Glasses part 2

So..I decided to shop around a bit as I wasn't completely happy with the glasses I picked out last weekend. I still have a few days before I can finalize my choices as the insurance company doesn't make exceptions, even in cases where eye exams are required annually.

Sears has a special going right now.



So I decided to see what they have to offer.

I ended up picking 2 pairs for less than my original choice, with anti glare treatment. The pair on the right have a slight purple tint to the frames, picked for me by my daughter Rachel.

Rachel had another lesson tonight, this time on Bailey, who happened to be the first horse she rode.

Rachel leading Bailey in for her lesson

2014 - more of the same

I continued into 2014 with regular follow ups with Dr. Rodriguez for my eyes, Dr. Khalidi for the pachymeningits and auto-immune treatment and Dr. Klurfan for the IIH. I was having regular blood work done and MRIs every 4-6 months.

In May, I stopped taking the Plavix that Dr. Klurfan had placed me on along with daily ASA for prevention of blood clots following the stent. The balance of my medications remained the same, though I was finding the gravol was not sufficient for anti-nausea purposes and began using Ondansetron (Zofran) twice daily. Dr. Klurfan decided that there were indications in my MRI that I was experiencing over-drainage from my shunt.  A procedure to tie the shunt off and prevent it from draining was scheduled for June.

Plavix: Clopidogrel is used to prevent heart attacks and strokes in persons with heart disease (recent heart attack), recent stroke, or blood circulation disease (peripheral vascular disease).It is also used with aspirin to treat new/worsening chest pain (new heart attack, unstable angina) and to keep blood vessels open and prevent blood clots after certain procedures (such as cardiac stent).It works by blocking certain blood cells called platelets and prevents them from forming harmful blood clots. This "anti-platelet" effect helps keep blood flowing smoothly in your body. 

Daily ASA: Aspirin, the common pain reliever that has been in our medicine cabinets for more than a century, also has a talent for prevention.Aspirin prevents blood clots from forming in your arteries. This can prevent a heart attack or stroke. 

Ondansetron: blocks the actions of chemicals in the body that can trigger nausea and vomiting. Ondansetron is used to prevent nausea and vomiting that may be caused by surgery or by medicine to treat cancer (chemotherapy or radiation). Ondansetron is not for preventing nausea or vomiting that is caused by factors other than cancer treatment or surgery.

Healed incision from shunt procedure
virtually invisible

The procedure to tie off my shunt was done June 10th.  A relatively simple process done in the procedure room of the Clinical Neurosurgical unit of the Hamilton Health Science. Done under a local anaesthetic, a small incision was made in my neck and sutures were placed around the shunt tubing then the incision was closed with a couple more stitches.

My headaches continually worsened. I spent the days in bed unable to lift my head from the pillow. The pain was even worse than before the shunt was tied off, the swooshing increased and I became even more sensitive to light and noise. I was on the phone daily with Dr. Klurfan's office. She reversed the procedure a week after it was done and while relief was not complete, I returned to previous levels almost immediately.

In July, Dr. Klurfan made some referrals for me, specifically Pain Management Clinic and Bariatric Medical Program. Appointments for both were booked in October.

I continued with follow ups, medication and more MRI's from July to September. During my visit to her office in September, Dr. Klurfan booked me for a lumbar puncture to clarify what was happening. My MRIs showed small ventricles on the right side, almost to the point of collapse, but on the left I was showing larger ventricles. I was booked for the procedure room once again and Dr. Klurfan did the LP herself on October 1st. A plan was in place contingent on the LP results.

My opening pressure was 31 that day, a complete opposite of what the doctor was anticipating. She drained off a bit and took a second reading.  Closing pressure was 20, considered to be a high normal. I felt immediately better, the swooshing eased off and the intensity of the headache dropped significantly. I was sent home with plans to return on the 3rd for yet another MRI, a follow up and pre-op clinic on the 8th, a CT scan on the 9th and shunt surgery on the 10th.

Based on my previous shunt experience, I made the decision to start out with a hair cut to make the clean up and after care simpler.

underside of pre-surgery cut

Pre-surgery cut

I was also sent for a CT scan the day before my surgery was booked to have "doughnuts" placed, technically, markers for guidance during the surgery.

Guides in place 
 must stay on until after surgery

Surgery was scheduled for noon on the 10th, and again went well. I woke up with 2 new incisions on the left side this time, one in my head and one on my stomach and my hair was shaved even more.

Post-op Incision - Left Skull - 2014

I found the post surgical pain this time wasn't as intense as it had been in 2012, perhaps due to slightly different techniques of the surgeon.  The tubing for this shunt was not as obvious as the one on my right side as the path seems to be at the back of my neck rather than towards the front.  I did find that post-op nausea and vomiting was much worse than previous, continuing daily for about 2 weeks. I once again relied on Ondansetron to counter-act it. I gradually improved and managed to carefully bake a cake and take part in Rachel's 5th birthday!

Healed Abdominal Incision
Left - 2014

Healed Abdominal Incision
Right - 2012

My appointment with the Pain Management Clinic was cancelled as they had overbooked and I was advised they would contact me with a new appointment at a later date.

October 27th, I had my first appointment with the bariatric program. It was not quite what I was expecting. I was not aware that the first appointment was actually a group information session to learn about the program and how it worked.

The technician explained that for 12 weeks there would be weekly group sessions to learn about and discuss our weight loss. They also explained that the medical program was strictly diet.  The available surgeries for weight loss were discussed, the options being gastric by-pass or gastric sleeve. Gastric banding was also mentioned, but not as an offered procedure as it is a reversible option that is not covered under provincial insurance. If we were still interested in going forward with the Bariatric Medical Program, a further appointment could be booked. If we were looking for weight loss surgery a new referral to the Bariatric Surgical Program was needed.

I booked my appointment for November 19th with the intention of discussing the medical program further with the doctor.  I had a primary concern that I could not travel weekly to Hamilton to attend a 2 hour long session. I don't drive, and when my sight was first affected, I was advised by Dr. Rodriguez, if I had my license he would be required to pull it for medical reasons.  This meant that any appointments I have, Rob needs to take the time off work in order to take me there, spend the time waiting, then stay for the actual appointment and that doesn't include the time to travel from Brantford to Hamilton and back.

When I saw the doctor, he agreed that I could pursue a one-on-one schedule.  I think the fact that I had managed to lose 40 pounds between my referral and my first meeting with him helped to show that I could lose the weight successfully and really only needed monitoring.  We agreed that I would continue as I had been and return for a follow up visit after the new year.

December 2nd, I was scheduled for a follow up CT and appointment with Dr. Klurfan.  She was happy with the results of the surgery. Ventricles appeared to have evened out in size and I also appeared to be draining normally and once again the swooshing noises had disappeared, though I still had headaches, they were much more manageable. At this point Dr. Klurfan advised that I no longer needed to see her as we had explored and used all options currently available to us.  I was left with the reminder to call her if my neurological symptoms increased or changed in any way.

2012 - FINALLY!! Official Diagnosis

2012 was the beginning of true progress for me.  This is a long one! A lot of results in such a short time when you consider my journey started in 2008 and I'm only just receiving an actual diagnosis 4 years later.  

Following the surgery in 2010 I continued regular follow ups with the surgeon and had been advised that the headaches and the whooshing sounds in my ear were something I would just have to live with. When I started getting visual white-outs, I just chalked it up to yet another symptom to be lived with and didn't make a rush visit to my doctor.

My next follow up was April 2012. Mentioned once again the whooshing in my ears. 
A difficult sensation to describe but the best comparison I could find was to say it was as if I was holding sea shells over my ears all the time. I'm sure anyone who has done this and "heard the ocean" knows that in reality what you are hearing is actually the noise of the surrounding environment, resonating within the cavity of the shell. You can simulate this by holding a glass or even a hand over your ears. In my case though, nothing was covering my ears, and I was hearing whooshing sounds all the time, worse if I was to bend forward or do anything that required straining, not the best thing after a recent move as shifting a box would increase the sensation. Additionally, I had the white-outs. Those continued to increase in frequency and duration. By this point, I would lose my vision for roughly 30 to 45 seconds, not long, but certainly enough to cause concern. 

Papilledema - Swollen Optic Nerve

During my follow up, Dr. Harvey dilated my pupils and did a more thorough examination of my eyes. Then, I'm not sure if it was a slight panic or just a sudden "I know what's going on" as he left the room stating he'd be right back. A few minutes later, he returned with another doctor with him. He introduced me to Dr. Rodriguez, telling me that he was a Neuro-Ophthalmologist with the Eye Clinic. My eyes were again examined and I was told that I had swelling of both optic nerves (Bilateral Papilledema) and further follow up was required. 

Papilledema is a condition in which increased pressure in or around the brain causes the part of the optic nerve inside the eye to swell. Symptoms may be fleeting disturbances in vision, headache, vomiting, or a combination. Doctors make the diagnosis by looking in the person’s eye with an ophthalmoscope. The disorder causing increased brain pressure is treated as soon as possible. 

An appointment was booked for late May, along with a referral to a rheumatologist  and an MRI scheduled prior to that appointment. I was also booked for some additional tests at the Eye Clinic including specialized pictures of my optic nerves. 

I had my MRI (with contrast) then Rob and I took Rachel, Krystal and my sister Mandee to French River for the May 2-4 (Victoria Day Weekend in Canada). Great weather and for the most part a great time for everyone but I was rather restricted in what I could do. 

Anatomic arrangement of the facial nerve and muscles.
 nerve is depicted on the right side 
facial muscles on the left.

While standing in the kitchen of our cabin, I experienced a sensation that terrified me. I had no control over my face, and to me, it felt as though the facial muscles were twitching and pulling down towards my collarbone. Everyone that saw it told me it actually looked as though my face was pulling upwards with a weird smile on one side of my mouth while the other remained still. 

I had a lumbar puncture after the holiday weekend and discovered an opening pressure of 37, normal pressure readings with a patient laying on their side as I was is 10-18. I recall during that LP the radiologist drained 5 vials of CSF (Cerebral spinal fluid) then left the tap in place to drain for an additional minute. I was immediately started on acetazolamide (a-set-a-ZOLE-a-mide sometimes referred to as Diamox) to help keep the levels of CSF lowered.  At this point, I was officially diagnosed with IIH, (Idiopathic Intracranial Hypertension).

Due to the pressure on my optic nerves I did experience some permanent vision loss.  I regularily have Visual Field Tests performed.  I basically stick my head inside a drum and push a button every time I see a flash of light.  One eye is tested at a time, and the machine plots the results into a chart. The image below is representative of my VFT results on my right eye. I do have minor loss on the left eye, basically on the inside corner towards my nose. 

Sample Visual Field Test Result

The VFT illustration can be translated into my actual vision. Darkened areas are either non existent (toward the outer edges) or dim and shadowed when looking through my right eye.  The jagged appearance of the central white area translates into the actual view-port I have.  One thing I've noticed when having an eye exam is that I have to move and adjust my head, particularly when using the right eye in order to determine the parts of a letter or number that make up the whole.  The letter "C" for example, if I don't move my head around and bring the the open part of the letter into my central view, I can easily interpret it as the letter "O". 

A lumbar puncture (also called a spinal tap) is a procedure to collect and look at the fluid (cerebrospinal fluid, or CSF) surrounding the brain and spinal cord. During a lumbar puncture, a needle is carefully inserted into the spinal canal low in the back (lumbar area). Samples of CSF are collected. The samples are studied for color, blood cell counts, protein, glucose, and other substances. Some of the sample may be put into a special culture cup to see if any infection, such as bacteria or fungi, grows. The pressure of the CSF also is measured during the procedure. 

CSF acts as a cushion, protecting the brain and spine from injury. The fluid is normally clear. It has the same consistency as water. The test is also used to measure pressure in the spinal fluid. 

Acetazolamide is used to treat glaucoma, a condition in which increased pressure in the eye can lead to gradual loss of vision. Acetazolamide decreases the pressure in the eye. Acetazolamide is also used to reduce the severity and duration of symptoms (upset stomach, headache, shortness of breath, dizziness, drowsiness, and fatigue) of altitude (mountain) sickness. Acetazolamide is used with other medicines to reduce edema (excess fluid retention) and to help control seizures in certain types of epilepsy.

Intracranial hypertension is a neurological disorder in which the increased cerebrospinal fluid (CSF) pressure has generally arisen and remains elevated over a sustained period of time. It can either occur without a detectable cause (idiopathic intracranial hypertension) or be triggered by an identifiable cause such as an underlying disease or disorder, injury, drug or cerebral blood clot (secondary intracranial hypertension). It is frequently a life-long illness with significant physical, financial and emotional impact.

On the drive from Hamilton to Brantford after my lumbar puncture, I experienced another facial spasm, this time I managed to capture a picture while it was happening. 

facial spasm in action
note the downward pull on the left of the face

My next appointment was with the rheumatologist, Dr. Khalidi, what a great birthday present! My first visit with him was very informative, he had received the MRI results and the report indicated that I had signs of idiopathic hypertrophic pachymeningitis. He also received the LP results which showed that the collection of fluids had all tested clear. Next stop - yet another biopsy.

Idiopathic hypertrophic pachymeningitis is a rare disorder of unknown origin. It is a fibrosing inflammatory process that involves the dura mater. 

Head and Brain Cross-Section

I was referred to Neuro-Surgeon Dr. J. Wells, who met with me the first week of June. I was scheduled immediately to have a biopsy performed on the dura, the outermost membrane covering the surface of the brain, also called pachymeniges.


My biopsy was performed as day surgery, using twilight sedation. I managed to shock a few people by opting to have someone drilling into my skull while still awake, but the recovery process was that much quicker as I did not have to deal with the after effects of general sedation.

Results from my biopsy showed minimal non-specific inflammation, which allowed the doctors to rule out a few more things including lymphoma, tuberculosis and fungal infections.  This meant that Dr. Khalidi could confidently start me on immunosuppressive therapy as well as high dose prednisone (60mg/day started immediately after biopsy).  I was on the high dose for a month, then began to taper it by 5mg per week.
 
August 22 was another follow up with Dr. Khalidi after having an MRI the previous week.  MRI showed an improvement in the pachymeningitis.  I had also been started on cyclophosphamide, 200mg/day, the previously discussed immunosuppression therapy.  I was tolerating that medication fairly well, but continued to have the swooshing in my ears and daily headaches along with some side effects from the acetazolamide. I had noticeable tingling in my hands and feet and a slight metallic taste with some food or beverages.  I also had persistent nausea and constantly felt as though my head was under water due to the constant and rhythmic swooshing in my ears.

Cyclophosphamide is used to treat various types of cancer. It is a chemotherapy drug that works by slowing or stopping cell growth.  Cyclophosphamide also works by decreasing your immune system's response to various diseases. It is used to treat a certain type of kidney disease in children after other treatments have not worked.

The prednisone was doing its share of creating side effects also.  I had quickly developed the notorious Moon Face and hunch.  Weight gain was also predominate while on high doses of prednisone, I quickly regained the weight I had managed to lose previously, partially due to cravings for sugars to counteract the lower blood sugar effect of the medication. 

Very obvious Moon face - Thanks prednisone!

One issue I face with my condition, is the proverbial chicken and egg. It is not known if the pachymeningitis created scarring that caused improper CSF drainage, or did the increase in CSF due to idiopathic intracranial hypertension create the conditions for the pachymeningitis to form.

I was having blood work done on a weekly basis to monitor various levels.  I continued on my medications until November, when it was noted that there appeared to be changes in my kidney functions due to the acetazolamide, it was immediately stopped and I was referred to a neuro-surgeon to discuss the next step in my treatment.

The recommendation from Dr. Wells was to proceed with placing a VP (ventriculoperitoneal) Shunt to assist in draining the CFS from around my brain and help to reduce the pressure that was causing the whooshing and headaches.  I was booked to have the surgery performed December 19, 2012.  

Ventriculoperitoneal shunt (VP shunt)

The surgery wasn't too bad in my opinion, I was placed under full sedation and woke up a few hours later in the recovery room. My first comment when I was awake was "it's quiet", the pressure, pain and whooshing in my head were gone. I did have some post surgical complications, nothing major, just a few issues with tolerating the effects of the sedation.  

One thing I did learn with that trip to the hospital.  If you have unpleasant reactions, or don't tolerate certain medications well, list them under the allergy section, and explain your sensitivity. I had a nurse basically storm out of the room after bringing me Percocets for pain. When I advised him that I couldn't take them due to nausea and vomiting he was a little upset that it wasn't noted on my chart and that was what had been ordered by Dr. Wells.

I had an incision at the back of my head on the right side and another incision on my stomach. Recovery wasn't easy, but I have to admit, I had more pain and weakness from the tunnelling procedure and placement of the drain than I had in my head.  I spent Christmas day at my in-laws dressed in festive red pyjamas, because I was too sore to wear more. The staples and sutures were removed by Dr. Wells at a follow up appointment before the end of the year. 

Neurosurgical Shunts: Shunt operations are also performed when drug therapy has been unsuccessful. Shunts may be used to control papilledema and prevent vision loss, as well as to treat headaches that have been unresponsive to any medication. A neurosurgical shunt is a surgically-implanted catheter that is used to drain CSF into another area of the body such as the abdomen. A shunt lowers intracranial pressure by removing CSF to another site, where it can be absorbed.

Such a wonderful year, first official diagnosis received for my birthday in May, tons of weight gain for Labour Day, and now Merry Christmas, here's an operation for you.  I will admit, I had a wonderfully quiet (no swooshing) New Year's Eve!

2011 - Not much change

My headaches continued to persist on a daily basis with no resolution in sight. I had repeated follow ups with Dr Harvey, and it was his opinion that I would have to learn to live with the headaches as he could see no reason for them. His primary concern was continuing to monitor my double vision and ensure that there was no re-occurrence of the pseudotumour.

Inactivity due to the headaches did contribute to a slow increase in weight, but I was finding it more difficult to keep active beyond caring for a toddler. 

Not so flattering - August 2011 approximately

In June, Rachel became an escape artist, climbing out of her crib, so we moved her to a toddler bed for safety reasons. 

Rachel testing out the new toddler bed

In October, I did begin to notice occasional white-outs of my vision when changing position, especially if I bent forward to pick something up. I didn't think too much of it at the time at least I didn't think it was worth an extra trip to Hamilton when I had an appointment coming up in the new year. I did manage to lose about 30 lbs of the weight I had gained but with the hypothyroid condition, weight loss is not easy. 

Rachel's 2nd Birthday

Rob got a new job in Brantford and after commuting for a bit, we decided that it would be better to relocate our family before winter made the commute more dangerous. We moved December 1, 2011.