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 21^st 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 31^st) for emergency surgery to have external drains placed. I had a PICC line placed on February 3^rd, 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 12^th, 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 27^th, 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 16^th 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 1^st 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

Hydrocephalus and autoimmune diseases (Guest blog by Keyt Harrington)

Hello! My name is Keyt (Kate) Harrington and Tim has asked me to tell you a little about my story.

I am 43 years old and it is believed that I was born with hydro, but sadly it wasn't diagnosed until I was 5. I spent my early years with terrible seizures, jaw and mouth issues, medication allergies and countless emergency room visits and hospitalizations. It wasn't until I was 5 years old when I had my first bought with cerebral meningitis (I've had it 3 times!) that doctors finally discovered why I had been so sick.

Since then I have had 20 shunt replacements or revisions and had my gall bladder, appendix and right kidney removed plus a full hysterectomy. I have also had multiple surgeries on my feet, mouth and several other exploratory surgeries.

All of that, believe it or not is not the worst of my battles.

Approximately 7 years ago I began developing strange thickness and curving of my fingernails. They didn't hurt right away but as time went on it didn't matter what I bought over the counter, nothing worked. About a year after that started, my scalp began to itch, and as I scratched it I had flakes fall like it was dandruff. I tried dandruff shampoos but it made it worse. I couldn't wear dark clothing, wear hats of any kind or use most shampoos. That was it, I gave in and asked my doctor. She looked at my fingernails and my scalp and just said it was an allergy.

It has been said often by those of us who battle hydro or any long term illness that we know our bodies better than most doctors. I knew this wasn't an allergy but my doctor wouldn't look into it further. That was when I surprisingly gave up too. I let it go for another year, didn't talk to anymore doctors and fell into a depression as my fingernails kept swelling and my hair began to fall out.

Then one day I woke up after only a couple of hours sleep because I was itching so much and I decided I HAD to do something. I had spent a lot of time reading about skin conditions and remembered that sunlight could help. I was a guard at the pool at my apartment so I thought it would be perfect! I had nothing to compare myself to all those photos I saw online except for my nails because I had no idea what my scalp looked like! So I asked my neighbor's husband if he could shave all my hair off. He looked at me funny but I told him it wouldn't be a big deal because when I was little, neurosurgeons would shave off all of my hair instead of just the area they were operating on like they do now!

The day Mike shaved off all of my hair, his wife, Justine invited a bunch of our neighbors and we grilled some burgers, had a few beers and made a party of it!

But...once my hair was all gone, I saw Justine crying. When I asked her what was wrong, she handed me a hand mirror. Before I even looked, I took a deep breath. I knew I was going to see something bad, but little did I know what I'd see!

It's called plaque psoriasis. Those little white things raise and sometimes fall off on their own. I eventually developed a habit of picking them off because they would pull my hair and really hurt! Plus notice how it's very close to my shunt scar? Well, this was 3 years ago. Not long after this photo was taken it did creep onto my scar and down my neck. I just assumed I would have to live like this forever.

That was until I met my boyfriend, Aric 7 months later. When we met I barely had any hair. He said it didn't bother him, but what did was the fact that I was so miserable. He called around and found a different general doctor for me and insisted that I make an appointment. I had already spent 2 years not seeing any doctor so I didn't have any interest, but he told me I already had enough going on that I didn't need this too. So I made the appointment and joked that the poor doctor will kick me out because I had so many health problems!

Dr. Coombs was shocked that one person could have as many health problems as me and still be alive or not in some mental institution from major depression! She immediately sent me to a dermatologist but after a few months there and only receiving a few injections, I wasn't happy. By this time I could barely walk, lift, bend over or anything and she knew it had to be something more than just psoriasis. She decided to send me to a rheumatologist because they deal in both skin and bone conditions. After a couple of blood tests Dr. Dinning confirmed  psoriatic arthritis. I cried because finally someone believed me and confirmed what I had known for 7 years!

Basically, psoriatic arthritis is psoriasis and arthritis together. It's much like other forms of arthritis but it can often be worse because of the skin irritations. It's an autoimmune disease. That happens when the immune system mistakes some part of the body as an organism and attacks it's own cells. Anyone at any time and any age can get an autoimmune disease, but usually people who have had surgeries (even just one!) or some other disease (trauma) will develop an autoimmune disease. Psoriatic arthritis is just one, there are other more common ones such as celiac disease, eczema, lupus and multiple sclerosis. 

Unfortunately since my official diagnosis last year I've gotten worse. It's harder to walk, I can't stand for long periods and my sleeping patterns are worse. In March, my doctor decided it was best that I try infusions. As soon as he said that word I cringed. Not because the idea of infusions bothered me, but the needle did. I'm not scared of needles, but when you've had as many surgeries and hospitalizations as I (and many of us) have, your veins begin to fight back!

This tatt gets a lot of attention. When in an emergency room I've had people come from all over the hospital to see it or people take photos with their cell phones!

The first two times I did an infusion it took 6 tries each to get a needle in a vein. Since I had to have them regularly (and will have to for the rest of my life!), my doc sent me in for an infusaport. It's like a PICC line but it's under the skin and can stay forever.

Now all it takes is a poke into a small little port under my skin and I don't feel a thing!

The infusions don't completely take the disease away. In fact, after I began them, I developed raised spots on my legs.

These are just as itchy and uncomfortable as the ones on my head! I have about a dozen more since this picture was taken a month ago. Wouldn't you know they develop during summer too! But, here's how I look at this; I'm alive. I can't work anymore but I have a wonderful boyfriend whom I live with in a home that's paid off. We build things to sell and make money plus I get Social Security so it's not all that bad. We have a small farm with approximately 80 animals so they along with Aric all keep my spirits up. I try to look at what I do have instead of what I don't anymore.

Plus I have wonderful friends like Tim who allowed me to tell my story and spread the word that things could be so much worse!