Got our GOSH results today, not sure what to think

[bambiandthumper]

We got the results from DTD's biopsy today...they want to preform a radical nephrectomy remove her kidney with the lowest function :(

In a way this is good as they are almost positive it is damaging her 'better' kidney,and so should stop its function declining, but what if it doesn't stop declining, or it stays at the same function?

I know people have kidneys removed the whole time due to transplants ect.. and manage fine but I'm concerned as she already has decreased function in the surviving kidney, and what if it continues to decrease for what ever reason (she is more vulnerable to infection) then what will she do?

I know its GOSH and this is obviously the best decision, but it just seems so...drastic

I am not a kidney expert, but do they know what the cause is? What symptoms did she have before going in, was it kidney infection or something else? How old is she?
(in fact I have a friend who is a kidney dr, might ask her view next week for you)

Thanks nightcat, thats very kind of you :)

They are not sure what exactly 'it' is, but it appears her 'bad' kidney is damaging the better one. Apparently this is very unusual and so they think the best thing is to remove the bad one so it doesn't continue to impair the better one. Her Consultant said this isn't a treatment plan per say, its just really what they can do to get function back to normal, and removing the problem could be all thats needed, fingers crossed! Its more the whole going into the metaphorical uncharted territory thats worrying me.

She had HUS which damaged her kidneys, though there were no outward signs of this, we only found out on what was supposed to be her final check up.
Here was the original thread ,after we were referred to GOSH they preformed a biopsy of her 'bad' kidney, which I think has formed the base of their decision.

Hi bambi - I've been looking out for your results. Sorry it's not been brilliant news and yes this does seem v scary. But if that kidney is irretrievably damaged then she's better off without it. Her function has declined with it in situ, I think that leaving it there is very unlikely to promote a recovery.
It's a tough situation for you . Worst case is that she has this kidney removed, her function still declines and she needs dialysis/transplant. That's a very challenging situation and it will affect the rest of her life - and yours - but if it's coming, it's coming and it can't be prevented by any choice you do or don't make.
There's a good chance though that this will do the trick. Definately a chance worth taking imo.
Are you going back to GOSH to talk some more? What's the next step?

Bambi, I will chat to my friend later in the week.

If I were you I would probably try and ask drs lots of questions, especially on ALL possible options regarding the treatment.

Afaik, kidneys being at the end of the process of clearing out metabolic end products wouldn't be failing without a reason (my ds was at hisk of hemolytic anaemia so I had to look into it a bit in the different way).

Has your dd had a totally normal liver function tests? Was it possible to establish that she may have had e-coli to explain the kidney problem?

My ds liver function was low and he had a risk of kidney damage b/c of that. There is one enzyme called G6PD which can lead to hemolytic anaemia, it's one of the enzymes that process glucose and deficiency is apparently common but often symptomless at early stages.

eg here

This may not be what's affecting your dd, but if I were you I would try and get copies of any tests and look through them yourself too.

Or, has a possible autoimmune cause been mentioned? That would be another line I would investigate.

Kidneys shouldn't be failing w/o reason, there might be something earlier in the metabolic process that is possibly not working as it should putting a lot of stress on them. I know people can live with one kidney and of course on dialysis too, but maybe there is a way to avoid it by exploring all possibilities.

Talk to the drs about all angles and find out what else they may have found out about your dd metabolism apart from the fact that they biopsied the kidneys.

I was in a desperate place with my ds where drs told me there was absolutely nothing they could do, but we managed to reverse some damage through diet and my ds is doing far better then expected.

What are the timescales for your next appt?

Its in about 3 weeks as he wanted to give us time to think about everything that had been said, but I think this option is probably the best one they could come up with, and her consultant did say this failure as a result of HUS is very very unusual. They have done liver function and every other test, all of which came back as normal, just the GFR which was very low.

Perhaps fortunately her kidney's are not failing without reason, its a a result of the HUS, of which she had the most atypical type of (the type not caused my pneumonia or e.coli), and kidney failure is one of the rare long term effects of it :(.

I will definitely ask about the GPD6 deficiency and metabolism and diet though, as even if they do not cause it it could definitely impact on it -either reducing or increasing function. At the moment, apart from nursery and friends houses, I am cooking all meals from scratch, making sure not to use salty foods and adding no extra salt to the DC's meals, and I make sure DTD drinks lots and lots of water and cranberry juice.

I'm very sorry to hear about all you had to go through with your DS, it must have been so worrying.

Sorry Northernlucker didn't see your message. Thank you so much for thinking of us, and your right, she won't get any better if we just leave everything as it is, its just so hard to believe as you could not tell anything is wrong from looking at her.

Thanks *pixie, I think it would be almost better in a way if she was very tired, or looked unwell in some way because then you can see the need for it, its just the fact she is like any other 3 year old, and looks the picture of health that makes it harder.

But then again you wonder how low her kidney function would have to decrease before we noticed something was wrong, which is even more worrying in a sense.

We have got a while to mull it all over, its just a bit of a shock at the moment.

How is your DD2 getting on?

That is such good news that they are pleased with her progress :) I am right in thinking she is in her last year at prep?

Is she ok about not going back, in terms of missing all her leavers things and friends, or will she be able to go back to visit. Also if she is going to a senior school that would need CE, have you looked into getting dispensation? My DH had it as he had appendicitis over the exam week, but still got in.

Your right, theres no point worrying about what could have happened as it hasn't, and I know GOSH would only operate if necessary, so i'm not worried from that pov, and knowing her she will be fine whatever happens.

One of my friends in school had only half a kidney, they removed one and then later had to remove half of the other. The surviving half worked well enough for her not to need dialysis... although she was under close observation and needed to go to the loo every half hour or so.

Bambi - I think I may have mentioned this already on another thread but it is not at all uncommon for people to not show significant symptoms with kidney failure until quite late on. It's really good that your dd is so well and long may it continue but as you know it doesn't mean that there isn't a potentially serious issue that needs to be treated.
It sounds like you are doing all the right things to help her and she will be in great hands.
Nephrology is their life's work for most people working in that field. There is something about it that seems to grab you. I certainly feel that and I'm only the admin person! The team looking after your dd want to do their very best for her and for you. You're in good hands.

You know Bambi, if it was me I would want to have copies of any biochem tests, eg liver, kidney etc for my own records. My ds tests were sometimes described as no significant abnormality, but on close inspection I found some results to be borderline high or low and they later proved to be significant and meaningful. I just kept asking questions as I wanted to understand exactly what was going on. I am still working through some of my ds tests to check each and every item that is borderline even tho the overall coment was "normal".
If your dd proposed treatment is not a routine one, I would also want to know all pros and cons and also the success rate and how often it's done and with what results and what prognosis. Rather than sit and wait 3 weeks for the appt you could list all the questions in the letter and ask for copies of relevant test so you have time to get to understand it all and prepare more questions.

Thanks summeRain, that is really reassuring, hopefully we'll stop at one kidney though!

We will be asking about that nightcat, and one of DH's good friends who is a doctor is very kindly going to find some studies looking at nephroctomies, and what the outcomes are in terms of future health.

We are currently making a list of any questions we have regarding her treatment, i'll probably end up posting on here to check I have't missed anything out :)

DTD thankfully is completely unaware of everything and on top form. She decided she wanted to do my lipstick for me, so I currently look like the joker.

If it's any small comfort dh had a nephrectomy at 7 due to a type of renal cancer which had also spread to the surrounding area. So since then he's only had the one kidney (and interestingly it's got 2 tubes or something which affects it's functionality) but he's 36 and fine (and a nephrologist himself ). We'll be thinking of you - it's very tough to make these sorts of decisions.

Got the BKPA newsletter thing at work today and it mentioned that they are putting £400,000 a year for 5 years towards the new renal unit they are building at GOSH. Sounds like the facilities are going to be absoultely amazing and there were quite a few references to the cutting edge research and treatment going on. I firmly believe your dd is in the best possible hands.

Thank you, both of you, that is really really sweet of you. I do know DTD is in the best hospital, being treated by the best doctors, and I couldn't ask for better care then we are receiving! But as you all know, when its your baby you just a worry a lot :o

HI bambi - just wondering how dtd is now?

Aww, i'm so touched that you've been thinking of us!

We are going to GOSH on Friday, but I think that is to go through the nephrectomy procedure, and possibly be given a date for it .

DH and I have done lots of research, and spoken to Doctor friends, and we have come to the conclusion that this is the best thing, as it is, as far as everyone can see, the only way to potentially stop her loss of function. But nightcat I will be asking for results of all her tests, not least because I am unsure of how this will affect our health insurance when we move to the States.

DD herself is great, and has been the only one of us to evade a horrible cold which DS caught, and then spread to the rest of us.

Pixie how is DD2, I hope she is improving, has she managed to pop into school yet? Also, may I ask how on earth you are getting about with a double pushchair and a wheelchair? And, perhaps more importantly , Happy Birthday for yesterday! I hope you had a lovely day and were incredibly spoiled.

Good thought about the insurance - yes I think it may make some difference. Don't stir from these shores before everything is sorted!

Sounds like you've got a firm grip on things and I hope your appointment goes really well. It's not an easy thing to face at all and I think you're doing really well to take such a sensible, measured approach.

I have to agree with you there Northernlurcker, the NHS is a godsend.

We were originally meant to be moving in the end of May, but I think the timeline GOSH gives us for treatment will have a bearing on that. NY apparently also empties out in the summer, and I really can't think of anything worse than being stuck in a hot city, not really knowing anyone.

DH isn't actually starting work there till the beginning of July, but i'm definitely tempted to stay this side of the pond for as long as possible!

How are you though Northernlurker? Hoping for some warmer weather i'm guessing?!

Bambi, all I can say is that I've spent the day at GOSH again today, and we just have to remember that it's the most superb children's hospital. Your DD is in such good hands there, and any decisions they make will be the right ones. My DS had a ruptured brain aneurysm in January - his chances of survival were very slim, but the fantastic Drs there saved him. We had to completely trust in them. I'm sure they will be as wonderful for your DD. Good luck.