RE: Langan- word from the nephrologist Can anyone decipher

[Guest guest]

,

This does not sound too bad to me. It says to me that the proximal renal

tubular is not working completely the way it should and dehydration has

enhanced the problem. This problem existed before the ketogenic diet was

started but it was not affecting her in a significant way that needed to be

treated. If they had understood the problem prior to being on the diet I

expect the specialist would have not treated it but continued to monitor it.

Unfortunately the diet has enhanced the proximal renal tubular problem.

But, now that you know what the problem is you can work with it. In other

words, the diet needs to be done in a way that keeps Langan well hydrated

(Shan was sensitive to fluids in the beginning but after about 6 months we

went to unlimited water) and allows for adequate bicarb. The good side of

it is that you now know what the problem is and you need to take into

consideration the problem to make the diet work. It is somewhat similar to

our discovering Shan's lactose intolerance after being on the diet for over

year except that she suffered a year before we figured it out. Now, we work

with it and are having the best success yet. I agree, med school would have

been helpful. Don't I wish I had all the medical knowledge and experience

to go along with our personal and group experience!

Be encouraged. You now have something to work with.

Rhonda

Langan- word from the nephrologist Can anyone decipher

Here is what the nephrologist just emailed to me. He is wonderful

and very accessible. It is actually 7pm in his time zone and yet he

is taking the time to write to me.

This doesn't sound so good. Can anyone translate? Does it indicate

something like mito? An amino acid problem? Something else? Is

there a treatment? Ugh. I should have gone to med school so I

could understand this stuff!!

Here is what he wrote:

I have now reviewed all of Langan's test results pertaining to her

acidosis, including the ones we did yesterday. I know you are

anxious

to hear from me, and here's what I think:

1. She has persistent metabolic acidosis, which clearly predates the

ketogenic diet therapy. In some cases, this is associated with

hyperchloremia and a normal anion gap, consistent with renal tubular

acidosis (RTA). In all the urinalyses done, the urine pH has been

normal, which is evidence for normal distal tubular acidification and

makes distal RTA unlikely. In all the urinalyses done, she has some

proteinuria, which goes along with the diagnosis of a proximal RTA.

Proximal RTA can be associated with several inborn errors of

metabolism, seizure disorders and developmental delay, and I know

that

Neurology and Genetics at CCHMC are working hard on trying to pin

that

down. It is not unusual for patients with proximal RTA to require

huge

amounts of bicarbonate therapy to maintain serum bicarbonate

levels. As

discussed with Dr. Holder, I suggest stopping the bicitra, increasing

the dose of sodium bicarbonate to 15/15/20 cc, and recheck bicarb

levels

in 1-2 weeks.

2. In some cases, Langan's metabolic acidosis is accompanied by a

high

anion gap. In the blood and urine tests done yesterday, her urinary

sodium and chloride levels were extremely low, her urine specific

gravity was high, and her serum bicarbonate level had dropped to 13

with

a high anion gap. This suggests a pre-renal component (mild

dehydration) which is contributing to the acidosis. Children need at

least 80 cc/kg/day as maintenance fluid requirements. In Langan's

case,

this translates to 800 cc/day or about 27 ounces of liquids per day

that

she would need to prevent her bicarbonate levels from falling.

However,

we need to also take into consideration the effect this may have on

her

ketogenic diet, which is working so well to control her seizures. I

will defer to Neurology and Dietary on the issue of what and how much

fluids Langan should be encouraged to ingest. I am copying this

note to

them.

Thanks!

" The Ketogenic Diet....a realistic treatment option, NOT just a last

resort! "

List is for parent to parent support only.

It is important to get medical advice from a professional

keto team!

Subscribe: ketogenic-subscribe

Unsubscribe: ketogenic-unsubscribe

[Guest guest]

I question how well this guy understands keto (the nephrologist we had

clearly didn't have a good knowledge base of keto even though he was

extremely intelligent and up on everything else) and why he would send

you so much doctor speak which clealry needs translation.

At one point, after having added diamox to diet to make Jess more acidic

because her body had learned to fight the acidity of the diet, a

nephrologist walked in the room and told me that Jess was in a state of

renal tubular acidosis. Naturally I freaked inside. Took a deep breath

and asked if it was caused by the diamox. He said probably. Went home

and researched RTA, very scary subject. Went back to neuro and asked him

which of 3 types of RTA Jess had. He said Huh? What are you talking

about? Who said she had that? I have no idea......

Now my neuro ain't no stick in the mud I tell you.

Point of the story........tread carefully around nephrologist (who are

excellent drs when it comes to issues of acidity and effect on kidneys

of peeing out too much calcium, in general) when doing keto. For

example, the diet is calcualted so as to keep body in dehydrated state.

It appears that trace protien in urine is common on keto diet although

no one seems to know why. These are normal keto diet findings. I think

he may, and I say may, be using them to diagnose some underlying problem

that may or may not be there for Langan.

So, why did he say Langan's acidosis pre-dated diet. If it did, someone

likely picked it up somewhere in her bloodwork pre-diet, and perhaps

should have told you. If it pre-dated diet it should have been an issue

discussed before starting diet. But I would want to know from what

source he got that it pre-dated diet.

Just my thoughts

langansmom wrote:

> Here is what the nephrologist just emailed to me. He is wonderful

> and very accessible. It is actually 7pm in his time zone and yet he

> is taking the time to write to me.

>

> This doesn't sound so good. Can anyone translate? Does it indicate

> something like mito? An amino acid problem? Something else? Is

> there a treatment? Ugh. I should have gone to med school so I

> could understand this stuff!!

>

> Here is what he wrote:

>

> I have now reviewed all of Langan's test results pertaining to her

> acidosis, including the ones we did yesterday. I know you are

> anxious

> to hear from me, and here's what I think:

>

> 1. She has persistent metabolic acidosis, which clearly predates the

> ketogenic diet therapy. In some cases, this is associated with

> hyperchloremia and a normal anion gap, consistent with renal tubular

> acidosis (RTA). In all the urinalyses done, the urine pH has been

> normal, which is evidence for normal distal tubular acidification and

> makes distal RTA unlikely. In all the urinalyses done, she has some

> proteinuria, which goes along with the diagnosis of a proximal RTA.

> Proximal RTA can be associated with several inborn errors of

> metabolism, seizure disorders and developmental delay, and I know

> that

> Neurology and Genetics at CCHMC are working hard on trying to pin

> that

> down. It is not unusual for patients with proximal RTA to require

> huge

> amounts of bicarbonate therapy to maintain serum bicarbonate

> levels. As

> discussed with Dr. Holder, I suggest stopping the bicitra, increasing

> the dose of sodium bicarbonate to 15/15/20 cc, and recheck bicarb

> levels

> in 1-2 weeks.

>

> 2. In some cases, Langan's metabolic acidosis is accompanied by a

> high

> anion gap. In the blood and urine tests done yesterday, her urinary

> sodium and chloride levels were extremely low, her urine specific

> gravity was high, and her serum bicarbonate level had dropped to 13

> with

> a high anion gap. This suggests a pre-renal component (mild

> dehydration) which is contributing to the acidosis. Children need at

> least 80 cc/kg/day as maintenance fluid requirements. In Langan's

> case,

> this translates to 800 cc/day or about 27 ounces of liquids per day

> that

> she would need to prevent her bicarbonate levels from falling.

> However,

> we need to also take into consideration the effect this may have on

> her

> ketogenic diet, which is working so well to control her seizures. I

> will defer to Neurology and Dietary on the issue of what and how much

> fluids Langan should be encouraged to ingest. I am copying this

> note to

> them.

>

> Thanks!

>

>

>

>

>

> " The Ketogenic Diet....a realistic treatment option, NOT just a last

> resort! "

>

> List is for parent to parent support only.

> It is important to get medical advice from a

> professional keto team!

> Subscribe: ketogenic-subscribe

> Unsubscribe: ketogenic-unsubscribe

>

>

>

>

>

[Guest guest]

,

Thanks for the tips. I do think this doc is pretty up on keto. he

said he was, and he was the one who pulled the bicitra b/c he said it

had too many carbs for the diet.

We are in the process of now trying to figure out if this pre-dated

keto or not. Through talking to him (and I am amazed at how

accessible he is!!), we have learned that the data he was relying on

was actually POST keto but before we started at Cincy- hence the

confusion. It was actually the 1/04 blood work that our old neuro

never followed up on. So we are trying to pull all of Langan's old

bloodwork (including the testing done to get a baseline for keto) so

we can see if they give any clues. He was the first to admit the

mistake about the date. I have been very impressed with him, and he

is working really closely with our neuro, so hopefully we can get

somewhere with this.

I know what you mean about the RTA stuff being scary- I got worked up

into a mess reading it. Hopefully we can make some sense of this and

use it to try to make some sense of what is going on with Langan. In

the meantime, our challenge is to keep her hydrated. He said she

needs a minimum of 27 ounces a day. Not since nursing has she ever

had that much fluids! So now we are constantly bombarding her with

it, and her dietician ok'd 1/2 a cup of jello a day as free food to

try to help. Wish us luck!

Thanks for everything! I'll keep you posted!

> >

> > I have now reviewed all of Langan's test results pertaining to her

> > acidosis, including the ones we did yesterday. I know you are

> > anxious

> > to hear from me, and here's what I think:

> >

> > 1. She has persistent metabolic acidosis, which clearly predates

the

> > ketogenic diet therapy. In some cases, this is associated with

> > hyperchloremia and a normal anion gap, consistent with renal

tubular

> > acidosis (RTA). In all the urinalyses done, the urine pH has been

> > normal, which is evidence for normal distal tubular acidification

and

> > makes distal RTA unlikely. In all the urinalyses done, she has

some

> > proteinuria, which goes along with the diagnosis of a proximal

RTA.

> > Proximal RTA can be associated with several inborn errors of

> > metabolism, seizure disorders and developmental delay, and I know

> > that

> > Neurology and Genetics at CCHMC are working hard on trying to pin

> > that

> > down. It is not unusual for patients with proximal RTA to require

> > huge

> > amounts of bicarbonate therapy to maintain serum bicarbonate

> > levels. As

> > discussed with Dr. Holder, I suggest stopping the bicitra,

increasing

> > the dose of sodium bicarbonate to 15/15/20 cc, and recheck bicarb

> > levels

> > in 1-2 weeks.

> >

> > 2. In some cases, Langan's metabolic acidosis is accompanied by a

> > high

> > anion gap. In the blood and urine tests done yesterday, her

urinary

> > sodium and chloride levels were extremely low, her urine specific

> > gravity was high, and her serum bicarbonate level had dropped to

13

> > with

> > a high anion gap. This suggests a pre-renal component (mild

> > dehydration) which is contributing to the acidosis. Children

need at

> > least 80 cc/kg/day as maintenance fluid requirements. In Langan's

> > case,

> > this translates to 800 cc/day or about 27 ounces of liquids per

day

> > that

> > she would need to prevent her bicarbonate levels from falling.

> > However,

> > we need to also take into consideration the effect this may have

on

> > her

> > ketogenic diet, which is working so well to control her

seizures. I

> > will defer to Neurology and Dietary on the issue of what and how

much

> > fluids Langan should be encouraged to ingest. I am copying this

> > note to

> > them.

> >

> > Thanks!

> >

> >

> >

> >

> >

> > " The Ketogenic Diet....a realistic treatment option, NOT just a

last

> > resort! "

> >

> > List is for parent to parent support only.

> > It is important to get medical advice from a

> > professional keto team!

> > Subscribe: ketogenic-subscribe

> > Unsubscribe: ketogenic-

unsubscribe

> >

> >

> >

> >

> >

[Guest guest]

Rhonda,

Thanks. It is always so nice to pass this by you guys! It makes me

feel like I am not alone after all. = )

It looks now like there is a question as to whether this preceded

the diet or not after all. The data the doctor was relying on was

actually date from AFTER we started keto. So we are collecting

other bloodwork records to see if there are any clues. I am

starting to think it is diet related. If Langan needs 27 ounces a

day to maintain, then there is no way that is happening. But now

that we know we are working on it.

Thanks again for the support!

>

> I have now reviewed all of Langan's test results pertaining to her

> acidosis, including the ones we did yesterday. I know you are

> anxious

> to hear from me, and here's what I think:

>

> 1. She has persistent metabolic acidosis, which clearly predates

the

> ketogenic diet therapy. In some cases, this is associated with

> hyperchloremia and a normal anion gap, consistent with renal

tubular

> acidosis (RTA). In all the urinalyses done, the urine pH has been

> normal, which is evidence for normal distal tubular acidification

and

> makes distal RTA unlikely. In all the urinalyses done, she has

some

> proteinuria, which goes along with the diagnosis of a proximal RTA.

> Proximal RTA can be associated with several inborn errors of

> metabolism, seizure disorders and developmental delay, and I know

> that

> Neurology and Genetics at CCHMC are working hard on trying to pin

> that

> down. It is not unusual for patients with proximal RTA to require

> huge

> amounts of bicarbonate therapy to maintain serum bicarbonate

> levels. As

> discussed with Dr. Holder, I suggest stopping the bicitra,

increasing

> the dose of sodium bicarbonate to 15/15/20 cc, and recheck bicarb

> levels

> in 1-2 weeks.

>

> 2. In some cases, Langan's metabolic acidosis is accompanied by a

> high

> anion gap. In the blood and urine tests done yesterday, her

urinary

> sodium and chloride levels were extremely low, her urine specific

> gravity was high, and her serum bicarbonate level had dropped to 13

> with

> a high anion gap. This suggests a pre-renal component (mild

> dehydration) which is contributing to the acidosis. Children need

at

> least 80 cc/kg/day as maintenance fluid requirements. In Langan's

> case,

> this translates to 800 cc/day or about 27 ounces of liquids per day

> that

> she would need to prevent her bicarbonate levels from falling.

> However,

> we need to also take into consideration the effect this may have on

> her

> ketogenic diet, which is working so well to control her seizures.

I

> will defer to Neurology and Dietary on the issue of what and how

much

> fluids Langan should be encouraged to ingest. I am copying this

> note to

> them.

>

> Thanks!

>

>

>

>

>

>

> " The Ketogenic Diet....a realistic treatment option, NOT just a

last

> resort! "

>

> List is for parent to parent support only.

> It is important to get medical advice from a

professional

> keto team!

> Subscribe: ketogenic-subscribe

> Unsubscribe: ketogenic-unsubscribe

>

>

>