This blog takes its inspiration from a recent Instagram post by @thefightingirishmartins. This is a fantastic blog written by Michelle Martin, the mum of twins born at 27 weeks, who is passionate about raising awareness primarily about the syndrome that precipitated their early birth, and also post-IVH hydrocephalus.
Michelle’s Instagram post had a roaring response from parents because it hit on the nub of the issue when it comes to kids that have shunts to treat hydrocephalus – the impossibility of knowing whether the shunt is working or not.
I hope Michelle won’t mind if I requote most of the post here:
“Wouldn’t it be nice [she writes] if your shunt could tell you when it was failing, about to fail, or when you should get it checked by neurosurgery… BFORE launching a person into an emergency health crisis requiring urgent brain surgery as the only course of action?
We have self-parking cars. We have Bluetooth speakers. We have powerful computers inside the phones in our pockets. We have video calling around the world. We have space missions and robot guided surgeries and implantable glucose monitors and pacemakers and so much more.
Yet the shunt, the main treatment for hydrocephalus, has not changed much from its original design in the 1950s.(…) Sometimes the only way to see if a shunt is working is to drill a burrhole in the skull and go in with a scalpel and take a little looksie.
In 2021, when my $100 Fitbit can tell me what my heart rate is 24/7, the only way to check if a critically-important, extremely serious medical device implanted in the brain is working is to look at it with EYEBALLS. In the OR.
It’s time to make a better shunt. If I can buy a “smart” thermostat, alarm system, phone, speaker, and Bluetooth lightswitches, it’s time to make a smart shunt.”
@steffiecherry responds:
“These are possibly the best posts I’ve seen on the shit-show of shunts (clap icon). Honestly – of all the things I worry about with my kid, the shunt second guessing is the heaviest. “
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THE CONUNDRUM
If you have read our story on the SuperLily website, you will know that Lily has post-IVH hydrocephalus and had a VP shunt surgically inserted when she was 40 weeks old. The potential risks associated with her shunt lurk in the back of our psyche ALL THE TIME. Even though we don’t actively think about it on a day to day basis, when Lily is unwell in any way, the second-guessery kicks in, and Sean and I become completely paranoid.
The signs for shunt malfunction, we are told, include lethargy, headaches, loss of appetite, vomiting, irritability, fever, swelling or redness along the shunt tract, enlargement of the head. How vague is that? Apart from the last two, any of the others could easily be due to teething, to a tummy bug, to the flu. How on earth are we to tell whether it’s because her shunt isn’t working?
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A NEED FOR AWARENESS AND SOLIDARITY
Michelle Martin is right to raise this issue. According to a study[i] published in 2018, there are 400,000 new cases of hydrocephalus globally every year. The greatest burden of the condition occurs in low and middle income countries especially in Africa, Latin America and Southeast Asia, accounting for 75% of all new cases.
In the USA[ii] one out of 770 births result in hydrocephalus (making it as common as Down’s syndrome) and about 1 million Americans live with the condition.
Hydrocephalus can affect anyone but is most common in infants and older adults. It can be congenital or acquired. The causes and types are quite complex and not the area of focus here, but if untreated, it can be damaging or even fatal.
On the other hand, if treated early enough, and managed carefully, often children with hydrocephalus can lead normal lives.
There is, however, no cure, and as this blog post will show, living with hydrocephalus comes with many unknowns.
This blog is partly to raise awareness, but partly also for parents especially of premature babies confronting the possibility of hydrocephalus for the first time. It can be terrifying, and I think the honest truth is that as they get older it never becomes less terrifying, but the good news is you are not alone. There are amazing people out there – like Michelle Martin – who share their stories and from whom we can all find some solace and camaraderie.
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QUICK MEDICAL SUMMARY – HYDROCEPHALUS DUE TO HAEMORRHAGE IN PREMATURE INFANTS
What is it?
Simply put, hydrocephalus is the build-up of fluid in the brain – (hydro from the Greek for water, and cephalie – brain). The brain and central nervous system make cerebrospinal fluid (CSF) every day, which circulates around the brain and spinal cord. Its job is to bring in nutrients and carry away impurities. In the brain it flows through open areas called ventricles and then is absorbed into the bloodstream. This absorption is important to keep pressure in the brain at a normal level.
When the ability to absorb CSF is disrupted (either by blockage or poor absorption) then CSF will accumulate in the ventricles and increase pressure in the brain. This pressure can ultimately cause brain damage, and can be fatal if left untreated.
What causes it?
In premature babies, it is most often caused by intraventricular haemorrhage[iii] (IVH), or infections such as meningitis. IVH is bleeding into the brain ventricles that happens in about 50% of extremely premature babies due to the weakness of their blood vessels next to the ventricles. Changes in blood pressure in a difficult birth or ventilation can cause these blood vessels to break and blood to flow into one or more ventricles of the brain.
IVH is graded according to its severity from I – IV. Grade III and grade IV bleeding causes a swelling or obstruction in the flow of CSF in and out of the ventricles, which can result in hydrocephalus.
How is it Diagnosed and Treated
IVH is usually on the radar for NICU staff especially if the birth was difficult or the baby born extremely early. Sometimes it is even picked up on scans before the baby is born. It can be detected by swelling of the head or fontanelles, or by ultrasound or CT scans. Low grade IVH can resolve itself over time, but if more severe, it can lead to hydrocephalus.
The most commonly used treatment approach for hydrocephalus is the surgical insertion of a small tube called a ventriculoperitoneal shunt (VP shunt) into the ventricles to drain off the CSF. If you look at the diagram below, you will see that the shunt has two small thin tubes or catheters connected by a valve. One tube is placed in a ventricle, and the other is in the peritoneal cavity which is where the stomach and the bowels are. The valve opens when the pressure in the brain gets too high and fluid drains from the ventricle into the stomach, where it is absorbed into the bloodstream, filtered out into the kidneys and exits the body through urine.
The shunt is inside the body under the skin.
www.childrensmn.org/educationmaterials/parents/article/18525/vp-shunts
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PARENTING A CHILD WITH HYDROCEPHALUS
Getting used to the idea of a drain in your baby’s brain
When Lily was born she had to be resuscitated, her heart didn’t beat for over 20 minutes. Her blood pressure needed boosting and she was ventilated to help her breath. On her first night an EEG showed that she was experiencing seizures, which were controlled medically. The NICU paediatrician consulted with a neurologist and an MRI was done, showing IVH grade IV bleeding and dilated ventricles. Further ultrasounds confirmed the IVH on both sides.
A few weeks later the doctors began to have concerns about hydrocephalus. The problem was that Lily was still too little to have the brain surgery needed to insert a shunt – the neurosurgeon was concerned that her skin wouldn’t tolerate it and that there was a very high risk of infection (I believe as a rule VP shunt insertions for babies less than 2kg have poor outcomes). A couple of lumbar punctures were done to try to relieve some of the pressure in the interim, but didn’t have an impact.
We were horrified by the thought of a shunt – of what Lily would look like with this thing sticking out of the side of her head, of the risks associated with the surgery. We reviewed the situation almost weekly with the neonatal paediatricians and neurosurgeons, and when she was nearly full term, it was agreed that the timing was right – she weighed nearly 3kg and had progressed well clinically on other fronts, so she was in a stronger position to withstand the surgery.
The thought of sending our baby into the operating theatre under general anaesthetic for frankly, dangerous brain surgery, was mind-numbing. We already knew she’d sustained brain injuries at birth – what if anything went wrong?!
But through conversations with our medical team, consultations with outside specialists, and the calm encouragement of a friend in South Africa whose little girl had gone through something similar, we had become convinced over the weeks prior to the operation that a shunt would maximise Lily’s potential for further brain development in the future.
Before the operation, we consulted with an ex-head of the NICU at the leading teaching hospital in Hong Kong, who confirmed that the neurosurgery team were very experienced. This gave us a lot of confidence – I would always recommend a second opinion if you feel you need one.
The truth is that the shunt does a very important job and once it was in, we got used to the idea pretty quickly. Lily was lucky to be in a hospital with access to an excellent neurosurgery team and that the operation went off without complications, or further infection in the days or months after.
Here’s our little enchanted sprite with her magical horn – shortly after the surgery and now hidden by her curls. Lily’s shunt is on the right side of her head, positioned just above her ear, and the drain runs behind her right ear over her shoulder and down into her tummy. I have since discovered that it is more common these days for shunts to be run down the back of your head.
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Shunt Paranoia
The thing with shunts is that they are always top of mind as a parent. Lily’s shunt is really the most stressful thing in our lives. Every time she gets sick and has a fever, we are in a panic as to whether it’s because the shunt has a problem.
To outsiders it would be easy to think that we get way too stressed about how to manage a fever… but the truth is that a blocked shunt can be fatal, so effectively we can never be too careful.
Similar to taking care of a preemie, taking care of a child with a shunt is simply not the same as taking care of a typical child. Parenting a child with a shunt comes with a completely different level of paranoia.
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The Shunt Guessing Game
NO WAY TO TELL IF IT ISN’T WORKING!
Referring back to the @thefightingirishmartins – other than the symptoms mentioned above, there is no possible external way to tell if a shunt is malfunctioning other than invasive surgery to open it up and have a look. This “not knowing” is a universal concern amongst parents of children with shunts.
PROGRAMMABLE VALVE
The only thing that can be done externally with Lily’s shunt is to measure and adjust the rate of pressure/flow of the valve using a magnetic adjuster that is held over the site of the valve and moves the setting magnetically. Even then it is not clear without an MRI to know whether the valve may be over or under-draining. In our experience re-setting the valve has only been done after each of Lily’s MRIs, because MRIs have very strong magnetic fields which can alter the setting.
STAY AWAY FROM MAGNETS!
We have been advised that Bluetooth, computers, security scanners, metal detectors, microwave ovens, mobile phones etc. do not have a strong enough magnetic field to have an effect – but one neurosurgeon we spoke to did mention magnetic toys as a possible risk, so we had to exchange the doll Lily’s granny sent her for Christmas because it had a magnetic dummy. Was that taking it too far? Hard to tell really, but you wouldn’t want to risk it, would you!
DON’T KNOW HOW LONG IT WILL LAST!
“It is difficult to predict how long shunts will last, but some practitioners note that about half of all shunts need to be revised or replaced after 6 years”[iv].
We have been told variously that we will not know if Lily’s shunt needs replacing or is no longer necessary until one day perhaps there is a reason for it to be removed or replaced surgically. We know that often shunts need revision – some children have to have multiple surgeries to correct shunts, and usually a shunt will need to be replaced or removed at least once in 6-10 years. But for the most part it is “wait and see”.
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ADVANCES IN SHUNT TECHNOLOGY
A 2013 article[v] described the three key problems with shunts, their high failure rates, difficulty in detecting failure and a very limited ability to control them externally.
Despite some improvements to shunt technology (e.g. the materials used to make them), nothing has yet been brought to production that addresses these specific problems. The truth is that dynamics inside the brain are very complex.
From my own desktop research, it seems that a few interesting projects are investigating different ways to develop a “smart” or “intelligent” shunt, i.e. one that adapts how the shunt behaves to the day to day needs of the patient. One even appears to have received FDA approval in the USA and is expected to be commercially available in about a year from now…
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PARENTS AROUND THE WORLD UNITE!
To be quite honest with you, whilst it is always lurking in our consciousness, in day to day life we ignore Lily’s shunt and cross all our fingers and all our toes and hope like hell that all will continue to be well.
If the technology really is slowly evolving, it is very powerful to align with other parents around the world in advocacy and if nothing else in moral support – something that is so easy to do in this social/digital day and age.
So please share this blog, follow Michelle Martin, follow SuperLily and let’s continue to raise awareness on this critical topic.
For further reading see:
https://interfacegroup.ch/project/smartshunt-the-hydrocephalus-project/
https://royalsocietypublishing.org/doi/10.1098/rsif.2019.0884
https://www.hydroassoc.org/a-look-at-new-shunt-technology/
https://www.hydroassoc.org/technologyupdate-moving-towards-a-self-sensing-shunt/
https://www.sciencedaily.com/releases/2018/10/181031141550.htm
[i] Dewan, MC, Rattani, A, Mekary, R and Glancz, LJ. (2018). Global Hydrocephalus epidemiology and incidence: Systematic review and metanalysis. Journal of Neurosurgery. 130(2):1-115.
[ii] https://www.hydroassoc.org/about-us/newsroom/facts-and-stats-2/
[iii] See (https://www.hopkinsmedicine.org/health/conditions-and-diseases/hydrocephalus/hydrocephalus-in-children) for other causes of hydrocephalus in children.
[iv] (https://www.hopkinsmedicine.org/neurology_neurosurgery/centers_clinics/cerebral-fluid/procedures/shunts.html)
[v] Lutz, BR, Venkataraman, P and Browd, SR. (2013). New and Improved ways to treat hydrocephalus: Pursuit of a smart shunt. Surgical Neurology International, 4(Suppl 1):S38-S50. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3642745/
Robyn, this is such an informative and well-researched article!! Amazing job putting this together. It really would be great if shunts could tell us they’re the problem, right, instead of “oh it is just a cold after all.” I don’t think parents of kids without hydrocephalus realize that we really do need to go to the ER for almost “every little thing.” I’m so glad you liked my instagram post and thank you for sharing about it here. 🙂
Michelle, thanks so much for your comment. It’s very encouraging to share experiences with and learn from you and other parents around the world. It does seem as though the technology for shunts may one day get there… Let’s hope so!!