Is there anyone else who has portal vein thrombosis and portal hypertension as a primary diagnosis? No other liver concerns and no cirrhosis of the liver.
My son was diagnosed a year ago. He has regular banding. Enlarged spleen, the low platelets, no ascites. Although currently vitamin A deficient and on supplements.
Could those of you who have PVT / PH or have children with the disease, share with me their experiences. Good or bad, I’m well aware of some of the horrendous complications (we’ve fortunately had no bleeds) so you won’t scare me 🙂
Thank you 🙏🏼
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EHPH
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Yes, my son is 10 and was diagnosed with portal hypertension at 9 months old. He had open heart surgery at 4 days old and the PH came as a spectacular bolt (bleed) out of the blue. My son has portal vein agenesis which means he doesn’t have a portal vein, not a blocked portal vein like other patients. It’s extremely rare. With his congenital heart defect, he clearly developed vascular anomalies in utero. He has had 3 bleeds all in first three months of diagnosis when he was a baby - his varices were many and to quote his Consultant, ‘spectacular’. We have had 9 years of endoscopies and things are currently stable. Ask me anything you like and all the best to you.
Hiya, thank you for replying. My son's 7, and the cause of his condition is unknown. Which is infuriating lol.
At 16 months our GP referred him to hospital because he thought his spleen felt enlarged. Hospital thought nothing of it so discharged him. In 2018 by chance and a nurse very trusting of my instincts she ran some bloods. It took 10 months just to get a diagnosis.
My sons had 3 endoscopies so far and had bands applied at each one. Grade 2 and at the last one grade 3.
He’s otherwise well, and you’d never know he has to live with this condition. I’m so very thankful for no bleeds, as I’m aware this is how many become to receive this diagnosis.
We’re still investigating whether he’ll be a candidate for the REX shunt.
My main concern and worry for him is if he can’t have surgery, that he’ll have to have endoscopy treatments for life, and only ever be symptom management.
How is your son? Does he cope well with treatment? Do they still put him under anaesthetic?
A Rex shunt has been ruled out for Evan because there is an absence of vein rather than a blocked vein. If you think about it, the shunt becomes unviable. I know Rex shunts are not gone into lightly and I was told about 9 years ago that they are not always successful. Ask lots of questions about that. I wonder if they will see how Roman copes with endoscopes first?
Very difficult route to diagnosis for you. Preferable to a bleed tho.
A truly awful thing to happen. Well done for being persistent.
I can understand how upset you must feel about ‘endoscopies for life’ but hopefully slowly they will get further and further apart. Evan was having them every few weeks when he was a baby and we are now one year apart. I would like to say we forget all about them between treatments now but that’s not always the case because he has and has had a lot of other stuff going on (heart surgery, Gastro problems, glue ear...). However his I’ll health has become a way of life. It’s not easy dealing with a hidden and rare illness that no one understands or has ever heard of, except maybe for raging alcoholic old men ☹️. If it’s helpful, Evan deals with anaesthetics very well both physically and emotionally and is positive about going into hospital. He’s had over 30, 40? GA’s in his life so it’s no big deal at all for him and I have to say and I’m quite sorry to say, pretty routine for us.
Where is Roman treated? We are King’s College Hospital, London.
It’s great that your son copes well, Romans abit of a nightmare when he comes round lol but in a hilarious ‘oh gosh, here’s grumpy’ kinda way.
I know how you mean, it’s a new normal way of life. There’s been people who’ve asked me how I cope with it but I remind them my son is otherwise healthy and we are very blessed with that. There’s always someone worse off than ourselves.
I know there’s always a chance of bleeding, and Roman checks his poo and let’s me know it’s normal because there’s a risk of lower Gastro bleeds too.
The waiting list is so long for the Rex (there’s only 3 surgeons in the U.K. who can perform it ) but if successful there’s hope his spleen size would decrease, his platelets would increase and he’d only return yearly appointments to check the shunt is still open. If he’s not a candidate or the Rex fails then he’d go back to regular scopes.
I agree It’s a scary thought when your child’s endoscopy becomes so routine, it’s normal. even after 3 Romans so excited for the 2 days off school and all the extra ice-cream he’s allowed to eat.
We’re under QMC in Nottingham but Roman also has a liver specialist consultant at Leeds hospital that’s holds clinics at QMC. Again I feel so fortunate that we have a great team working for us and with us.
Our boys really are great aren’t they? Take it as it comes and don’t let their rare diseases get the better of them! It must mean we’re pretty incredible mums too!! 😉🤣🤣
That’s so nice of you thank you, sometimes talking to people who have kids with illnesses eases the anxiety, I’m lucky I have great support but sometimes the ‘he’ll be fine’ attitude isn’t what I always need.
I like to dissect it all lol
Yes from Nottingham!! So Queens is right in our doorstep!
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