Addison's crisis - Not dead yet, Jim!

I haven't blogged for a bit as I was on holiday and when I returned, my hard drive mysteriously had crashed. At least we weren't burgled, as that is one of the favourite half-term pastimes in our neighbourhood.

It was quite an eventful holiday as we had occasion to use Kirk's emergency 100ml intramuscular hydrocortisone injection.

Star Trek would have it sorted by now

It's pretty official now... Kirk's developed a condition with a name! Addison's disease.
Besides obviously already having a tumour and having lost all the hormones his pituitary makes, he's also lost cortisol, which is produced by the adrenal gland. Well, at least it has a name, some notoriety, and they make you carry a blue card for emergencies. Kind of cool.

Even cooler would be if the 21st century ever delivered what I've come to expect from the future as presented in the past. Star Trek is just one example. How light they make of those perfectly treatable diseases that were still killing people left and right back in the 20th century. No such luck. No quick diagnosis by tricorder, no immediate treatment with a one-size-fits all hypospray and a short spell in sickbay. Your choice of doctor: grumpy drinker, career mum, genetically engineered genius, or hologram.

Science fiction isn't always far from the truth though. Why recently, for the remake of Tron, they scanned the actors into the computer exactly like they imagined in the original. Back then, they made it up. And within their lifetime, it came true!

Anyway, I've invented the world's first artificial pituitary.

On the one hand, the pituitary gland is a kind of thermostat. It measures the values of certain elements in the blood and reacts accordingly to adjust those levels. It also reacts to certain other bodily states in order to, for example, stimulate more cortisol production in times of stress. As I understand it, fairly simple computers can perform similar tasks, like an electronic blood pressure meter or a fingertip pulse oximeter. Given recent experimental developments like IBM's chip on a molecule (http://www.theregister.co.uk/2006/03/24/ibm_nano_chip/), it should be possible to create a measuring device and processor small enough to be portable or even, ideally, implantable.

Of course, implanting foreign objects in the body can cause rejection, so I would look for a solution by building the processor out of biological materials. There's already been successful experiments of this as well (http://news.bbc.co.uk/1/hi/sci/tech/358822.stm). You could use cells taken from the patient's own body. Perhaps even cells from their own pituitary gland, which, helpfully, actually contains adult stem cells so they would be ideally suited to engineer into a new form.

The other role the pituitary fulfils is that, after having monitored the body's needs, it also produces the necessary hormones or stimulating substances for other glands to produce other hormones. Now, making new cells out of scratch can be a tricky proposition. At the moment, replacement hormones are synthesized in big laboratories and then injected or taken as tablets etc. An intermediate solution could be to do much as the continuous glucose monitoring system for diabetics does. A small device is put just under the skin to measure glucose levels and it then radios to another small device to inject synthetic insulin as required. The artificial insulin comes out of vials and needs to be kept topped up.

However, I'm being ambitious here. I'm designing a completely artificial pituitary, to effectively replace a broken one. It needs to be small, implantable, and virtually maintenance free. I'm willing to put up with it needing replacement every 10 years or so (like an artificial heart valve) and I can allow it to be bigger than the actual pituitary gland, as we'd probably not want to implant it in the brain anyway. I was thinking upper arm, and roughly the size of a contraceptive implant.

The replacement pituitary would, similarly to the original one, have to use building blocks that are present in the body to construct the hormones it needs. As we can synthesize hormones in the lab, I assume we have their 'recipe'. Then, it is merely a question of building a finger-sized lab/hormone factory. This would take some serious nanotechnology, but I'm sure both the molecule microchip and biological processors can provide a solution here.

Come on people! This is the 21st century. Let's stop injecting ourselves with needles and taking tablets. None of those methods even come close to mimicking the intricate machinery of the human body. I want my flying car, my robot housemaid, and some miracle medical breakthroughs.

Cortisol... check! Last one to go

After a recent blood test - by recent I mean a few weeks of course, we are talking NHS here - it turns out that as we suspected, Kirk's cortisol levels are now worryingly low. This was to be expected. For the past year, his pituitary gland has been gradually shutting down all production of adult growth hormone, testosterone, thyroid stimulating hormone, and now cortisol. The good news is: it doesn't do anything else so nothing else can go wrong. Unless the adenoma turns into a prolactinoma, but that's unlikely. If it was going to, it would be one by now.

Cortisol deficiency is a bit scary. It's the hormone that the body uses to cope with stresses. It is given in standard daily doses, but sufferers need to carry an 'emergency' dose with them to deal with unexpected stresses. And they have to take extra doses, for example when they're fighting an infection. If levels of cortisol are too low to help the body cope, then the person can suffer some sort of crisis and black out. I'm not quite clear on the details yet, but surely Kirk will learn more from the consultant later this week.

But, ever the optimist, I now look forward to seeing my husband gradually improve as all the different replacement therapies are tweaked and balanced out. With any luck, his condition will stabilize and he might be able to regain the strength, health, and fitness he has lost over the past 18 months of deterioration. Maybe, in a few years, he might return to work? If only the tailored exercise and rehab programs that are recommended for his condition were actually available on the NHS. But that would mean they have to have personal and sustained attention for a patient with individual symptoms. Good luck with that.

Optimism and reality don't match.

Get well soon!

One of the worst things about Kirk's broken pituitary is that nobody understands what's wrong with him. He's been home for more than a year now and unable to work. Summer before last, he finally gave in and stopped working. The GP could find nothing wrong and was about to write it off as 'stress' or chronic fatigue syndrome. After some research on the internet, we guessed it might be an endocrine issue and as Kirk had private insurance through work, the GP grudgingly gave a referral.

Turns out it wasn't stress. It was a pituitary micro-adenoma; a benign tumour on the pituitary gland that was causing the pituitary to shut down. Unfortunately, this is a chronic condition so after the diagnosis, we were sent back to the caring surroundings of the NHS.

More info on http://www.pituitary.org.uk/

The pituitary is responsible for releasing several hormones into the body. You might think an adult doesn't have much need of hormones but nothing is further from the truth. Thanks to hormones, people can recuperate from the stresses of everyday life. And I'm not talking about those things that are generally considered stressful like angry bosses, deadlines, paying the bills; or physical stresses like running a marathon. Everything little thing we do every day puts some level of stress on our body and mind. That's why we have a rest, eat some chocolate, and sleep. Mostly while we sleep, but also generally throughout the day, our pituitary releases hormones as we need them. That's how we add and maintain muscle mass and bone density; it's how we deal with the mental stresses of the day; it's how we build up strength and resilience.

Without his pituitary functioning properly, Kirk has been losing muscle mass but gaining weight, he's developed memory and concentration problems and easily becomes agitated or even depressed, he comes down with every infection that is going around. He's constantly fatigued and in pain. Resting doesn't help and only prescription pain medication even takes the edge off.

And there's where it gets annoying. Try and explain this to all those people who unwittingly ask: "How are you?" What silly suggestions we get! Everybody is tired after a long day of work and school runs and housework. Everybody is so used to hyperbole, that the meaning of the words 'fatigue' and 'pain' are lost. Have we tried their favourite brand of multivitamins? Perhaps some acupuncture or pilates will help? If some other part of one's body stopped functioning, like a leg or a lung or even a heart, would anyone suggest herbal remedies and a cranial osteopath? Of course not. But it's just one of those 'invisible' illnesses, isn't it?