin in place as it healed and reattached to my skull. I spent about two and a half days in hospital being absolutely off my tits on morphine before I was sent home to sleep sitting up, with drains hanging out the side of my head. Now sleeping sitting up is possibly the hardest thing in the world especially when you have to do it for over 10 weeks. No matter how many pillows you position in this way or that, you will never get a full night sleep; trust me I’ve had to do it four times.

The first operation has been my only experience of an infection, touch wood. It was a pretty horrible infection and meant I was in and out of hospital for appointments with the nurse every 3 days for a fortnight, and lots of puss and oozing. Luckily this wasn’t too bad an experience as my nurse was a very hot Australian. But the downside was the infection caused me to lose some hair, so I had a bald spot to go with a massively swollen head and about 50 metal staples running from one ear to the other. The clips which were now lodged into my skull felt every strange, I still have sensitivity from where a clip was affected a nerve, it used to send an awesome shiver across my skull every time I ran my fingers across it. The clips dissolved after about 8 months and I no longer have that weird sensation, you can miss the strangest things sometimes.

Four weeks after the surgery, I had fought off the infection and had all stitches and staples out, off to university I trotted. Next stop March 2006, Easter holidays from university and operation number two.

This time I had a lateral facial lift, so the incisions were around the ears and my cheeks were pulled back, I’m pretty sure that isn’t the full medical description but you get my point. This operation was just 6 hours and 48 hours later I was at home sitting on the sofa ordering people around. My recovery was much more successful this time, no infections and I didn’t head home with drains hanging out of my head, never a good look. I only spent 6 weeks sleeping sitting up this time around, which helped as I was back at university after just 3 weeks. My head was still swollen, but not the size of a football as it is after surgery, and I had lots of massaging and ointments to help recovery.

I tried to take the surgeries in my stride but in all honesty I was all over the place at the time. I have two major procedures within just over 6 months, and my head and body couldn’t take it. No matter what people say if you have over 12 hours on a surgeon’s table in half a year, your body takes forever to recover. I doubt I will ever get my head around any of this or be able to drill down into details of what happened on the table. (The pun is completely deliberate)

Correct Diagnosis

Well the next couple of years were the real game changer. New symptoms made it clear that it was very different to what I had already been diagnosed with. From the age of 16 to 18 my skin started to change dramatically. It started with a thickening of my facial skin and the skin on my hands. Over about 6 to 9 months the joints in my wrists and hands began to swell, in a similar way to which my knees and ankles had. I was fairly oblivious to this happening; it was mainly my family that noticed. Unlike my knees and ankles, my hands and wrists have never caused me any pain, so I kind of just thought they had grown with the rest of my body and didn’t realise that they weren’t normal. The other changes over this period were much easier to recognise as they weren’t normal. The thickening of skin on my head had developed into folds on my scalp and forehead and sagging of the skin around my eyes and cheeks.

My doctors at the time were baffled, so this resulted in many more blood tests, this time there were some really horrible experiences. I think the worst tests were for a hyperactive thyroid. It started by drinking a sugar solution, YUM. Then over the next 4 hours my blood was taken every 20 minutes, not so fun. A prick every 20 minutes might have made me feel like a pin cushion but would have been preferable. Instead I had an intravenous needle on the inside of my elbow. Not helped by a shockingly bad placement by the nurse, this was an unbelievably painful experience, I can still remember the pain, from all 3 times I have the test. All of the tests were again showing no consistent results for any definite diagnosis, but they did rule a lot out, most importantly acromegaly.

By the summer of 2003 my doctors, my family and I were at our wits’ end, so my very kind rheumatologist recommended I see a former colleague who was a dermatologist he “really rated”. Off I trotted, to yet another new doctor but to my surprised I walked in the room and the doctor pretty much said, this is what you have. Ok, he might have given me the once over first but he knew the moment he saw me. I was about to be anointed UNIQUE. He diagnosed me with Pachydermoperiostosis; he had seen it before but not to the degree that I had it.

Over the 18 months to two years that we hunted down a diagnosis I tried to pretty much ignore my condition. I didn’t talk to anybody about it and tried to continue with my life as though nothing was happening. This was not because I didn’t thinking about it, I just couldn’t deal with it in reality, speaking about it would’ve made it a part of my life which I wasn’t ready for it to be. I guess I did suffer in silence but only because of my own stubbornness. I wasn’t in a happy place. I felt anger towards my parents at the time. They would research all the possibilities and get worried as to what the outcome would be. They didn’t share any of the information with me which definitely made me feel as though they were treating me ‘like a child’ a big no no for any teen. In reality I was acting like a child, ignoring my conditions and refusing to discuss them. They were also protecting me from some of the possible diagnoses which would of just lead to constant worry.


First Diagnosis

Right, where to start.

I guess at the start of my symptoms would be a good idea. So I’ll take you back to 2001.

George W was inaugurated, George Harrison died, Jeffery Archer went to jail, Enron filed chapter 11, Shaggy was topping the chart with “It Wasn’t Me” and Hear’say were formed. Oh and something happened on September 11th.

I was 14, in the 4th form at senior school, which is year 10 in new money. I had played sport throughout my school years but by this time, what was thought to growth pains was heavily impeding me. Having seen physiotherapists regard the pain and swelling in my knees I was recommended to consult a rheumatologist once the pains and swelling had started to affect my ankles.

While all of this was going on with my joints, I was suffering from fatigue, doctors immediately considered ME and glandular fever. So I embarked on possibly the longest series of blood tests in row with completely different results every time. I was anaemic one week, then had hemochromatosis (too much iron) the next and so on. The only thing that was a continuous throughout the tests was that I did not have glandular fever and I wasn’t suffering from ME.

At my first appointment I was diagnosed with chronic juvenile arthritis and the process of managing my pain was started. After about 6 months of playing with prescriptions and dose I was settled into a regime to remove my pain and allow me to continue as normally as possible.

My attitude during this 12 month period was frustration; the initial impact upon my life was hard to take, but when a diagnosis was found and more importantly a method of pain management introduced, my mood lifted, I thought I could move on. I thought that although it would always be there it would only have a minimal ongoing impact. I could get on with being a normal 15 year old.