What are Posterior Urethral Valves?

What are Posterior Urethral Valves? :

Over the past year I’ve wanted to start a blog to provide a resource of practicalities of parenthood and caring for a child with a hidden health condition. It’s based on my experience of my son’s care within the renal and urology healthcare world.  

I’ve been thinking about the best place to start and decided to start at the beginning. Posterior Urethral Valves, the first diagnosis my son received, our experience, the impact and what it means. This I hope will set the tone for the future blog posts and also give some hope to the reader. In future posts I’ll discuss Chronic Kidney Disease and Transplant, but for now I’ll start with PUV.

For reference, I’ve included links to useful websites throughout the post, and at the bottom. The information I’ve written here is taken from InfoKid, Evalina and the GOSH websites, they are informed through the work of the NHS.

Our experience:

I was told throughout my pregnancy that I was low risk and everything was normal. This all changed when my son was born, he was born needing immediate help. Within the first few days of his life it was found that his kidney’s and bladder were damaged. It was at this point that we were first told about Posterior Urethral Valves (PUV).  In short, his valves had completely blocked the bladder’s ability to drain and his bladder and kidneys inflated; like a water balloon.  His bladder is about 3 times the size it should be. His kidneys were functioning at around 10%, for the first couple of years of his life he had limited kidney function and around 2.5 years old he had a kidney transplant. The PUV were removed shortly after birth, and since his health has been managed with diet, nutrition, catheterisation and some surgeries.

The initial diagnosis was overwhelming and led me to go down various routes in Googling. For my son PUV caused damage to his kidneys. As a family we have grown used to the uncertainty around managing kidney health, we take each day as it comes and celebrate the wins when we can. It hasn’t stopped my son turning into such a wonderful little boy. He is kind, empathetic, loves to learn and play. He runs around at lightning speed and doesn’t let anything stop him, I will always be impressed by his ability to adapt. I’m biased writing this as his Mum, but seeing him and other children who manage their health as part of their everyday day is inspiring.

In future I’ll blog in more detail about CKD and kidney health, including the practicalities of the renal diet and a baby. Briefly my son had specific formula from a few days old and as he grew and we moved into weaning, we had to cook food in a specific way. Fluid targets and nutrition consume my thoughts, getting food into my son was difficult and now post-transplant it is much better- I honestly don’t think I’ll ever tire of seeing him eat knowing how difficult it’s been. If you’re going through this at the moment, we had some superb advice about managing my son’s renal diet from newborn to toddlerdom from an amazing renal dietician, I’d recommend asking your clinical teams for contact with them. There is also an amazing resource on Instagram called Kidnykids who have some wonderful recipe ideas and Kidney Care UK also have recipe ideas too.

Don’t get me wrong it’s not easy. Coming to terms with everything to do with a diagnosis, managing life as a new parent or with a young baby, explaining things to others, and then managing everything else on top is overwhelming. Some days you’re on top of it some days may be tougher than others. Just know, you’ve got this. You’re doing better than you think. Be kind to yourself and if you feel overwhelmed then please do speak to someone like your GP or someone you trust, its ok to ask for help for you. There are some amazing organisations who can help support you as you adapt to life caring for a child with a long term health condition Contact have some amazing resources.

I think as well it really is important to remember that you and your needs matter, so much is happening and there is so much to process its ok to do things for you and to ask for space when you need it. I always have emergency chocolate and a nice tea bag in my son’s change bag for me, just in case he is admitted to hospital. A friend mentioned they were in limbo and no-one looked out for the family. Hopefully this blog offers a little comfort and to make you feel less isolated,

What are Posterior Urethral Valves (PUV)?

Posterior Urethral Valves (PUV) are tiny flaps/valves which sometimes grow at the back of the a babies’ urethra during pregnancy. They occur only in males and roughly 1 in 8,000 are born with them (GOSH, ONLINE 21).

The urethra is a tube connected to the bladder, it is the tube which urine flows through as we wee. The tiny valves, can block the bladder and cause the urine to build up inside the bladder. How blocked the ureter is can be different between children as can the damage caused. The specialists who will look at the bladder, remove the valves and who may suggest different treatments to make the bladder safe are called Urologists.

Symptoms:

Antenatally some of the symptoms may include reduced amniotic fluid, they may be suspected on scans but not always. The Great Ormond Street Hospital has a really useful page with more details for babies, toddlers and older children.

In short, if you suspect there are problems with how your child is weeing, if they have pain or have problems with continence after potty training, frequent UTI’s speak to the GP.

For my son he was diagnosed at around 2 days old. He’d had a blood test as his creatinine level (marker of kidney function) was incredibly high for his age. He was rushed to ultrasound and had a scan of his kidneys and bladder. The whole system had been blocked and he’d sustained substantial damage. We were taken to a side room and told what was happening.

One of the first questions I had was, what did I do wrong? Honestly, I still ask myself this frequently. However, there is no known cause to PUV. Crucially they aren’t due to something that anyone has done wrong.  

What happens next?

How PUV affects each child is different and so what happens next will be determined by yourself and the clinical teams involved in your child’s care.

For some children PUV can be identified antenatally, or like in our case, immediately after birth. They can also be identified later in childhood or in the teenage years. PUV can block or partially block the urethra. The extent of the blocking is different in each child. sometimes block to an extent that the urine backs up into kidneys, which may cause kidney damage. If this is the case there will be a specialists Renal team involved in your child’s care, clinicians involved in kidney care are called nephrologists.

For my son, PUV, caused damage to his bladder and kidneys, for the first couple of years of his life his kidney function was very reduced, and after about 6 months we knew that a kidney transplant would happen in childhood. The valves were removed during his first 8 weeks of life, but his bladder continued to reflux into his kidneys. To stop the kidneys being further damaged, one of the tubes connecting his kidneys and bladder was made into a ureterostomy, a small opening on the surface of the skin that drained urine. This acted as a pressure release valve and over time has helped to stop the urine refluxing into the kidneys.

Where does treatment happen?

I can only speak from a UK perspective, but there are some incredible Paediatric Renal and Urology units and teams throughout the country. I will always be grateful for the opportunity they have given my son to live and thrive.  

Depending on how and where things happen, you may find yourself on a Neonatal Intensive Care Unit (NICU) with your child. The charity Bliss are incredible and here is a guide to what to expect in NICU.

Your child, when old enough, may then be transferred to a Specialist Paediatric Ward. The jump between NICU and then to a Paediatric Ward I found to be a shock. On a Paediatric Ward you are far more involved with your child’s care and you will stay on the ward with your child, which you’re not able to do in NICU. I’m not sure that I was prepared for this, I didn’t even know I was allowed to put clothes on my child do anything. It was a very strange time. It’s also a difficult time as you may be recovering from birth yourself. I found it useful to have a note book at this time and just even write down the odd thing or doodle, just to get things off my chest.

After the initial input your child may be monitored or have different treatments through the specialist centre but you will also have other input from the healthcare service. Your GP is an important point of contact, as alongside all of the specialist input there will be things like vaccinations etc that your GP surgery will arrange. Overtime you may become familiar with your local hospital, where they can provide emergency support. It is useful to ask if its possible to have your child under the care of a local Paediatrician so that as your child grows they can support your child to access the care they need locally. On this, in some cases for local hospitals Open Access can be granted if your child frequently needs hospital support. This means that your child is on the system and you can call to let your local A and E know if you’re headed in/ or need advice regarding.

Alongside this, for younger children there is the health visiting service who will do developmental checks etc. When you see them, it is useful to ask about the Perinatal Mental Health Service, who are specialist health visitors who support families to look after their health and wellbeing with the diagnosis. Looking after yourself can feel almost impossible, but it is really important. This team speaking with your GP, friends, family anyone you trust to help you look after yourself can be helpful. Some of the specialist centres also have specialist support for families who have children with complicated diagnoses. Locally too, check out if in your local area there is a Carers Organisation, as they can help signpost you to support for health and wellbeing, finances, practical resources etc, there may also be support groups to help you meet others in a similar position.  

From my experience and speaking with others there are lots of differences between the treatments children need. Specifically for us my son’s initial kidney failure was managed through diet and nutrition, for some the number of medications is overwhelming. Combining this with specialist milk formula’s, fluid targets and everything else, you become organised very quickly. Some children may need to have the support of a Nasal Gastric tube which allows food/fluid to go straight into the tummy. If it helps, there is a great programme called Get Well Soon Hospital by Dr Ranj there is a programme all about NG tubes for kids which may be of help too https://www.bbc.co.uk/iplayer/episode/b06pyw26/get-well-soon-hospital-series-1-2-ng-tube.

A future blog post will be about routines and fitting things in to hopefully support you. The best bit of advice I can give though is to write things down in a way that makes sense to you. I have a weekly diary I write everything in and take it with us to clinic appointments (how much fluid he’s had to drink, food, meds, timings etc).

Where to find out more information?

There are so many different avenues you can go down when looking for information on your child. I’ve found that some of them are more unhelpful than helpful, so I try and stick to the NHS and reputable sources to not scare myself. In addition, there are some brilliant Facebook groups which are parent/carer led and they offer amazing first hand experience and support if that’s something you would like. Some organisations to look at that I have found useful are ERIC the bladder and bowel charity, Kidney Care UK and Kidney Research UK.

https://www.bliss.org.uk/parents/in-hospital/about-neonatal-care

https://www.infokid.org.uk/posterior-urethral-valves-puv

https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/posterior-urethral-valves-puv/

https://contact.org.uk/ .

https://www.evelinalondon.nhs.uk/our-services/hospital/kidney-services/condition-treatments.aspx

https://www.carersuk.org/

It is also worth mentioning, and there will be a future post on this. The impact of being in and out of hospital can be demanding on your finances. Speak with the specialist teams about the social workers in the teams to see if they can help you out to apply for benefits or funding to support your child. In some hospitals you can claim back travel and congestion charges. If you’re in the UK Disability Living Allowance links to other support so it is worth considering applying for this, social workers involved in your child’s care may be able to support.

There were, have and continue to be multiple questions I have, and I don’t think we’re the only family to think them. What will my child’s life look like, will they grow, will they be potty trained, how can I encourage my child to drink that much fluid, what do I pack for a hospital visit, what do I need in an emergency?

I’m going to end this blog post here, and use it as a guide to start the next post. It would be great to have your feedback, and if there is something specific and practical please do get in touch. Thank you for reading and I’ll have another post soon.

Lucy x

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