Alpha 1

[Vickie362]

Hello, first post on here!

I've never had any support since my daughter was diagnosed at 4 weeks (shes now 2) and I just need a 'friend' to talk to. I feel as though I'm naïve about her condition. I've read about it but I've never been told how serious the condition is? I've been told there is a risk of liver transplant but that is all. We sore our consultant at BCH when she turned one and aren't seeing him again till she is three? Feeling really worried!

Thank you

[JenColeman]

Hi Vickie, my son also has alpha 1. He was diagnosed at 7 weeks old and is now 21 weeks. I have also read lots about it but have never really had any doctors explain much to me. From what I understand they have no idea what will happen with an alpha 1 child, there are a few different routes it could take. They could fail to put on weight as a baby, and end up with a transplant before their first birthday, they could start suffering from emphcyma in their twenties, they could have a completely normal life and just suffer from shortness of breath in their 70's or I believe they could live a completely normal life with no problems at all. I have also read about them suffering from liver disease due to the alpha 1 being trapped in the liver but I'm not too sure about that. We have just been back to kings for our first appt since diagnosis around a month ago. We are still awaiting our blood results despite numerous phone calls so not sure what if anything has changed since we first found out about his condition. He is doing really well and continuing to put on weight which is good. How has your daughter been doing over the last 2 years? There is a page on fb called liver mums, if you want to send me a private message I can get someone to add you too it. There are lots and lots of helpful people on there who are in the same situation x

[kody2010]

Hey Vickie, I have a little boy with alpha-1 who is now 4 and diagnosed at 9 weeks after becoming bery poorly.. Alpha-1 is very unpredictable and can go one way or another or one day they are fine next day they are ill! Kody has never really have normal liver functions and poor weight gain and had to be NG tube fed until he was 2 and still struggles with his weight.. CLDF are fantastic for information so if you ask them they could pass you some on? And Jen is right the Liver mums group has been a life saver to me!

Hope all is well,

Megan x

[Vickie362]

Thanks for your reply girls.

Have emailed you Jen so hopefully can be added to the facebook group.

She's been generally well over the last 2 years, she struggled in the beginning but is now like any other 2 year old and is now classed as being 'chunky'. Really pleased with how shes progressed. She only has to take multi-vitamins now. How is your little boy? x

[kody2010 in reply to Vickie362]

He's just out of hospital actuall his little body can't seem to fight infections just as well he has progressed Portal hypertension due to alpha-1, so every 2 years he gets an endoscopy to make sure no varies need banding he's still on a lot of meds mostly vitamins but Urso and another two for itch! I find if the can keep weight on it helps them so much! We are from Northern Ireland so our local children's see kody every 3 Months and then doctor mckeirnan from BCH yearly glad to hear your little one is doing good xx

[Vickie362 in reply to kody2010]

We're under dr McKiernan too !

[bluenat77]

Hi Vickie ,my son Matthew also has Alpha 1 he was diagnosed as a baby and was very poorly for quite a while as local hospital telling us all was ok eventually got an appointment at kings college hospital children's liver unit where the staff where brilliant . Matthew is now 7 years old and doing well he only has appointments ever 2 years but if we ever notice any changes we can go back whenever or ring a clinical nurse there to chat over what has changed ie, yellowing of the whites of his eyes , weight loss , pale stools or just generally not right. Other than his medication on a day to day basis he just gets on with his life as normal plays football plenty of exercise and having grown up with it and us teaching him as he grows he has become used to the stuff you need to try to get him to avoid ie, people smoking , spray can deodorants and any other lung irritants for the lung side of alpha 1 . And as for things to avoid liver wise mostly medication like ibrobrofen which unbelievably some hospitals and gps will still try to give home but stand your ground and insist they speech to your consultant at the liver department. If you ever have any concerns you can always speak to someone at the hospital or someone at the cldf they are brilliant and always there to help . I remember how hard it was at first and struggled to come to terms with it, but it does get easier . So much I could say really so if there is any think else you want to know just let me know and will let you know whatever I can

Natalie

[Vickie362 in reply to bluenat77]

Thanks for your reply Natalie. I had no idea about avoiding ibrobrofen. We were told it was ok as her liver function has returned to normal. Thank you for letting me know as I will now avoid!

[bluenat77]

I also read that they should avoid red meat as much as possible as the liver struggles to break it down. Matthew loves shepherds pie so we let him have it sometimes but not regular. Luckily he's not a huge meat fan anyway.

We're off to KCH in December for lung function tests and liver check up. Fingers crossed all ok.