Today, September 4th is the American National Polycystic Kidney Disease Awareness Day (PKD)
It’s important to me because I have polycystic kidney disease.
Poly = many
Cystic = fluid filled spaces
In essence, instead of containing healthy functioning nephrons, the kidneys become non-functioning organs composed of many cysts.
There are different types but the more common is Autosomal Dominant PKD. 1-2 people in every 1000.
It’s genetic, inherited – I was born with it. My mother had it, her father, his mother … That’s as far back as we know.
“Autosomal dominant” means the abnormal gene is not on the X or Y chromosomes but on one of the others, and that having just one copy alone means you are affected. It also means there is a 1 in 2 chance (50%) that you will pass the gene onto your child.
If you are quick thinking you will have realised that for each of my children there was a 1 in 2 chance that I passed it on to them.
They know that. It’s a story we all live with even if it’s one I haven’t often told.
What happens is that over the years the kidneys, which are designed to filter and maintain a chemical balance in the blood, struggle and eventually fail.
Academically I find the physiology behind all this quite fascinating, but maybe that’s a defence mechanism by which I avoid the more emotional aspects of certain kidney failure.
The overwhelmed kidney gets confused and sends out messages that raise the blood pressure and that just makes the whole situation worse. The PKD patient will encounter antihypertensives early on and, although you know they are a “good thing”, you sometimes wonder if they don’t make you tired and slow. It’s a complex picture, other parts of the body step up in an effort to correct the disequilibrium but, not designed to do the work of a kidney, they muddy the field even more.
In medical school we were taught that a body can lose up to 90% of kidney function and still “go on”. It may mathematically be so, but I suspect that most patients with chronic kidney disease will experience some indication of impairment quite a bit before they reach the 10%-left-point. Nowadays the measure of renal effectiveness is the estimated glomerular filtration rate (EGFR). The downward curve below 60 is arguably more predictable than the stars, at least the astrological ones if not the astronomical. When it falls below 15 then you probably need some outside help: dialysis or transplant. Dialysis is just a holding measure.
Today in Britain there are 5616 people on the kidney waiting list. Actually phrasing it that way, on a waiting list, somehow distances it from the reality – there are 5616 people waiting for a kidney. So far this year there have been only 478 deceased donors. Just 703 kidney transplants have been performed this year. The numbers don’t compute perfectly because some donated kidneys aren’t usable and then some patients are fortunate to have living donations. But over 5000 people go to bed at the end of each day disappointed, more than disappointed, desolate.
On Bermuda the figures are smaller but tell a similar story. In fact it may be a sadder version because only 4 or 5 Bermudians each year will be lucky enough to have a kidney transplant. This week the dialysis unit on Bermuda will keep 160 people alive. Most of them will not get that longed for telephone call to tell them an organ match is waiting. Last year one of them did get the call but she couldn’t get to Boston in time for the operation and the kidney went to someone else. It is too painful to imagine how she must have felt.
Joseph Murray (1919-2012) was the surgeon who performed the first successful human kidney transplant, December 23rd 1954. He worked at Brigham Hospital in Boston. For this and subsequent work in the field he received the Nobel Prize for Medicine in 1990. In 2012, coincidentally and sadly the year Joseph Murray died, my daughter, then a medical student at SGUL, was involved in the development of a teaching case for their new approach of using PBL (problem based learning) and virtual patients for the delivery of the ethics curriculum. The virtual patient was named Joseph Murray. The scenario involved organ donation, permissions, dealing with relatives – if you want to try working through the case it is freely available, even has a video (look out for the patient’s mother – closest I will get to an OSCAR)
I was at home yesterday and in the unforgiving midday heat I sought respite inside and without any guilt at all sat watching TV. My selection, via AppleTV/UnblockUS/BBCiPlayer was a programme on tissue and organ procurement, The Human Tissue Squad.
It left me wondering just how organ and tissue donation works on Bermuda. Clearly it isn’t a big enough country to run it’s own procurement programme and there are no facilities for transplant surgery on island. So what happens?
Since proximity to US dictates a link to American healthcare, even though actually a British Overseas territory, Bermuda is part of the New England Organ Bank. So Bermudians can be organ and tissue donors – forms from doctors, TCD and post offices. But there must be huge logistical problems with a 3 hour flight each way to Boston, the closest transplant centre. In US and UK the donor-recipient distance has an effect on allocations and on outcome.
I was surprised to read about the differences in allocation processes between US and UK. In the States the system is to be enhanced to include a donor index which acknowledges that some kidneys are better than others for factors not relating to the specifics of tissue matching. Bluntly, the non-smoking-kidney will probably be better suited to the new job description.
A person where I once worked had a strange way of paying a compliment with “That’s a nice jumper. Can I have it when you die?” We all laughed, politely, if a little nervously.
Yes, you can have all my jumpers when I die – I won’t have much use for them myself.
But, you know that kidney? Can I have it when you die?