Portal hypertension but LFTs still normal.

[LisaLockley]

My 3 year old's spleen is now 13cm and ultrasound shows progressing p/h. His LFTs remain normal.

We thought he was doing so well. Is this comparable with any other kids? Does p/h stabilise ever?

[McSousa]

My boy has PH and spleen was that size at 3 (he's 5 now). His LFTs are always way off, (it's as though someone's randomly added an extra digit sometimes!) I think some cases of PH do stabalise if the blood flow isn't too bad and scarring in the liver is in the "right" places but that's not our experience.

There are loads of factors involved in doing well- growth, milestones, intellect, quality of life, happiness etc so perhaps he is still doing well mainly, if you see what I mean.

[LisaLockley]

Thank you. Yes, in the main he's doing well, but I was completely blindsided by this. Boo! McSousa, is your little man on list because of LFTs or PH? I am terribly scared about bleeds. I'm reeling now, but will breathe, take stock and carry on! Xxx

[Nelly123]

Hi - my son is 12 - has p/h and has had since a baby... Not sure what you call stable - he is a strapping boy - although spleen has steadily grown - is now 20cm - he exhibits no major side effects. Height/weight fantastic - bloods good- on appointments only every two years - no bleeds. He has had two endoscopes showing one small varicella of the lowest grade which has not changed - some how his body is just getting on with it! I was like you and worried endlessly at first but you will relax as time goes on. The hospitals need to make you aware of the potential risks but they are not always an everyday reality. If you are still concerned you could speak to your consultant about a future endoscopy but it will involve a general so they will need to agree its necessary. I think it's a lot about not knowing what's going on in the body and being able to control it that worried me do I was always reassured after the endoscopy! We only had the two endoscopes due to cautious registrars at clinic when they found out we were travelling to far flung places - my son is probably having another soon as we went to clinic and I said we were thinking of going to Sri Lanka over the summer! We have travelled many places with ourson with P/h and not had an issue - Asia, South America, USA, Europe so it does not have to be the end of normal life... No need to wrap I cotton wool- just assess your risks and plan ahead for if anything goes wrong! Good much... Hx

[Skibud in reply to Nelly123]

Hi, so lovely to read what you've written, as its gives us hope & reminds us that we can still do normal things our son is 14 months old, his condition is still undiagnosed, they think he'll need a transplant at some point in the future, but no idea when. He's doing gr8, piled on the weight, full of energy, normal lft's, would never guess from looking at him that anything wrong. Hes got enlarged spleen & ph, but not caused him any probs to date. We are at Leeds just now waiting for him to come out from his latest MRI scan, which they're doing every 3 months under a general, to check his cells haven't turned pre-cancerous (which would accelerate transplant) We take 1 day at a time, living it to the full but now we've been cleared to take him out of uk, are keen to take him on holiday. We were going to do Eurostar, to be on safe side, but now you've given me confidence that if results ok we can also fly & you've re-assured us that we can go further afield in future if dr ok it, so thank u 4 sharing x

[LisaLockley]

Thank you, that has made me a lot calmer! We are with BCH, he had an endoscopy last year and will have another next year. We are booking our first holiday abroad this month... Life is for living, right? X

[Skibud]

Hi, see my comment that I've put above. Yes definitely life is totally for living thanks 4 asking question that we've just been asking ourselves. Think we just need to do a day at a time & do as many fun things with them that we can holidays here we come xx

[Eleanorb]

Hi, My son is 3 and has portal hypertension as he has no portal vein. He has had 3 bleeds (as a baby, none since then thankfully and touch wood) and between 12 & 15 endoscopies (can't remember exact number!) to treat his varices. He is generally well but has numerous issues bubbling under; enlarged spleen, chronic diarrhoea (for which he is prescribed Imodium on a daily basis), low appetite etc. He also appears to have dilated bile duct(s) but LFTs otherwise normal. We have been advised not to fly even though his endoscopies are now 6 - 12 months apart (they were every few days during his bleeding / crisis period). Do get in touch to share more information. All the best to you all.

[LisaLockley]

Thank you Eleanorb... I spoke to his liver team at BCH yesterday morning and they explained that despite his large spleen and dampened blood flow, his liver is compensating well, his albumin and LFTs look good as his nutrition. She did explain that he is at greater risk of a bleed but at his endoscopy last year they found one tiny varices and had no reason, going on ultrasound to think this had changed. She did say if he had a bleed and LFTs are still normal they can divert the blood flow- surgically I assume. Has this been suggested to you, or have they had to do this as your little boy has no portal vein?

I think I just felt that it was a massive kick in the guts this weekend, and I felt exactly the same as I did at diagnosis!

X

[Sunshine74]

Hi there

I love to hear the 'strapping and fine' experiences! My boy is 19 months and has increasing ph. I was told by our transplant team recently he is doing really well which never quite computes. I was also told by another mum to stop waiting for the storm to pass and learn to dance in the rain. I have my wellies on as we speak!!

Kelly x

[Eleanorb]

We have been given reassurance when Evan has only had level one varices. Probably because he started with level three, and many many of them. That's when he was having endoscopies every couple of weeks, but they couldn't operate any more frequently as they risked blocking up his oesophagus with scar tissue. It was a very scary time and he was still a baby (he also had open heart surgery to repair a complex congenital heart defect at four days old).

Evan is a King's patient, they have not seen absence of portal vein as a cause of ph before (quite disconcerting) but we too are trying to learn to dance in the rain! Surgery has pretty much been ruled out for Evan as presumably there's nothing to 'stent' if there's no vein there in the first place. They ruled it out early doors but we have never had a long enough conversation with his team about the rationale for this decision.

What is the cause of your child's ph? Does he have another liver diagnosis? I'm not sure about stabilising, my understanding is risk of bleeds, enlarged spleen, distended abdomen, lack of contact sports etc is life long. I find this very difficult.

[LisaLockley]

My little boy has liver disease (biliary atresia) and its the cirrhotic liver causing the PH, despite liver's synthetic function being good. Ultimately he will need a transplant and we are assured the PH will disappear then. We will look into a spleen guard when the time comes. I know one boy with PH who plays rugby... With a spleen guard. He was adamant he wanted to play and that was the compromise. I agree it is incredibly difficult not to look ahead and feel sad xxx

[Eleanorb]

Oh I see, our sons' ph have v different causes don't they? The need for a transplant must feel like it's hanging over you, as if you haven't been through enough already.

We are a football mad family and it saddens me to think he might not be 'allowed' to play, but maybe the spleen guard is the answer. I guess taekwando might be out though

Trousers falling down at wil (due to distended abdomen) is the problem du jour - very minor but very tedious! Sound familiar?

Good luck to you xx

[LisaLockley]

Oh yes... Continuous builders bum here! I am part of a large liver community on line and the older kids who have had PH for years seem to grow into their tummies. Things adjust and shift internally,

In The US they take spleens out or reduce them... BCH don't do that for liver disease, but I know it's an option.

Much love x

[Eleanorb]

That's really good to know (about growing into their tummies) But I'm not sure Evan will, we've not been given those reassurances at hospital. I think the size of Evan's is a problem, I don't think he does though. Spleen out? Yikes. Are your online liver links here or in USA? Have really struggled to find anyone with portal hypertension either linked to no portal vein (v rare) or not linked to liver disease. On the off chance that you know of anyone please let me know. Hope you enjoyed the picnic yesterday. Thanks Lisa x

[Kerry06 in reply to Eleanorb]

Hello, I have a 6 year old son with Portal Hypertension not caused by liver disease he has a portal cavernoma which was a portal vein clot but now is a mass of collateral veins that his body has grown to bypass the blockage. A cause for the original clot is not known. He is treated at Kings childrens liver clinic and stays on rays of sunshine ward when in for endoscopies. There is a facebook group called portal vein thrombosis & Portal Hypertension. I cant say Ive heard of anyone before your child without a portal vein. But there is people in the group from all over the world sharing experience with the symptoms which I think are similar. There is also a a great web page that an American guy set up with so much information about portal Hypertension his name is Clint Steenson. If you type his name and portal Hypertension into google it comes up. Hope I have helped. Kerry

[paul71182 in reply to Kerry06]

Hi my 9mth old son has portal carenova also, the same as you have mentioned above. I am very worried about him as I have read all sorts of bad things online. How is your son doing now if you don't mind me asking/

Paul

[paul71182 in reply to Kerry06]

Hi Kerry

My 9mth old son was diagnosed with the same condition as you mention above it sounds like we have a long road ahead of us ,what are your thoughts on this condition.? any info I would appreciate it

Paul

[cookies897]

Aaaron has portal hpertension but his lft's are normal as his is extrahepatic ie outwith his liver, but aarons spleen is blocked as well they are looking into digeorge syndrome with aaron as its linked to my heart disorder, his spleen is 4 cm down from his ribcage