Baby girl, dilated miocardyopathy from Argentina, need help

[Aleta]

Hi willy and Corrij. I haven't the English interface on the phone, so I pushed a lot of buttons deuch until I got here.

We talked to willy by facetime for about an hour.

Homework: try to get kidneys and liver status/ think about what changed in the last couple of months.

First I've called the pediatrician, she said that don't see as a good idea pin Lara again and put her in a sick child place (in which she is?), and ta she has no sing of kidney or liver problem. Next week we have appointment and she wants to have a long talk. She plans to do pro BNP next month again and see what happens with the new meds dosage and sodium resttinction. And wants to us go to meet another cardiologist.

I've email the resident that works with our cardiologist but still no answer.

Changes in the last 3 months since last echo: Diet and activity. As Lara was a little better cardiologist folds us that she could it ANYTHING, just no salt. So, as she is difficult to eat, we've started to give her kockies, pudin, cake, pies, chocolate, and so on. No crackers or chips, but almost normal diet. Even sometimes fries and pizza with no salt, but everything has sodium! Anything that comes inside a pack has sodium. I try to look what has less, but always a little has.

Activity: she started to walk around January graving the walls or fernature, and now she walks by herself and does not stop, she walks, climbing the sofa, and never looks tired. I believe that made her heart works harder?

So, they raised enalapril to double, spironolactone a bit and carvedilol is the same. All in pills now. Carvedilol was 4mg at day and now 3.125, they told us is the same but I can't see it!

In January we raise carnitine to 900mg.

January 7 we took her for the fast breathing scare, and cardio told us the numbers was the same, so I don't know what is going on.

In august we started full protocol, October was the fort improve, SF goes up from ‰20 to %28 and mitral valve insufficiency goes down from %85 to %80. December SF goes up to ‰29, mitral valve defficiency goes down to %70 and pro-bnp drops to 64.
Last week SF drop to %24, mitral valve is the same and pro-bnp goes up to 45.
Measures ddvi and dvsi are the same since diagnosis, maybe 1 or 2 mm more or less, so I believe the z score is better but no conformation from cardiologist.

If I don't get wrong, BNP goes up responding to the vasel working hard? It would de bad idea start with fish oil besides the super dosage (2,5 mg) of enalapril?

I'll be posting the reports soon.

Best regards

Aleta an sebastian

[Aleta]

Sorry, pro BNP for up to 459.

[Willy]

Two things about here diet

Avoid sugar and salt!!

Sugar has long term health consequences and to much salt translates directly to more fluid retention what is a heavy burden for the heart.

The metabolic docter changed the carnitine to 900mg / day I did know that. That is almost half the dosis we give to an adult 2000 mg = 70 kilo

Carnitine is not toxic but I would ask the metabolic docter to measure here blood value.

[Willy]

So the metabolic docter does not see any signs of toxic side effect in liver and kidneys, oke let's leave this for te moment

Hypothesis

What if you made a mistake with the pro-BNP in december 2014 and that the value was NOT 64 but 640

Then everyting can be explained.

Lara is improving from 640 --> 459

In august 2014 she had a 2300 and according to your info that dropped to a value 0f 64, well within the healthy reference range.

THIS IS A LOT in 4 months

Is the december 2014 value of 64 correct? Check it. Because if it was 640 then the lower SF value can then be explained by Lara's jumping around, i.e. wrong measurement

And we do not have to worry any more

[Aleta]

I¨ve just sent you by email the results from december and the ones from this week. It says 64, but what I find weird is taht the reference valie is not the same.

we did the test in the same lab thinking that they would use the same method and reference chart.

Lara does not eat salt, I don´t cook for her with salt, but she didn´t have a sodium restrintion. now she has. The pediatrician even told me to give her some times pizza (she was very confidence about Lara´s improvments).

Could be the sodium she is ben having??? almost everythin has sodium, what would be the limit of sodium at day for her?

[Willy]

First solve the 64 issue.

Check the laboratory result with the hospital

[Willy]

I saw the results on the lab forms

december 4 2014 64 pg/ml ref 0 - 263

march 21 2015 429 pg/ml ref 5 - 169

Do not undersand why the REFs are different, ask the docter about that

Schedule for late april the next pro-BNP blood test

[Aleta]

yes, we are trying to call to the lab.

about the Q10, remeber that we are giving Lara ubiquinone, not ubiquinol?

if you recomend a trand we can order for it, it will arrive in a month, or so.

[Aleta]

In the lab told Sebastianthat the difference in the reference chart is because they use diferent reactives, but if we or the doctor have a problem with the reading should talk to the biochimistry because is to technical to explain to us. ................................

[Aleta]

New hypothesis (meybe is crazy):

in the mornig Lara takes Carnitine, Fursemida, Magnesium and the Q10.

We wait 15 minutes between the magnesium and the milk.

we give her the Q10 right before the milk in order to have better absorption with the milk´s fat. But the Q10 (remember is a capsule full of powder that we solve in milk) has been prepared at the same time of magnesium, 15 minutes before she have it.

So, the Q10 is solved in milk for about 15 minutes or a while more if she fell sleep again.

Could be that keep it solve in milk for a while makes the Q10 loses the propertys? Just thinking about that, thats one of the things we´ve changed in the last months.

Regards,

Aleta.

[Willy]

That is something for Corrij to answer

She knows more about bioavailability

But...

Is this the procedure since january? And was it in 2014 different?

[Aleta]

I don´t know exacly the month, but was january or december, because we talked about give Lara the Q10 right before the milk about that date. before that I´ve give it to her at 12 (midday) no mather the milk, and it was prepared in the moment.

and as we give magnesium 15 minutes before milk or food, that is what is going on with Q10.

[Willy]

I think magnesium and milk is also not a good idea, is it Corrij?

[Aleta]

If we have to re schedule the supplements it´s fine, we´ll follow you.

Have in mind the meds schedule:

8 am fursemide (usually carnitine but we can change that if you think so), here we put magnesium and Q10 and then a bottle of milk.
12 (midday) spironolactone, carvedilol, enalapril (i give Ribose here)
14 pm fursemide
16 pm (all of this can be changed) ribose, iron (until she gets 10 kg), supradyn, D3 sunday and thursday
20 pm fursemide
00 (midnight) spironolactone, carvedilol, enalapril (usually carnitine but we can change that if you think so too) here we give the ribose too.
01 am Fursemide

In green is what we can´t change.

[Aleta]

Sorry, forgot the magnesiom at 20 pm too. 1/3 twice at day.