Friday, April 11, 2014

Guest blog (Berkley's life story)

Hi, my name is Berkley, I'm 18 years old, and I'm from Ontario, Canada. So, I was born with hydrocephalus, which is caused by an accumulation of fluid in the ventricles of the brain. I have a shunt that drains this excess fluid from the ventricles to my abdomen where it can be absorbed properly. I have had 21 brain surgeries, and 27 all together because of it. 15 of those surgeries have been in the last 3 and a half years. Before I was even one year old, I had 9 shunt malfunctions until a second shunt was placed. I was fine from my first birthday to around my 15th birthday. I had shunt tube lengthening when I was 9 and ended up having emergency surgery because I was playing soccer at school and was pushed into the ground. This caused my stomach incision to come open. In October of 2010, because I had been feeling nauseous in the morning since about July of that year, I went into the clinic to have a shunt series, and a CT scan done. In November, I went back to review the results with my neurosurgeon. She then informed me that the shunt tubing was disconnected at my neck, so I had surgery the next week to reattach it. My shunt tubing was worn on both sides (I had 2 shunts at the time) so my neurosurgeon decided to take out the tubing on both sides and replace it with new tubing. 

In the months following, I developed symptoms of chest pain when I was breathing deeply, a constant headache, abdominal pain, and shunt pain. In March of 2011, I went into emerg with pain in my side. They suspected appendicitis so they took my appendix out. On April fools day of 2011, a constant headache started. A few weeks later it hadn't let up so my mom took me into emerg again and they admitted me to do some tests. They did a shuntogram (basically a spinal tap in the shunt rather than the spinal column) and a couple of days later, they put an ICP (intracranial pressure) monitor in my brain so they could monitor the pressure in my head. Right when I came out of the operating room, my neurosurgeon found out that I had a shunt infection. She finished the monitoring and then gave me the options I had for treatment, and I opted for the surgery over 2 months of IV antibiotics. I was absolutely terrified because this was a surgery I had never had done before, and I didn't know what to expect because they'd have to drain the fluid outside of me into collection bags. I was expecting something completely different from what it was actually like. 
They put the drains in, and gave me heavy doses of IV penicillin over 13 days. When it came time to put my shunts back in once the infection cleared, my neurosurgeon decided that she was only going to put one shunt back, remember, I had issues when I was a baby until they put the second shunt in, then I was fine until all of this. A few days after they put the shunt back in they discovered that the tubing was balled up in the wrong place in my stomach so they had to go back in and fix it. I was discharged the next day.

 Over the next few months I had developed more symptoms; dizziness when standing, nausea, the headache and a bunch of other things I had with the infection were still there. I had been admitted over the summer a few times for observation, and a different neurosurgeon finally decided to try a VP programmable shunt. They didn't believe it would make a difference, but they still put it in, and did another ICP monitoring at the same time. This all happened to be on my 16th birthday. In the months following that, I still had the dizziness, constant headache, now shunt pain, and trouble sleeping with the new shunt. I went to see a different neurosurgeon in November of 2011 who did nothing for me, and said that my headache would go away once my body got used to the new programmable shunt I had placed. I had been on multiple different medications to help with the headache and nothing touched it, and still doesn't. 
In January of 2012 they did another ICP monitoring because I developed papilledema (swelling of the nerve in the back of the eye). It is also a sign of increased pressure in the brain. The numbers were fine (typical for me) so they still have no idea what is wrong or what they can do to fix me.  
Since the pressure monitoring, I had still been dealing with these medical issues. I was sent to a new neurosurgeon in December of 2012, who, in the beginning, was unsure of what to do for me. He originally thought of once again doing an ICP monitoring due to the fact that he's at a different hospital from my original surgeon, and wanted to see readings on his own equipment. After this appointment, we did not hear from him for more than 6 months. When we finally did in July of 2013, it was because of the fact that the headache had become even worse. I was admitted the same day, and had an MRI the next morning. My neurosurgeon, upon reviewing the results and comparing them to past CT's and MRI's, realized that my ventricles were slightly larger than at times when I was well. Although there was very little change, he decided to go with this idea, and opted to do exploratory shunt surgery. Through doing this surgery, he discovered that my shunt was blocked, and had likely been since shortly after it was placed in August of 2011. He replaced the component of the shunt that was blocked, and I was sent home a couple of days later.
After this surgery, once I had healed a little bit and the pain from the surgery was gone (about a week later), I realized that my headache was no longer there, I was so happy! Life felt like it was beginning to go back to the way everything was before all of this started. On August 4th (A day after my 18th birthday), the headache returned. I went back to the hospital the next day, and had another MRI done. This came back with very clear results, showing that my ventricle that did not have a shunt placed in it anymore, had blown up to be almost 2 cm in diameter. This made diagnosis very easy, and led to another surgeon who was working while my original surgeon was on vacation, to decide on placing a second shunt back into this ventricle. It was successful, and my ventricle went down in size right away. After the surgery, my headache was still there. My neurosurgeon was once again unsure of what to do for me, as I had my second shunt back, and my ventricles had shown to still be very small (slit-like at the time) based on MRI's I was having done monthly since the second surgery that summer.
The incision from the surgery in July had never healed properly, so on January 21st of this year, I went in to have an incision revision done to reclose it. After this was done, I was sent home on antibiotics. When I was in the OR, a shunt tap was done. A couple of days later, we got a call from my neurosurgeon who told us that my shunts were infected, along with the area around where the incision that never healed was. They asked us to come back that same day to re-do the shunt tap to make sure it wasn’t just a contaminant that interfered with the culture results. The next day, we received another call from my neurosurgeon because the results had already come back with a fast-growing bacterium. I was told to come back to the hospital the next day (January 31st) for emergency surgery to have external drains placed. I had a PICC line placed on February 3rd, and was put on vancomycin and meropenem to treat all of the 3 infections that I had (2 in the shunts, and 1 in the incision). The meropenem was stopped a couple weeks later because they didn’t think that it was helping with anything, but I was kept on vancomycin for 6 weeks.
On February 12th, I had the ETV (Endoscopic Third Ventriculostomy) procedure done in hopes of not requiring a shunt once the infections had cleared up. It basically involves burrowing a hole into the floor of the third ventricle to allow fluid to flow through it, and this theoretically allows for the CSF (cerebrospinal fluid; the fluid that protects the brain and spinal cord) to absorb within the brain like it would in a normal person. After this surgery, I ended up with aseptic meningitis (blood in the CSF), which is extremely painful. I was on steroids for a couple of days, and was left to let the blood clear from the CSF on its own, which was not a pleasant process. Over the next couple of weeks, my drains were clamped and unclamped a few times to see if my body could handle absorbing CSF the way a normal, non-shunted persons body does. Even when the drain was open, I leaked CSF from the drain insertion site. I had to have extra stitches put in, and this didn’t seem to fix the problem. We were told that when this happens, the only real solution is to have a shunt put back in. So, on February 27th, when Infectious Disease had finally cleared me, I was booked in to have a new shunt placed. This time, it was going to be just a regular fixed pressure VP valve (this is what I had up until my 16th birthday), rather than a programmable. After the shunt was placed, I was unable to sit or stand without getting sick, and had severe neck and shoulder pain. They figure that it was caused by there being a lot of swelling and blood along the shunt tract due to a reaction from where the shunt was placed, as I had never had a shunt in this ventricle before. The only relief I had from this pain came when I was lying completely flat. I was sent home on March 1st on PICC line antibiotics. A week later I returned to the hospital still in as much pain as I was before I was discharged. They did an MRI, shunt series (x-ray along the path of the shunt tubing), abdominal ultrasound, and shunt tap. These all came back to be normal, so I was sent home after consulting with the pain team who told us to try a muscle relaxant, which didn’t help with the pain at all. I had to lay flat as much as possible for just under 3 weeks before the pain finally subsided enough for me to function.
I have now been transferred to an adult neurosurgeon here in my city, and am waiting to see him still. I'm hoping that with this new surgeon, something will finally be figured out, and that my headache will go away.

 My Life With Hydrocephalus: Berkley

1 comment:

  1. Well done for writing about your own experiences, Berkley. I am 25 years old and I was diagnosed with hydrocephlaus and had a VP shunt inserted at 4 months old. I've had about a dozen neuro operations. I had a particularly bad experience when I was 16 when my hydro was not treated in time and the CSF built up over a period of weeks. That was around the time I was transferred from the children's hospital (The Royal Sick Kids) to the Adult hospital (the western General) in Edinburgh,Scotland.

    I have been raising awareness of hydrocephalus for the past few years and have been to ISHCSF conferences in Copenhagen, Kyoto and Athens and I presented my own E poster in Athens last year on the educational outcomes of young people with hydrocephalus

    I am still trying to raise further awareness of hydro and am hoping to go to more conferences, one in Bristol this year and another in Calgary next year (which is an exciting prospect as I have never been to Canada).