Wednesday, 5 February 2014

My VHL disease

I haven't brought it up before on my blog; it's mostly been a thing I've kept private. The last time it was of major concern was really before the days of full social media (facebook, google+, etc); before I developed my online presence, as much as it is.

I'm just going to say it for those interested: back on 17th of January I checked myself into the emergency room because I've been suffering a variety symptoms since September that were increasing in severity. I have now just been discharged back home the other day after a complicated pair of surgeries a week prior.

It's probably best to start from the beginning:

  • I have Von Hipple-Lindau disease. I was tested and diagnosed with it at fairly early age. Every three of so years since about 1997, I've had four operations, until recently when I broke I seven year stretch that was a combination of blind luck and not caring about my condition as much I should've been.
  • I recently had my third cerebellar tumour removed from the right side of my brain; the day before I had my left adrenal gland removed via laparoscopic (keyhole) surgery.
  • I am now without adrenal glands at all, and have to take steroids daily for the remainder of my life to prevent an adrenal crisis from developing.

What is Von Hippel-Lindau (VHL) disease?

Briefly put VHL is a rare, autosomal dominant (50% it'd be passed to offspring) genetic condition that predisposes individuals to benign and malignant tumours. It results from a mutation in the von Hippel–Lindau tumor suppressor gene that we all carry.

More depth-information on VHL can be found on wikipedia, and the worldwide community VHL Alliance, which has been a terrific resource for me and my family in understanding this condition.


What happened exactly?

Although originally I checked myself into emergency because the symptoms I believed were suffering were very similar to those I had in the past due to a cyst forming around a brain tumour in right side of my cerebellum, turns out I was partially correct; we soon all learned that the situation was much more complex, based on old scan data and recent testing on the 25th of January:

My doctors helped explain the complex nature of my cerebellar hemangioblastoma and the lesion on my adrenal gland: my neurosurgeon, liked have my brain surgery ASAP because I really needed it, but some members of the community question this decision, along with my prodising anaesthesiology. The complication the lesion on my left adrenal may cause on my brain surgery (chiefly high blood pressure concerns) may make such procedure more risky than needed.

The alternative suggested had been to have my adrenal removed first (following day, laparoscopically) and then following up with brain surgery once I have a bit of recovery time, about a week originally. This would a) remove any complications the lesion might cause on the brain surgery, b) along with any symptoms compounding everything, which hopefully in turn may give me additional time to deal with the brain symptoms, as well as make the procedure easier when I have it.

In retrospect I may have been attributing some if not all of my symptoms by the cyst/tumour pushing in the brain, but given time to consider it wasn't not entirely consistent with what I was experiencing: mainly night sweats, short rest periods, having frequently to go to the bathroom; along with the knowledge that despite being given meds to lower my blood pressure prior to surgery, I have seen from vital tests it wasn't really working as well as was hoped.

I gave my decision the following day; essentially nothing had really changed, just the last minute order of operations: the following day I'd had an adrenalectomy, followed by brain surgery soon to remove the hemangioblastoma on the day after that.

A photo of my icky head scar about 5 days post-surgery, complete with oily hair and unkempt beard.
My left side laprascopic scars, about nine days post-op. The only parts of me that feel fat and bloated are my head and belly, the rest is too skinny even more me. I aim to move the weight ASAP.


Needless to say the back to back operations were a success, though my body is dealing with both the double surgeries, hospital stay, and getting used to my medication; all whilst trying to heal is going to take some time.


So wait, you have no adrenal glands at all any more? Isn't that bad?

In response to an insightful inquiry and for those interested, here's the skinny: Normally everyone has two adrenal glands, they sit above your kidneys. Removal of one isn't a major concern mainly because the other can provide the necessary steroids for regular function. Unfortunately certain individuals with VHL like myself can develop tumours in these glands (known as pheocromacytomas) that may eventually become active; and even dormant ones cause complications on any surgery/accident by becoming active and causing a "pheo crisis", ie major blood pressure concerns.

Unfortunately my first surgery back around 1997 for VHL was a full adrenalectomy of the right side. So the options were pretty limited: I needed both surgeries ASAP, and the way the lesion was placed ruled out a partial removal/resection. We decided that a laparoscopic removal of my left gland was to be performed before my brain surgery to lessen complications. It was super convenient they we able to pair each operation back to back on Monday and Tuesday.

Now, to the crux of the question: what I have now, because I no longer have adrenals, is similar to Addison's Disease, if not fundamentally that (I presumed incorrectly. it's know as Adrenal Insufficiency). I have accepted that I'll have to take steroid replacements daily for the rest of my life. But also knew when I was younger I'd deal with VHL for the rest of my life too, so I figure I'm up to the challenge. :)


What does this have to do with gaming?

Everything and nothing. My time in the hospital made me realize more how much games, predominately RPGs and the social aspects of them do I rely on as a hobby, stress management, escapism, etc. I couldn't leave hospital, connect with fellow gamers socially via face-to-face or electronically; I had no way of dealing with my 'fix', thus I was forced to confront it head on.

Having spent some time attempting to rectify this problem I was reminded of good work the Child's Play Charity has done since 2003 by helping get games to kids in the hospital. Although I consider myself a child no longer physically, I'm still one at heart. The network and means are in-place for the younger generation, but what of adults?

I am still unable to provide a clear answer to this question at this time, but it has given me something to think about. :) Perhaps I'll add my two cents again in the future.

I would love to hear your experiences with gaming with a disease and/or being in the hospital. :D


Thank you again for all your support! ;{١