Baby girl, dilated miocardyopathy from Argentina, need help

[Tinus1979]

Hi Aleta,

Thank you very much for the information! Nice to hear the mitral surgery helped Lara!

[Aleta]

Hi Willy!
I hope things are doing well over there.

Lara had her check up a few day ago, but we went for a family tripo and here we are giving you the report.

Lara is great, her numbers are the same, the shortening fraction is %34-%36 inside normal range. The meds are the same, just Enalapril 1,3 mg in the morning and 0,7 mg at.

The Z-score the last time was 2.3, and now is 2.4. Tha worries me, but our cardio said tha is nothing to worry about because Lara grew 3cm in 3 months, so probably the heart grew a bit but not for the DCM. Even so, I'm worry.

Cardio told us that Z-score is a little big thn normal, and if she didn't knew Lara from before, she woldn't think that Lara has DCM, just a little enlarged like the sports heart. But as she knows Lara's past, she keeps treating her like a DCM patient, but she says that NOW Lara has no DCM criteria except for her past.

I guess this are great news, I'm very happy aboout it, but I know this can be tricky and the Z-score thing keeps me worrie.

We have to go back in november, a lot!!!

We are in the same dose with supplements.

Sending you big hugs!

Aleta.

[sasje1903]

Great news Aleta!!

We love you all! x

[Willy]

Excellent she slowly out gowths the DCM, that is what I expected would happen

the shortening fraction is %34-%36 inside normal range

6% to much max SF = 30%

Keep the sups on the same level, so she gets per KG a little bit less, we do not correct for weight until november cardio visit

May be a Face Time

[sasje1903]

Here i found Some normal values for Left Ventricle fractional shortning

https://www.echopedia.org/wiki/Normal_Values_of_TTE

[Willy]

Thank the cardio is correct in huis statement, within normal range

What was this value when we started?

[Aleta]

Hi, Willy and Sas! I didn't get the forum updates, that's why I didn't write back.

Lara's first shortening fraction at PICU was %19, after a round of levosimendan was %20. That was in june 2014, in august we started supplements and in october she got %28.
In December 2015 She reached %30 but she need mitral valve repair surgery. After the surgery she was in %25 and improve a little in every check up, unti November 2016 Lara reached normal range.

The Z-score went down always, but in january was 2.3 and in may 2.4, Cardio says is not relevant because Lara grew up a lot in that summer, but it worries me anyway. Check up in November!!! First time a six month check up, scary.

Thank you so much for everything, you are the best, really.

Aleta.

[Willy]

6 months is not scary

6 months = congratilation Aleta and Lara

The cardio trusts that everything Will be ALL RIGHT

PARTY TIME !!

[Aleta]

You made me SMILE

Thank you thank you thank you

[Aleta]

Hi Willy, long time no see!

Lara had her check up with cardiologist after six month, shortening fraction the same, %34-%36, heart size the same, but Lara grew a lot and gained weight, so Z-Score went down to 2.1! Pleaseee, she needs to keep on this track!

Also, meds still the same, only enalapril half of a 2,3 mg in the morning and a quarter at night.

we have to go back in six months! In may!!! This doesent't sounds so scary now because I see how she is growing and fills me with a lot of faith. I pray she keeps this way.

Cardio said next year we'll do a holter (never had one), and the other year a stress test.

We asked about those acids that were high and in the first clinic the methabolism doc prescribed Q10 and carnitine, and she said that we will repeat the blood work at 6 years to see what happened with that.

Also said that is totally on board to keep going with supplements.

We are thinking about change Lara (and Benjamin too) to another (better) school, she would be almost 8 hours there, now she is only 3 hours... Cardio said Lara can do it, she will have to sleep 10 hoursand we'll have to be stricts about that, I guesswe can, but I'm worry anyways. She will be five when the school stats again, but still.
Do you have a thought about that?

We can give her 2000mg of sodium at day, so now she can eat more stuff and never get that number anyway


So, that's the news! I hope you are doing great, and as always, thank you for being ther, support and helping us!

Best,

Aleta.

[Willy]

Excellent news!!

Cardio said next year we'll do a holter (never had one), and the other year a stress test.

Good idea

Do not understand the sentence about ACID and Q10 CAR

[Aleta]

Hi Willy!
At the biggining of all, in diagnosis time, in the first clinic we were reffered, they did a lot of tests. On of those was asked by the pedriatric methabolism specialist.
Those tests results was some acids a little high, and in case Lara has a mitocondrial desorder, they prescibed 150mg of Qo10 and L Carnitine. By that time we've met you, wo told you about this but it's been so ong that you must forgot lol
At the beggining we gave L carnitine made by Cassasco and the Qo10 made in a pharmacy, but you advice was give Acetyl L Carnitine and Ubiquinol and so we did.
We made those test a few times and thos acis were always a little little high, nothing conclusive, nothing to make sure that some mitocondrial stuff is happening, o not.
At that time the doctor said that some healhy children has those acids high too. The pediatrician said that she doesn't believe in mitocondrial disorder, and cardioolgist the same.

[Willy]

Sorry missen this post

What do you want to know from me?

I read your post but do not know what to answer

[Aleta]

Hi Willy< I was just reminding you how things were at the beggining with thos blood tests.

[Willy]

OKE

But does this info have any impact on what we are doing at the moment with Lara's supplement program?