Wednesday, March 25, 2009
Wednesday, March 18, 2009
> something else but seems was wrong. Found websites to be vague. My
> disease is aggressiveand has been with me only since Nov 2008 when i had
> a viral infection. What happens next!!! My Gfr is 26 and im at stage 4
> and im being put on iron iv and epo injections along with Bp beta
> blockers and collestral tablets. I am 49 2 sons a wife and 2 cats. The
> consultant says its incurable and untreatable and will lead to
> transplant sooner rather than later, is it really as bad as this???
Welcome to the group.
I'm going to try to put your situation into some kind of perspective,
but it's going to take some explaining, so please bear with me.
IgAN itself is not renal failure. It's a condition that can lead to
it. It really doesn't do much of anything except cause immunoglobulin
A protein complexes to deposit and gradually build-up in the "filters"
of the kidneys. As this process carries on over many years, mostly
unseen and unnoticed, kidney function (measured as glomerular
filtration rate) declines, until eventually, there isn't enough left
of it to support life without dialysis or a kidney transplant.
Sometimes, it's so benign that the person has already had IgAN for
years before it's discovered, simply because there hasn't been any
symptom to check out. This may sometimes be the case when a person is
suddenly "diagnosed" with IgAN when kidney function is already
seriously impaired. What triggers the discovery is often a viral or
other infection that causes some degree of acute renal failure in
connection with the already underlying and unsuspected IgAN. This
often causes a biopsy to be performed, and lo' and behold, they find
the characteristic signs of IgA nephropathy in there. So, now you have
IgAN, but what you really have is chronic renal insufficiency.
Some of the acute loss of kidney function under those circumstances
may or may not be permanent, so, I would have to be cautious about
making any assumptions based only on short term information about GFR.
After some time, it's possible that some of the lost kidney function
will be recovered, but probably not all of it. For example, you might
be at an estimated GFR of 26% now, but it might stabilize around 35%
or more after the acute crisis resolves. But it really doesn't matter
that much other than buying some time. These GFR estimates are not as
accurate as counting pennies. It's not like today you have 26, and
tomorrow you have 25.
Once kidney function is down below 50%, chances are that the person
would need renal replacement therapy (dialysis or transplant)
eventually. This is because irregardless of what kidney disease caused
the decline in kidney function, having less than half of it left is a
condition that feeds on itself. Loss of kidney function causes more
loss of kidney function (even if the original kidney disease were to
be magically cured).
What we can't predict, without the person already having a few years
or at least months history of known declining GFR, is when renal
replacement therapy might be needed. It takes a longer history of
these numbers to be able to make a good prediction. Some people could
stay in the 20 or 30's for years, while others might decline faster. A
GFR of 10% is usually about when dialysis or transplant is needed
(although a person might be able to live without it for some time even
All the above is my best attempt to explain why dialysis or transplant
will probably be needed, and to explain how at some point, it's not
the diagnosis of IgAN that matters so much as it is the diagnosis of
the fact you have somewhere in the area of 25-35% kidney function. You
might say that at this point, your disease is more "chronic renal
insufficiency" rather than the IgAN that caused you to get there.
Generally-speaking, it's prudent to start thinking ahead about renal
replacement treatment choices when a person is at the level of GFR you
are. This is because there are choices to be made eventually about
dialysis methods and transplant arrangements. Woe the person who
denies it and waits until he or she needs emergency hemodialysis via a
neck catheter some night and has to be rushed to hospital by
ambulance. Much better to have been prepared in advance with an
informed decision about dialysis method (peritoneal or hemodialysis
and the various flavours within those categories) and transplant
options explored. It's a lot less stressful to both mind and body when
you can be eased into dialysis before it becomes an outright
emergency, or you can get a pre-emptive live donor transplant because
you have had time to have both yourself and volunteers evaluated for
it. This all takes considerable lead time.
Don't despair. I have no idea what will happen in anyone's case, but
all I can say is that I had IgAN for years, was on dialysis for 4
years, currently have had a kidney transplant for over 2-1/2 years...
and I feel better now than I did the 2 years leading up to dialysis.
There are no guarantees for me or for anyone, but that's the way it is
in the present, and it's the present I live in.
About 7-8 years ago, before I started dialysis, I had declined to the
point where my beautiful, custom made-to-measure all-Italian racing
bicycle mostly stood against a wall of my living room as a memorial to
my past glories (no, never been a racer, but I was an enthusiastic
recreational rider). At that time, I could barely ride the damned
thing around the block. Very gradually, after I had started dialysis,
I felt well enough to gradually start riding it more and more. I felt
even better after I finally got the kidney transplant a couple of
years ago. I was able to gradually increase my riding distance from a
few blocks until eventually I was able to easily ride 30 km in less
than a couple of hours. Since, I've ridden even longer. It's not much
in the grand scheme of things, but for me, it's just totally amazing.
But at the very least, it does help me show others that there is hope
after dialysis and kidney transplant.
Best of luck to you, and here's hoping that dialysis or transplant can
be delayed far into the future. Life doesn't come with a guarantee of
health, but we can certainly make the best of what we have.
Monday, March 9, 2009
wondering if for me a higher hgb target might be a better option. Of
course I can discuss the pros and cons of epo shots with my neph, and
I have, quite fully. But with modern medicine I think often the
discussion for an individual patient can go into territory beyond what
the current dominant methodology of the evidence-based model
proscribes. Evidence-based medicine is great, as far as it goes. But
what comes out of this methodology, generally, are general outlines
for the average patient. The problem is, some of us don't fit the
profile of the average patient.
I think that with the black box warning on epo, pushing my neph
to go higher than the 10-12 range is asking quite a bit. Especially
since my investigation into the whole issue is far from complete. In
my experience, in this modern world of the internet and interconnected
libraries, Pubmed, etc, a motivated patient can become a mini-expert
on any given condition. What the patient lacks of course is clinical
experience, the ability to read between the lines b/c of years of
seeing patterns and having strong hunches.
As you suggest in your post, (and a bunch of other authors suggest
in papers written about the CREATE and CHOIR studies), there is a lot
more that is unknown about the issue of epo and ESAs. And I'm
thinking that one group that is motivated to explore the boundaries
here are patients who have the most at stake.
Its great to hear back from other people with their individual
experiences. It helps fill in the puzzle. Like Betsy's experience with
her son - the filling in of lived experience that helps all of us know
enough to pursue the best clinical outcome. The randomized,
placebo-controlled, double-blind trials give us the big picture,and
warn us of things we might not see coming. And then I think there is
room to become unblinded, so to speak, and look closely at whether or
not we match the phenomenon the study was looking at. Its tricky, to
say the least, but also the new frontier.
I saw it when I worked in an HIV/AIDS clinic in the mid 90's -
guys who were on the internet every night pushing the boundaries of
what was then known were typically the first guys to get ahold of the
triple therapy and then some of them, I dare say, may have saved their
own lives. Of course these same guys were sometimes annoying as hell,
but that comes with the territory !
Again, thanks ! - I'm getting some of the geeky discussion I was
looking for, after all!
studies but thought I'd pass on some info related to my son's
experience with EPO that is somewhat related. At first, his neph
referred him to a hematologist because he had a familial anemia as
well as the anemia of CRF. This guy was determined to "cure" the
anemia, but what we discovered was that as Ryan's hematocrit
approached (and often went beyond) the normal range, his blood
pressure would destabilize. He did not have a history of hypertensive
problems but was on lisinopril for the kidney-protective aspects and
as kidney function declined his natural bp did creep up some. When his
hematocrit was in the normal range as a result of EPO injections, the
blood pressure went way up and caused headaches, difficulty sleeping,
etc. Then the hematologist would cut back on the EPO and we'd have to
wait for Ryan to crash, hemoglobin-wise. It was a whip-lash pattern of
highs and lows, and I got frustrated with the hematologist's attitude
and ask the nephrologist what he would do if he were managing the EPO.
He had a more conservative, steady even if mildly anemic approach, and
my son quit the hematologist and let his neph manage the EPO with much
more satisfactory results. Although you'd like to get rid of the
anemia, a mild level of anemia seems to avoid the high and fluctuating
blood pressure issue, and the subsequent damage caused by that. What
my son also found was that he adapted to the slightly anemic level
maintained by the more conservative approach to EPO dosage and was
able to be quite active. He is not a high-endurance athlete but does
ski and hike (including 14'ers - we live in Colorado were hiking above
12,000 ft. is readily available).
Monday, March 2, 2009
> I am someone that lurks but as my family has had some experience with
> what you are asking about I decided to respond.
> My husband was DX Apr93 at the age of 39 in acute renal failure
> w/IGAN (no earlier symptoms that we knew of), he rec'd a 6/6 antigen
> transplant from his brother Oct93 that lasted until Apr03 his failure
> in 2003 was a reoccurance of IGAN. Our son donated (Apr03) but then
> this kidney failed DX Jan08 due to chronic failure. He's been on
> dialysis since Jan09 but we have been cleared for another transplant
> scheduled for Aprl 7. I will donate this time. He has been told that
> he has a very aggressive form of IGAN. So we have gone into each
> transplant hoping for longevity but have known that there isn't any
> guarantees and to do the best you can and to make the most of each
Sunday, March 1, 2009
working on PD, and then with a transplant. This assumes employment
that isn't too physical and that allows a bit of flexibility, and it
assumes you understand that everything about PD or transplant doesn't
work perfectly all the time. By that, I mean you have to expect some
inconvenience and some unforeseen problems. For example, you could be
running just fine on PD for months, and then all of a sudden, you've
got a peritoneal infection. When that happens, you might need some
hospitalization time, and then you might have to be on hemodialysis
for a few days or weeks until the infection is totally cleared,
depending on how severe it is. Or, if you start having some problems
with pain or whatever, they could have you come into the PD unit for a
day or two. This is where flexibility in the workplace comes in.
Or you could decide to do your PD overnight with the automatic cycler,
but then find out that it's not giving you enough dialysis, and now
you have to keep doing the overnight routine plus one or more twin bag
fluid exchanges during the daytime. There's just no way to predict
these kinds of problems.
Transplant is kind of the same idea. I mean, you never know when you
might need to be hospitalized for an infection or something. Just
don't expect to work the first month, and realistically, a couple of
months after the surgery. You will probably also tend to catch more
colds that go around the office than most people do, and when you do,
they can last longer and make you feel worse.
When you have little problems that crop up, you have to go to the home
PD unit, the transplant clinic, or the hospital, and that's not
counting other appointments with various specialists they might set up
for you. So you have to be available for that, because you usually
have to take the appointments as they are for the most part.
So, as long as you know what to expect, and you do have a bit of
personal flexibility in your job, there's no reason not to continue
working. That being said, I know a lot of people who are on various
forms of dialysis or who have a kidney transplant. Some work, some
don't. There can be no guarantees. Ultimately, you end up doing what
you have to do, because your life depends on it.