Meds

[amjm]

My son vinny has alagille syndrome. He has several meds to take. takes them all with no probs except sodium bicarbonate. we have been mixing it with diluting juice. Does anyone have any other methods that might be a bit tastier.

[Twick]

Hi, our son has alagille syndrome and we has recently been prescribed sodium bicarbonate as well. He is over a year but still on heparon junior so we just mix is in and he takes it okay. I guess that your Vinny is a bit older though? I haven't posted on here previously but I have been meaning to get to asking other parents how they are getting on with alagilles children.

Hope that you are all doing ok.

[amjm]

Vinny will be 2 in July he's on genarade plus milk powder. Do you mix with the whole bottle or after he's drank a bit. Vinny has been put on transplant list since jan. he's the youngest of three the other two are fine a right handful though Reece is 7 and Ryley is 5. Vinny keeps us awake with his itching. How do you get on with your son.

[Twick]

Hi, Oscar is tiny, below the 0.4 percentile for height and weight. We are trying to get enough nutrition into him but he just won't eat most of the time. We have been giving him duocal and have just yesterday been maxi joule but this was only after hearing of other parents talking about alagille feeding. He is itchy but we use a lot of e45 and e45 kids bath milk. We also have some antihistamine but can only use it occasionally. And the rifadin dose has been upped in January which helps a bit. Have you seen 'scratchsleeves'? Not cheap at about £8.45 but they help.

We mix the sodium bicarbonate into the whole bottle. Usually for a dream feed he will have about 170ml so he gets most of the bicarb.

Glad to know that your other children are well but sorry to hear that Vinny is on the transplant list. Has this been mentioned for some time because Kings have skirted around the issue a little bit and mentioned it in passing but we were under the impression that only a low percentage of alagille children will need a transplant. I guess that might have been optimistic though.

Which hospital is Vinny being looked after by?

[amjm]

Vinny is being looked after by kings as well. We have to go back in June for a check up we're not their often as we live in scotland but vinny is in and out of the hospital in dundee every couple of weeks. We were at kings in October for a pre liver transplant examination their were complications as one of the arterys in vinnys heart is squashed. so test were done to make sure he could with stand the op. we got the ok that was a relief as they were wanting to operate on his heart. Vinny takes 4 capsules of the sodium bycarbonate a day. is Oscar on the capsules aswell? Seen the scratch sleeves ordered some thanks for that could have done with them from day one. Vinny still doesn't feed well still on about 5 to 6 bottles a day. He likes cereal and he will nibble at most things but not great with food. Milk is main feed don't know how long we can keep that going.

[Twick]

Hi, it must be a lot of expense and stress having to come to London for you. Oscar has just started on one capsule. They said that it might make him eat better but we will see about that.

Oscar has pulmonary stenosis and we have had some range of opinions as to whether anything needs to be done. It is so upsetting to be told one time that he needs an operation then another time and another doctor says it is okay then back around again.

Oscar goes between nibbling and on rare occasions happily eating a bit. He will not usually let us feed him though. Kings have advised us to try to give him 700ml which by my roughy calculation of his size to mine would be like me drinking over ten litres of formula per day. So we are wondering if this has a lot to do with his lack of appetite... I understand that children with alagilles can't absorb food as well as other children so they need the formula but still think that he should be learning to associate hunger with feeding himself.

We've just ordered some more scratch sleeves but stupidly I ordered 9-12 month but it will be a while before he fits them so I will order some 6-9. Oscar scratches himself when ever he can get at his skin but he tends to scratch his ears the worst and often wakes up crying covered in blood if we haven't got anything to put on his mitts.

[amjm]

Vinny is always scratching inside the ears aswell and his forehead is covered in scars. He has pigmentation marks all over his body it started on his hands at first now it's everywhere it looks like scars. They have told us its caused by the cholesterol deposits that the body struggles to break down so try's to escape through the skin. Are you and Oscar at kings often. How's his talking? We had the speech therapist last week and they explained that his speech is the same as a child that has just turned 1.

[Twick]

Oscar has the same marks and he has some of the little white marks that look like spots that are caused by the bile build up. I never read up on this sort of biology before but the liver turns cholesterol in to amongst other things bile which is why the cholesterol builds up in their little bodies. We were very upset as at first it looked to us like his jaundice was getting better. The doctors at Kings mentioned that there might be a new medicine coming into use this year that might help with the cholesterol and itching but I don't remember exactly what it is or when it might be available.

We have to go to Kings for three month check ups and also to Brompton every three months for his heart check ups. We are fortunate enough to live around London at the moment so although the hospitals are still awkward to get to we don't have to stay over. I hear that Birmingham children's hospital is excellent though. My wife is frequently at our local hospital though for check ups and blood tests. He's had a few week long stop overs there for infections as well.

Oscar started to say a few words like momma and dadda around December and he is making a lot more speech sounds now but I would guess that he is somewhere around about where he should be. Kings never mentioned that there is a connection between feeding and speech development and it was a very good dietician at our local hospital who pointed this out. They gave us also me time with a speech therapist and typically despite usually being full of himself and loud he didn't make a sound.

About his feeding, we have cut one bottle a day for the past few days and he has been much happier to eat food. We will monitor this progress but we hope that it continues. I know that he can't absorb food as well as other babies but if he could just start to eat more protein it might help. Sometime we feel like the dieticians at kings only answer is to add another scoop of formula to up the concentration and it is frustrating.

It is such a lot of emotion and stress and heartache and wishing that they could get better so we know what you are going through. It would be good to keep in contact to share experience. It was only reding these pages that we found out about some of the dieters supplements.