October 2016 Update

I've been neglecting updating lately, things haven't changed much, so there hasn't been too much to say lately. I'm still in a holding pattern. My headaches are still occuring daily with not much change, my vision is stable and no new issues have come up. I had my annual MRI done yesterday and will be seeing my doctors soon for my regular round of visits.  I have added a nuerologist to list of doctors, and she has started me on a new medication in the hopes of reducing the headaches.

Torpiramate: Topiramate is classed as an antiepileptic medicine. It is usually used to prevent seizures in people with epilepsy and there is another leaflet called Topiramate for epilepsy which gives more information about this. However, topiramate has also been found to prevent migraine attacks from occurring. When used for migraine, it may not completely stop every migraine attack, but the number and severity of attacks are often reduced.  Patient Info on Torpiramate 

I am really dealing with the side effects of this medication! Tingles, pins and needles, my hands and feet constantly feel like they have fallen asleep, though it has improved compared to when I first started on the medication.  Initially, my extremities had a constant vibrating sensation. I can't think of any other way to describe it, now I only get the sensation when I move certain ways or if I'm extremely tired or cold which is certainly an improvement.

I just got a reminder on my Facebook feed.. 2 years ago today, I was recovering from my most recent surgery.  I had just had my second shunt placed, so I have come a long way in that regards. 

October 2014 - Shunt placement incision

I haven't been making as many cakes lately, I just haven't had the energy of course, I have been dealing with the seasonal back to school cold and sinus stuff which seems worse than normal this year, and I find they just don't get eaten when I do make them, though I will be doing some baking this coming week, Rachel turns 7 next week, and our niece will be a year old! Will post them up when done :) 

Speaking of Rachel, her riding lessons have continued and she is making huge improvements! She is off the lunge line and riding around the arena at a trot with ease, sometimes with a little more courage than we are entirely comfortable seeing!

Rachel trotting during her lesson 

I have started going back in for regular massage appointments! Just a little shout out here to Massage Addict in Brantford! I go in once a month for a massage to help ease the tension in my neck and back created from the vision and neck and does it help!! I've been sharing my story with my therapist and word of my blog is getting out there.

Just a reminder for anyone who is checking this blog out for the first time to be sure to go back to the beginning and read from the first post.

Hobby wise, I've gotten back into playing the Sims to kill time. Currently, I've been checking out The Sims 4.  It gets mixed reviews, but I don't have too many complaints so far.  If anyone is interested, I've been blogging my game (one reason I haven't had as much activity here I guess) My Sims 4 Blog and have been creating some walkthroughs and keeping my web skills up a little.  

MRI with Contrast

Interesting article posted today, one that could be of concern to people with IIH who have had multiple MRI scans with contrast. I know I personally have had many MRIs since I was diagnosed in 2012 and every one of them has been with contrast agents. I don't know if the contrast mentioned in the article is the same as the one used in Canada, but definitely something to keep in mind.

It's not known whether these deposits are harmful or can lead to adverse health effects. "To date, no signs or symptoms of adverse health effects and no pathological changes have been associated with these gadolinium deposits in the brain," the FDA notes.

For the full article see: Gadolinium Brain Deposits on FDA Radar

I received my referral to my next neurosurgeon last night. I'm going on to my third NS. First one retired, second one has been on leave since at least April and now I have a referral to this one as my follow up after my hospital stay in May never did contact me with a new appointment after his office cancelled my appointment. My appointment is booked for August 6th.  Not that long of a wait considering I've been in kind of a holding pattern since June with the follow up situation. Of course, the appointment notification came with the standard "if unable to wait, please go to nearest ER", which, although there is a hospital in Brantford, I interpret as "please go to ER at Hamilton General Hospital" simply because Brantford doesn't have the neurological facilities Hamilton does. 

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.

2013 - Remission?

Family - July 2013

2013 started out to be a great year!

Before getting into the year, a reminder, there is no cure for IIH.  The best we can hope for at this time is to enter a state where symptoms are non-prevalent. Similar to someone successfully treated for cancer, IIH is said to enter into a remission state.

My symptoms from the IIH were minimal to non existent. Occasional headaches would set off a warning sign for me though, always creating the concern "is it back?" only to have the headache completely disappear with a dose of acetaminophen or ibuprofen.  I wasn't medicating for pain on a daily basis.

Medication wise, I was still taking a number of things.  Eltroxin for the hypothyroidism, prednisone had been tapered down to 10mg/day, a much more tolerable dose. Tecta had been added to help counteract the indigestion issues I had with the prednisone. Cyclophosphamide was switched out for Azathioprine (Imuran) and I was on a daily dose of antibiotic for prophylactic purposes.  I recall telling my nephew at one point that I took 30 pills a day, 22 in the morning and 8 at bedtime.  That's a lot of pills to swallow. 

Eltroxin: Levothyroxine is used to treat an underactive thyroid (hypothyroidism). It replaces or provides more thyroid hormone, which is normally produced by the thyroid gland.  

Tecta: Pantoprazole magnesium belongs to the family of medications called proton pump inhibitors (PPIs). Proton pump inhibitors are used to treat conditions where a reduction in stomach acid is needed 

Azathioprine is used with other medications to prevent transplant rejection (attack of the transplanted organ by the immune system) in people who received kidney transplants. It is also used to treat severe rheumatoid arthritis (a condition in which the body attacks its own joints, causing pain, swelling, and loss of function) when other medications and treatments have not helped. Azathioprine is in a class of medications called immunosuppressants. It works by decreasing the activity of the body's immune system so it will not attack the transplanted organ or the joints. 

Prophylactic: A preventive measure. The word comes from the Greek for "an advance guard," an apt term for a measure taken to fend off a disease or another unwanted consequence. 

Cyclophosphamide: Cyclophosphamide is in a class of medications called alkylating agents. When cyclophosphamide is used to treat cancer, it works by slowing or stopping the growth of cancer cells in your body. When cyclophosphamide is used to treat nephrotic syndrome, it works by suppressing your body's immune system.

New Glasses - May 2013

I was still seeing Dr. Rodriguez and Dr. Khalidi every 3 to 4 months as they continued to track my progress through MRIs and Visual Field Tests. Everything was looking good, the pachymeningitis appeared to have stabilized, my vision was no longer deteriorating though it was determined the loss of peripheral vision was permanent.

Headaches were at this point "just a headache" they came and went like a normal, occasional headache.  Thanks to the reduction in prednisone, I was able to lose some of the weight I had gained and my face lost most of the moon like appearance.

Immunosuppression Therapy, in my case, treatment with cyclophosphamide and later the use of Imuran, of course has its own risks. Primarily, the risk of infection from a simple cut is much higher. I was on a daily antibiotic for preventative measure. Shaving is discouraged, ensuring all cuts and scrapes are cleaned and cared for becomes a priority. Simple tasks such as grocery shopping become a source of potential infection. Hand washing and the use of portable hand sanitizers are constant habits, leading to hands that easily become dry and cracked, so moisturiser is stocked up on.

Research into IIH to date has not confirmed a cause, and there is no cure.  There are many theories and hypothesis, with the unifying factor being the poor absorption of CSF.  Weight, certain medications, physical structures of the skull, or narrowing of the arteries within the skull are a few possible causes.  In some patients, another medical condition has actually caused the increased pressure and is referred to as Secondary Intracranial Hypertension.

A very technical read, but a lot of good information on IIH can be found at Idiopathic Intracranial Hypertension.

Ready for birthday dinner - May 2013

Everything was looking up. As far as I was concerned, I was in remission and had been for 8 months, or at least 5 if you allow for the first 3 months to pass before considering remission. I was ready to return to work. I examined my options and realized that the reduction in eyesight created issues with pursuing computer programming and even IT support, the 2 programs I had completed when my medical issues began.  Working at a computer all the time was difficult, and required just the right lighting and position, (something not easily accommodated for in most workplaces) or I ended up with neck pain and stress related headaches.

In August there was a job fair for a new Toys R Us going in close to home. Given my limitations, I thought retail, perhaps a position as a cashier was feasible. The day before the job fair I had woken up with a headache that was more difficult to reduce than I had become accustomed to.  The  next day, I woke up again with a headache and decided to wait for the second day of the job fair.  For the third day in a row I woke with a headache and noticed the swooshing and pressure had returned.

I contacted my neurosurgeon, Dr. Wells, only to discover that he had semi-retired and was no longer seeing neurology patients in his office. I then contacted my neuro-opthamologist, Dr. Rodriguez and was booked to see him the next day. Examination revealed a slight swelling of the optic nerve but no further damage.  He advised that he would meet with some colleagues and set up an appointment with a new neurosurgeon.

venous phase of cerebral angiography
shows bilateral transverse sinus stenoses (arrows).

I saw Dr. Klurfan, the new neuro-surgeon for the first time in September. Thankfully all of my specialists are located in Hamilton, therefore, they all have access to medical records and tests through Hamilton Health Sciences. Dr. Klurfan reviewed my past history and on examination of my MRI results, which included venous scans.  She saw evidence of transverse sinus stenosis.

Stenosis: an abnormal narrowing or contraction of a body passage or opening; called also arctation, coarctation, and stricture.

I continued to see Dr. Klurfan for monitoring and additional MRI studies. In November, Dr. Klurfan, suggested that a stent procedure to open the narrowed arteries within my head was the best course of treatment. Placement of the stent was performed in the Diagnostics Imaging department.

Stent Procedure: A catheter is inserted (usually in the groin) and then threaded through the arteries and in either the arteries of the neck or brain depending on the location of the problem. The stent delivery system follows the same path as the catheter, and thus, carries the stent to the treatment site. The stent is then placed. As it is positioned, it expands to conform to the inside contours of the artery wall. After placement, the catheter is removed and the stent stays in place.  

I had dual catheters used to allow gradient pressure measurements to be obtained on both sides of the occlusion.  Pressure readings were repeated after the stent was expanded and indicated a 90% improvement in flow.  The procedure was performed using twilight sedation and the lines that were used to access my arteries were left in place for 12 hours.  I was unable to move or sit up while the lines were in place to prevent arterial bleeding. Once the lines were removed, I was monitored for a few more hours then sent home later that day.

Twilight Sedation: Conscious sedation is a combination of medicines to help you relax (a sedative) and to block pain (an anesthetic) during a medical or dental procedure. You will probably stay awake but may not be able to speak. Conscious sedation lets you recover quickly and return to your everyday activities soon after your procedure.

View of transverse sinus stensois before and after stent placement
Transverse Sinus Stenting

I recovered relatively quickly after the stent procedure. I had some difficulty walking any distance for about a week, but over all pain was minimal, with the exception of my head. Having the stent placed did not appear to have any noticeable effect on my headaches or the swooshing sounds in my head. On to the new year!

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!

2010 - Starting to get some answers

January - Had a CT scan following the MRI results from 2009 in preparation for my first appointment at the St Joe's Eye Clinic in Hamilton. Dr. J. Harvey, an eye surgeon specializing in oculoplastic, reconstructive and cosmetic eyelid surgery, with sub-specialty skills in the area of lacrimal, orbital and cosmetic surgery.

My MRI showed a mass on the right orbit, the CT indicated that the mass was centred on the right lacrimal gland. I was booked for surgery March 31st, to remove the mass and have it biopsied.

In January, I also attempted to return to work.  Managed to work a month an a half in a busy tax office as an assistant manager, but found the combination of headaches, vision issues and having a 3 month old baby at home was just too much.  February 27th was my last day worked.

Surgery was postponed to April 28th, 2010. Fortunately, it was a day surgery, I would be in at 11 am and ready to head home by 4 pm. The operation was pretty routine I guess, I was asleep the entire time so I can't say officially how it went. I did have some trouble waking up afterwards and I do recall hearing the nurses saying "let her sleep a little longer, she's got a baby at home" as I drifted between being asleep and awake.

Day after Surgery

Day after surgery, bandages removed

48 hours after surgery

A couple more terms/diagnosis came up resulting from the biopsy.

Tumefactive fibroinflammatory lesion is an idiopathic fibrosclerosing disorder occurring in the head and neck region. It is one of a broad spectrum of entities named inflammatory pseudotumors and, as the name suggests, it mimics a lot of diseases such as malignancies or infections.

Sclerosing orbital pseudotumor is uncommon. Due to unknown reasons, these tumors behave differently than other types of pseudotumor of the orbit. They grow more slowly, cause less pain, and are characterized by scarring (hardening of the tumor tissue).

Histopathology reveals large amounts of fibrous tissue.

Sclerosing orbital pseudotumor is not cancer. But, by local growth it can cause bulging of the eye (proptosis), double vision (diplopia) and loss of vision.

I certainly did have all of those symptoms in my right eye. Fortunately the surgery fixed the physical appearance, at least as far as the bulging and swelling around my eye. The drooping of my eye lid would continue along with the double vision and everything appeared to have a slight tilt similar to the image below. The picture below is a close representation.  In actuality, my vision appeared as if the foot on the right of the top A and the foot on the left of the lower A were touching while maintaining the angle shown.

CN IV Palsy Horizontal, vertical and Torsional
This is about the closest representation I can get to what my vision was like at the time.

Couple weeks or so post op.
Not winking, that's the limit I can open my eye

This led to additional aches and pains as I developed a tendency to tilt my head to counteract the visual information I was receiving. For the balance of the year and for 2011 I continued to see Dr. Harvey on a regular basis as he monitored my recovery and progress.

I had 2 eye lift procedures in attempts to correct the eyelid drooping as a result of the April surgery. 

A follow up MRI was performed and revealed that there was no further occurrence of the tumour. 

At this time, my official diagnosis was listed as orbital pseudotumour. My headaches continued to remain 24/7, varying in degree of intensity. Treated only with acetaminophen and ibuprofen.


On a more positive note, Rachel celebrated her first birthday October 23 with the help of family and friends.
  

Cousin Ethan helps Rachel with her mash cake! Happy Birthday to our little princess!

2009 - Lots of good and bad

January - I got a little good news, my thyroid levels had returned to normal range with the help of medication.  The bad news, I would be on thyroid medication for the rest of my life. Diagnosis was Hypothyroidism.

Hypothyroidism is a condition in which the body lacks sufficient thyroid hormone. Since the main purpose of thyroid hormone is to "run the body's metabolism," it is understandable that people with this condition will have symptoms associated with a slow metabolism. 

Symptoms of Hypothyroidism

• Fatigue
• Weakness
• Weight gain or increased difficulty losing weight
• Coarse, dry hair
• Dry, rough pale skin 
• Hair loss
• Cold intolerance (you can't tolerate cold temperatures like those around you)
• Muscle cramps and frequent muscle aches
• Constipation
• Depression
• Irritability
• Memory loss
• Abnormal menstrual cycles
• Decreased libido

February and March continued pretty much as 2008. 

Constant 24/7 headaches with little to no relief. Towards the end of March I noted a marked increase in nausea and vomiting which I associated with the pain levels and a few new symptoms which seemed to be all part of the Hypothyroid issues. 

Rob's sister Amanda and her husband Steph welcomed the birth of my adorable nephew Ethan on March 7th. On March 30th, I did some mental math, added up some of the newer symptoms and while joking around with my sister Mandee about pregnancy decided a stop at the pharmacy was in order. Took the test the next morning, ironically, April 1st. Double line/positive results before the time to even check had passed. Followed up with family physician who confirmed that Rob and I were expecting! I was 38, soon to be 39 and honestly didn't think it was even possible.

Fertility and Hypothyroidism: If you know you have an under-active thyroid gland, talk to your doctor about having a baby. Your doctor will want to check your thyroid levels and make sure they are right before you start trying to conceive. If your levels are too low, you have hypothyroidism and may not be ovulating as you should. Taking the right dose of thyroxine, the hormone you lack, can restore your fertility.  

We told our families of the news in April and also contacted my Neurologist who immediately stopped all medication. The only medication I was allowed at this point was my thyroid medication, acetaminophen, Gravol and the occasional ibuprofen. This had the obvious effects of not being able to control the pain levels, resulting in increased nausea. I found myself spending more and more time in bed, between pain and weakness I wasn't able to do much. 

In May, I had my first visit with Dr. Giammarco in Hamilton ON. Her tentative diagnosis was New Daily Persistent Headache. A relatively new condition first described in 1986. However, further testing and diagnosis had to be put on hold until after the baby was born. Advised minimal use of over the counter analgesics and to get through the following months as best as I could, and to set up an appointment shortly after the baby was born. An MRI was scheduled for November as my due date was October 21st. The doctor did mention that with luck, pregnancy hormones could counteract and effectively cure the headaches and no further treatment would be required. 

New daily persistent headache (NDPH) is a chronic headache developing in a person who does not have a past history of headaches. The headache begins acutely and reaches its peak within 3 days. 

When I had Krystal, gender reveal wasn't a big thing, and considering this was Rob's first child, the anticipation of waiting for the delivery to find out if we had a son or daughter added to the excitement. Of course, our families (well his side more than mine), wanted to know, but we held firm, going with the tradition of selecting both a name for a boy and one for a girl. We decorated the baby's room with yellows and greens. 

Baby's Room all set up

Quilt belonging to Daddy when he was a baby

Things continued pretty normally (for me at least) until September. Rob had returned to school for his final year of Software Engineering Technology and I spent my days as best as I could. Resting, keeping to a darkened room, eating when I could. Mid September, I started experiencing contractions. Went into the hospital and ended up spending a couple days there hoping to hold off delivery for another month. I was given an injection to help mature the baby's lungs just in case, and sent home.

October 23, around 3 am, I called Rob home from an all night project session in order to head to the hospital. As my due date had passed, was told I wouldn't be leaving until the baby had arrived. Fortunately, Rob had some understanding professors who gave him (and his thankful team members) deadline extensions.

Getting some rest before the big event

I was in labour 16 hours, and was given an epidural which actually wore off partway through and had to be repeated. I didn't spend much time actually pushing, seems like once we got to that point things went pretty quick for me, and though my headache was along for the ride, at this point it was the least of my concerns.  

Still waiting

When the baby was born, we were happy to find out that we had another daughter! Rachel Josephine, born at 7:20 pm, weighing 7 lbs 11 oz, 20 inches long. I still remember Rob making the phone call to let his mother know she had a granddaughter, there was no way she was believing him, I had to confirm that she had her first female grandchild. (more below images)

Rachel Josephine Bray, Posing for her first photo shoot at 10 minutes old 

Meeting Mummy face to face finally!

Aunt Mandee and Big sister Krystal

Meeting Daddy!

Now that the baby had arrived, I was back on course for trying to determine the cause of my headaches (which didn't resolve with pregnancy) and hopefully get some relief. My MRI had been booked for November when I saw Dr. Giammarco back in May.  Contrast dye was used to ensure the best results.  After the results had been examined, I received a call advising me that I was being given yet another referral.  I was scheduled to go to the Eye Clinic at St Joe's Hospital in Hamilton in January.