“When are they going to get this thing figured out??” Over the past few weeks since my most recent health challenges, this is the number one question I’ve heard — from family, friends, online supporters, and people who follow my journey with hydrocephalus. And it’s a valid question, since I’ve had nine shunt related surgeries in just over three years.
And the answer, in my best no-nonsense, straight up, plain English - is that it’s complicated.
I’m not a doctor, or any type of medical professional. I’m simply someone who is doing their best to navigate life with this very frustrating health condition, and I am going to do my best to explain to you why it’s so complicated, and what makes it difficult for me, personally.
I believe that this may be the most important blog post I ever publish.
Please share this article with anyone you feel could benefit from this information, and share it online to help us raise awareness.
Thank you for taking the time to read and understand my story.
Hydrocephalus is defined as an abnormal accumulation of cerebral spinal fluid in the cavities of the brain called the ventricles. There are many reasons why an individual can either be born with or develop hydrocephalus. In my case, I have what’s called aquaductal stenosis - which is essentially a structural blockage of the passageway between two of those ventricles. This developed as I grew, for an unknown reason. Due to the blockage, the fluid is not able to properly flow through my brain, so it builds up, causing increased pressure and pain, and decreased function.
Cerebral spinal fluid (CSF) is continuously produced in the brain, is circulated throughout the brain and spinal column, and is continuously absorbed by the body. To most people, it seems like this whole situation should have an easy fix - because it’s just like plumbing, right?? Water in, water out? Well, not really. I’m going to do my best to explain. Again, this is all simply based on my understanding, the way it has been explained to me, and the research that we have done over the years.
They have to divert the fluid, since it can not flow properly, and the brain can’t simply absorb it. This is where the options for treatment come in, and they are both surgical.
The most common solution is the shunt. A shunt is a system that includes a valve and a catheter, to divert the built-up CSF from the brain to another part of the body, where it can be reabsorbed. In my case, it’s diverted to the peritoneal cavity (abdomen). While this is a good fix in most cases, 50% of all shunts fail in the first 2 years - primarily from occlusion (clogging) or syphoning.
The other surgical option is called an endoscopic third ventriculostomy (ETV). In very basic terms, they go into the brain where the fluid is blocked, and create a hole in the lining of the ventricle to allow the fluid out of the blocked area, into another area of the brain, where they hope that it will be reabsorbed. This is also a very practical solution, but you are relying on the brain to keep up with absorbing that fluid at the same rate that it is making new fluid…. and I have found that it is not commonly understood that ETV’s fail at the same rate as shunts. So, unfortunately, it’s not a permanent fix in many cases (including mine).
So here are the common questions that arise:
Let’s start with shunts.
Why can’t they make a shunt that doesn’t fail?
Some patients have good luck with shunts, and they last years, even decades. Then there are people like myself, who only average about 3-6 months between shunt failures. The simple fact is that my body needs the shunt, but hates it. As soon as it’s placed in my brain, my body starts to try to get rid of it, to heal it out. Tissue from the CSF coats the valve(s) and the catheters, which are small, and it doesn’t take very long before it stops working.
Why can’t they make a more sophisticated shunt that has a self-flushing feature, that could prevent all these issues from happening?
From my understanding, there is a general lack of awareness for hydrocephalus as a condition, which leads to a lack of federal dollars being earmarked for research, which then leads to medical equipment companies choosing not to prioritize new solutions. So, for lack of a nicer way of saying this….. there’s no money in shunts. In recent years, I believe that we are making strides in this area - President Obama recently appointed federal dollars for hydrocephalus research for the first time ever. We are hopeful that with more education and awareness, better solutions will arise.
If the problem is caused by a blockage, why can’t they just go in and clean out the blockage?
This does seem like an obvious solution, but the answer is simply that the risk-reward ratio is too high. The aqueduct where the blockage occurs is so tiny, you can’t just go in and roto-rooter it out. The risk of hemorrhage or other serious complications make this impossible.
Why isn’t the ETV a permanent solution?
The ETV is a really great option for those of us who are candidates for it - the best part about it is that you don’t have that foreign object in your brain (the shunt) that your body just wants to get rid of. If the ETV works for a patient, it can relieve the pressure, and for some patients, it can be a permanent or long term fix. However, in a lot of patients, either right away or eventually, the brain is unable to continue to absorb the extra CSF at a rate that keeps the pressure at a stable, lower level. In my case, it was 11 years.
Can’t they give you a medication to slow the production of the CSF?
Yes, they can. There are side effects involved, but it is an option that works for some. It has not been a successful solution in my case.
Shouldn’t you get a second opinion from another neurosurgeon?
This is one of the most common questions I get, and I try not to get frustrated by it… because it usually comes from people who are close to me, and just simply hate to see me going through the continued surgeries and the constant trauma. Here’s the hard truth - all neurosurgeons, all over the world, basically have the same options for treating hydrocephalus. What I believe, and what I have chosen to do, is to put together a team of medical professionals based on my ability to communicate with them, and my belief that they are open to my input in terms of my care. If you read my website and my blog, you’ll understand that we are choosing to be as proactive and progressive as possible. Every one of my providers and therapists strongly believe the best way for me to cope with the devastating aspects of the surgeries is to make sure that my overall physical and mental health are as good as possible. I have found my current neurosurgeon to be an excellent fit for me, personally - and would encourage all hydrocephalus patients to approach their care similarly.
Here’s the other thing I’d love for everyone to understand in terms of living with hydrocephalus, and this goes along with the “it’s complicated” statement that I started with. The symptoms of shunt failure are often different in every single patient, and often are different in every single episode even in the same patient. Sometimes the failures bring extreme and obvious symptoms, and you are rushed into emergency surgery. But other times, the malfunction happens slowly over time, and it takes lots of testing, patience, and perseverance to figure out that your current solution has stopped working. There are no tests that are absolutely fool-proof when it comes to diagnosing a shunt or ETV failure. Imaging (MRI or CT) often doesn’t give enough information to tell the whole story. Intercranial pressure can be tested in a number of ways, but also does not always tell the whole story. And in the end - pressure doesn’t always cause the pain. Extra fluid causes the pressure, but the pressure in your brain is ever changing throughout every minute of every day… and it is very difficult to pin down an issue. So you get some idea of how difficult this is for the doctors involved. It’s the epitome of the term “moving target”. It’s almost maddening at times.
In my experience, diligently tracking my daily functionality, noting any changes, and having a neurosurgeon who is very familiar with how I function when I am doing well vs. when my shunt is malfunctioning, have been the most important factors in diagnosing and quickly resolving my shunt failures. I rely on all members of my team to note when they feel that there has been a change in my function. This includes my neurosurgeon, my personal trainer, and my rehabilitation therapist. Even my family, close friends and coworkers will mention to me when they feel I might be slightly “off”. It’s so important to understand and track your baseline function, so that you can identify when you are truly having an issue, vs. just having a bad headache day.
I hope that this blog post will help you to better understand the answers to these questions, coming from someone who is living this every day. One of the main reasons I launched this website was to share with the world the real-life experiences and information that I've obtained over the years, and I hope that over time, the sharing of this information may bring hope, comfort, motivation, and closure to others who are battling hydrocephalus themselves, or supporting someone who is fighting this condition.
Never give up the relentless pursuit of better solutions, my friends.
My name is Amy but friends and family call me Am. I am a lover of dogs, good whiskey, and strength training. I'm a brain surgery survivor (x21), a fiddle player, a construction designer, and a boxing enthusiast. I have six real siblings, and five fake brothers. I love deeply, and consider my close friends to be family.