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My baby has weak muscle tone and brain cysts...anyone else?

35 replies

Arabica · 18/08/2006 14:58

Hello
Some of you may already know my baby, Esja, was diagnosed with choroid plexus cysts and ventriculomegaly whilst I was pregnant. She was born on 27 July and has been in special care (transitional care) since day 4. It was quickly apparent that she was weaker and floppier in tone than an NT baby. Chromosone disorders have been ruled out and she doesn't have Prader-Willi syndrome and the geneticist said she didn't think her symptoms fit any other syndromes. Doctors at Hammersmith hospital where she was refeered for 24hrs investigation think it's more likely to be a brain issue than a muscular one but there are various ongoing metabolic investigations & an MRI scan soon. Her major problem is that because she isn't strong enough to suck well she lost a lot of weight soon after birth so have mainly been concentrating on feeding her up.
Just wondered if anyone had been in a similar situation (if so my sympathies) and/or if anyone can offer clues on how to stay long-term in transitional care (ie rooming in) without killing a nurse! They're lovely but I am SO sick of all the rules and being patronised (lights being turned off at night without permission being asked, being called 'mummy' instead of my name, etc...it really rankles!)

OP posts:
prettybird · 30/08/2006 15:48

A friend of mine's 4th daughter had question makrs abotu her when she was born. It took a loooong time to get a diagnosis - and even then, it is not a definitive one, as they haven't yet identified the gene, so the disgnosis of "Kabuki" syndrom is done more on the bais of the accumulation of mearkers.

These are (from memory): a particular exaggerated eye shape (hence the name, after the Japanese dancers), poor muscle tone, a mssing part to a little finger, something to do with the apearance/location ears, feeding problems (she ended up having an NGT tube - not a probelm for them as they are both medics), possible hip problems, possible growth problems.

Having said that, this wee girl, who is now rising 8, is a lovely happy wee girl and is my god daughter. She is small, and has a some mobility problems (she had to have her hips doen, and has an artifical "turn-out" to minimuse wear on the joint) and does have an LSA at school. But she can read, has friends and is very much loved. She may never be able to live independenlty, as she doesn't have a concept of danger, but who knows? My friend never thought she'd be able to read and she can, fluently.

I know my friend did have problems with HVs making silly statements like "you should be feeding her cheese sandwiches by now" - this was at something like 5 months, to a child who still had a NGT tube. And to a mother who was a GP herslef! But it goes to show that even GPs can be pissed off by HVs - and even she had a crisis of confidience and double checked with the ditiecian at the hopsital that she wasn't being stupid!

All of that rambling is really jsut to say you may never get a definitive diagnosis - but you will work out a way though it all and work out what she needs whatever the lable attached. You do have an adorbale wee girl who is loved and cherieshed whatever the ultimate dx.

Arabica · 31/08/2006 11:35

Hello everyone. Tube still out and she has put on some weight--not a huge amount but the nurse said we can leave it out until tomorrow and see how she does. I'm trying to give more feeds via bottle as we are fortifying the EBM with a scoop of formula. It's hard though as she knows the difference and bottles are definitely second beast (or even second breast!) Lovely to have her home, trying not to think about her MRI and what they might find, or not find.

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bundle · 31/08/2006 11:39

oh Arabica, that's lovely news! the reinforced EBM sounds like a pint of Guinness plus a whisky chaser! see you soon, xxxxxxxxxxx

saggarmakersbottomknocker · 31/08/2006 12:04

Good news Arabica . Will be thinking of you on the 12th.

hoxtonchick · 02/09/2006 18:43

so pleased to read that e is doing well. let us know when you're up for visitors, lots of love xxx

bundle · 05/09/2006 14:22

hi arabica, how was your weekend?

hoxty and I would love to see you but do say if that would mean too many visitors all at once!

bundle xxxxx

Marina · 05/09/2006 14:43

Fabulous news that you're home arabica, it really is So happy that you're sprung at last and all together.
Esja sounds as though she is doing so well on the feeding front!
Ds and dd know all about her by the way, and have been asking how she is. They will be delighted to hear that she has come home now. XXX
Wishing you all the best for the MRI on the 12th.

Arabica · 08/09/2006 23:32

Just to update you, Esja is still at home and still doesn't have her NG tube. She's still not putting on weight quite fast enough, and is still doing more than her fair share of vomiting, so the tube may yet make a reappearance. But she's bright and alert and her muscle tone is definitely improving. Nurse comes twice a week. Am also acquiring a filofax full of appointments with various health professionals including dietician, occupational therapist, portage, plus general child development clinic, genetics clinic...
MRI is on 19th but I think they'll talk to us about their findings the following month, rather than that day--more bloody waiting!
Thanks for all your good wishes. I don't come on here much any more, there never seems to be time.

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Jimjams2 · 09/09/2006 08:23

been thinking of you arabica

emkana · 09/09/2006 08:26

Hi Arabica good to hear from you again.
Am thinking of you often. XX

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