He was not expected to survive past birth - but now 14-month-old Barney must live with the fact he will develop kidney failure at 'some stage in his life'

  • Just 1 in 6,500 baby boys develop the rare PUV disorder – a problem which often leads to chronic kidney failure
  • Baby Barney wasn't expected to 'last more than an hour', but continued to fight
  • He must live with the fact he will definitely suffer kidney failure during his life
  • But charity is helping to support the family, both financially and emotionally

Written by Samantha Lade for DonateToday

PUBLISHED: 14:10, 14th Sep 2017 | UPDATED: 16:42, 27th Oct 2017

Fighting from Birth

All that any expectant parent wishes for is to have a straight-forward and healthy pregnancy. However it was just over the 7 month mark when the news was broken to Sophie Hughes that her baby boy was already battling a severe condition affecting his lungs, kidneys and bladder.

Doctors said one of Barney's kidneys was 'as good as 'dead'' when he was born

'Barney’s issues were first picked up at week 34 of pregnancy,' explains Sophie, speaking to Kidney Wales online. 

'I had a scan due to high blood pressure and they spotted some potential cysts on the kidneys. We were later told it was likely PUV, as I had no water left and it was all sitting in Barney’s urinary system.'

PUV (Posterior Urethral Valves) is a condition found specifically in baby boys where extra flaps block the urethra – the tube that carries urine out of the body. Usually diagnosed after birth, PUV often causes severe problems in the kidneys or bladder during the boy's lifetime.

The condition is relatively rare – roughly 1 in every 6,500 newborn boys will be born with PUV – but the disease remains relentless, and potentially life-threatening to those who have it.

'Doctors said one of his kidneys was as good as ‘dead’ while the other quite seriously damaged – and his urethra was badly dilated,' explains Sophie. 

'We were told that Barney wouldn’t last more than an hour. However, we were told that if Barney were to survive, he would need a kidney transplant. I was induced at 38 weeks in Southampton hospital as the PICU in Cardiff was shut, and transferred to Cardiff when Barney was one week old and breathing on his own.'

"'We were told that Barney wouldn’t last more than an hour."

- Sophie, Barney's mother

Battling On

Now 14 months old, Barney and his parents are having to face life knowing that his chronic kidney disease brought on by PUV, means that at some point – whether next week or in twenty years time – Barney will develop kidney failure.

'He will need treatment through his whole life, and we’re prepared for the fact he may need more than one transplant,' says Sophie. 'It is also looking likely he will have to have his ‘dead’ kidney removed in the new year as it has caused a few serious UTI's lately.'

The condition brings along many side effects with it, that Sophie and her partner must be vigilant about spotting on a daily basis. Barney already has a vesicostomy (a surgical opening in the abdomen) to pass fluids, and becomes dehydrated very quickly due to his overactive bladder.

The infant's health issues do not end there, however.

Kidney Wales holds several fundraising events each year for children like Barney, including the Walk for Life, Children's Christmas Party and Cardiff 10k

'Barney struggles with weight gain due to his kidney issues which led to him being tube fed for the first few months of his life. We struggle with getting him to eat, and there are lots of tears.'

'UTIs are a daily hazard too. Any sign of illness means a trip to the doctor’s surgery or A&E to get a urine sample. The more UTIs he has, the faster his damaged yet working kidney will go into failure.'

"Without the Kidney Wales family we would be at a total loss."

- Sophie, Barney's mother

Looking to the Future

Although there is no cure for kidney disease and Barney will never get 'better', the family are learning to deal with hiss condition one day at a time. One helping hand that has been there through the entirety of Barney's journey is the Kidney Wales Foundation. For their help, Sophie is eternally grateful.

'Without the Kidney Wales family we would be at a total loss,' she states.

'The money they raise supports families in their darkest moments. It pays for play therapists who entertain Barney while he has his bloods checked. It provides support workers who have helped us claim money from the government to help us with Barney’s illness.'

Barney was also invited to Kidney Wales' Children's Christmas Party, where the children were 'able to feel normal for the afternoon and actually be children', in Sophie's words. The charity's work continues to make life just that little bit more pleasurable for these youngsters – like Barney – who have been dealt a rough start in life.

The future is unclear for Barney. He currently visits the Children's Kidney Centre for fortnightly check-ups – but this frequency is expected to increase as his condition becomes 'more unstable'. He also visits the hospital often for blood tests, scans, and further check ups with a surgeon.

Reflecting on the situation, Sophie shares that she's 'shocked at how little is known about kidney issues by people who aren’t directly affected.' However, events held by Kidney Wales 'highlight the issues of kidney disease' and continue to share advice and support to those families who desperately need it.

Please note that names may have been changed to protect the privacy of those involved. Images are used for illustrative purposes only and, unless specified, persons included in images are posed models, not the persons mentioned in the article.