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Joey’s Journey

On the 14th of August our gorgeous baby boy Joey, entered the world. We had our newest addition to our family and our hearts were just bursting with joy. Baby Joey became the little brother to Molly in 2021 and he became a big brother himself to Jordi in June 2023. Our family of 5 that we are beyond grateful for.

Unfortunately, Joey has had a traumatic two years of his short life, more than some of us will have in a lifetime. We nearly lost our precious Baby Joey in March when he developed invasive Group A Strep (iGAS), which left our baby on a ventilator in the ICU in CHI Crumlin for two weeks. All we could do was watch him fight for his life because due to his life threatening condition he faced many challenges to overcome along with the serious sepsis infection. One of his challenges was his tummy got so big they had to put in a drain and leave it there for 5 days. Joey spent a further 7 days in hospital being closely monitored before we finally all could return home to Molly who was staying with Granny and Gaga and who missed and asked about Joey everyday.

The January before this Joey was diagnosed with Portal Vein Thrombosis (PVT), which has led to a serious and rare condition Non-cirrhotic Portal Hypertension. Portal Hypertension has life threatening symptoms and the risk of death is 40% on a first episode of bleeding. The bleeding comes from varices that develop in his oesophagus so he will either bleed from his mouth or through his nappies.

This diagnosis came along after countless blood tests, numerous scans and investigations resulting from his first episode of sepsis. Yes! Our precious little boy has had two episodes of sepsis in his short life. The first when he was only 8 months old, he became gravely ill very quickly and we were told he had a Streptococcus Pneumo Sepsis Infection. He was quickly taken out of my arms and brought away for blood tests, x-rays and a lumbar puncture. Those moments are not easy, you would swap places with him in a heartbeat. The cries and the whimpering when he was handed back to me are moments I will never forget.

Joey is the funniest two year old boy with the biggest smile and the most contagious laugh that would brighten up the darkest day. He has been through so much in his short life and is the bravest little man who is loved so dearly by his big sister Molly and little brother Jordi.

From Joey’s first episode of sepsis in May 2022 to the most recent in March 2023, was the toughest for him. He was prone to infections and anything would wipe him out completely, he completely stopped developing and meeting his milestones and although he always had his amazing smile with the dimple on his right cheek he just looked miserable, uncomfortable and in pain most of the time.

As parents we are constantly living on the edge in fear of ‘is he going to cough up blood? Or is this the nappy that will have the blood in it? We hear him coughing in bed, we check straight away to see if there is blood on the sheets. Or is he having too much fun laughing and shouting, will this make him bleed?

Trying to keep him safe from infections, making decisions to go out as a family or go to play centres, going swimming, all these “normal” experiences we have to second guess and analyse the situation. Being on a daily antibiotic since the second sepsis episode has helped him greatly this year so far but obviously nowhere near ideal for him and his developing system.

Joey has a huge belly because of his enlarged spleen, which not only has caused medical issues but physically it exhausts him. He is a two year old boy and is only learning to stand now with lots of support. The weight and the size of his tummy has really hindered his mobility and it has been so hard to watch this as we can see all he wants to do is get going and run around with his sister Molly. As we have learned to live with this we notice signs of infection early such as his belly getting really hard and growing in size as he has an issue with retaining his urine, which makes him really uncomfortable and finds it difficult to sleep.

Our current protocol for Joey is if he gets a temperature we must attend A&E to rule out sepsis, we would do anything for our little man to keep him safe but this has and does take its toll on the whole family and most importantly Joey. As parents we feel like we are constantly putting out fires instead of quenching it and especially this time of year with every bug going it's difficult.

As he gets older it's harder and harder to see him not walking and playing like he should be. We know he wants to and he is becoming more and more frustrated in not having the ability to do these things.

With his liver not functioning like it should Joey has unfiltered blood moving around his system so as you could imagine it would not make you feel too good. As he gets older we are noticing behaviour and cognitive issues that develop neurologically from his metabolic system not functioning properly.

Joey loves his baby brother Jordi, his big sister Molly and our dog Merlot. Joey loves music and dancing. We have some of our happiest moments as family playing music and dancing around the kitchen and one of Joey’s favourite songs to dance to is ‘Oh My Johnny’ but he is not able to stand up and dance, he comes up to his knees and bops up and down or his daddy holds him in his arms. Joey loves monkeys and one of his favourite toys is his monkey teddy and his favourite book is ‘Monkey Puzzle’.

In August 2023 he began his management program for his condition, which is to go under general anaesthetic for an endoscopy procedure for banding of varices that develop in his oesophagus. This will happen 3-4 times a year for Joey with no improvement to other symptoms or his condition. This is the only treatment available for Joey in Ireland.

There is a Meso Rex Shunt/Bypass surgery that can be done for Joey. This surgery is available in Chicago and all in will cost €330,000, where we will have to stay anywhere between 8-12 weeks from pre-op to post-op. It is a 5 hour surgery that connects the superior mesenteric vein to the left portal vein using an internal jugular vein. This procedure bypasses the obstruction and restores normal portal flow into the liver. This can restore Joey’s system back to normal function. It would be both life saving and life changing and give him his life back.

The best outcome of the surgery is to operate on a system that has not had any bleeding yet i.e. a system that is not under too much pressure. And we are against the clock because the sooner we get this done for Joey we immediately eliminate the threat to his life of bleeding or another septic event. He won't be held back in his development and catch up where he has missed. His spleen will go back to a normal size, where his white blood cells and platelets will go back to normal levels, which his risk of serious infections will reduce and allow him to fight infection normally.

Without this surgery for Joey, there is a 40% risk of death in the first episode of bleeding.
Without this surgery he is at risk of a third sepsis episode and nobody knows how the third one would affect his body this time or if he would survive it.

If this surgery is completed in a healthy system (not bleeding) that Joey currently has now, there is a 90% chance of success and restoring Joey’s system and curing him of all other symptoms. Giving Joey this chance now in the next 6 months would change his life forever.

And as parents, we want Joey to go to preschool in September with his cousins, play football with his brother Jordi, dance in the kitchen with his sister Molly and simply live his best life that we can only hope and dream for if he gets this surgery.

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