2015 - Final Catching up Post

Wow! Quite the journey so far, and it's still not complete!

This is a big post, especially when you consider it contains happenings from January to May 2015. My posts after this will be more frequent, typically recording events as they occur.

The new year started out with the usual pattern for me. January was rather uneventful. I had the usual headaches but no swooshing noises.

February once again had me heading to Hamilton for follow up appointments with the Bariatric Clinic (another 10 pounds gone) and a routine MRI. While at my bariatric appointment, we discussed further the addition of a daily injection to my routine. Victoza was the medication in question, currently a diabetic drug that is in process for approval in weight loss purposes.

Victoza: Liraglutide, an injection treatment already approved for diabetes treatment, can help reduce body weight in people with obesity when used at a higher dose than is usually used in diabetes. Health Day Article

I was provided with a prescription for a 1 month supply with the instructions to inject 0.6mg daily until my next visit to the clinic. The first 48 hours following my first injection were not pleasant at all, I couldn't move without becoming sick. The nausea and vomiting gradually eased and I was able to consider food.  It's no wonder this medication helps with weight loss, if everyone becomes as sick as I did while taking it, their ability to eat becomes greatly reduced.  For the first week, the most I could tolerate was simple food, toasted English muffin, chicken noodle soup, and salted crackers.

Valentine's Day - Roses and dinner from Robert! We headed to one of our favourite places, The Golf Steakhouse in Kitchener, while Rachel spent a couple hours visiting her Grandma and Aunt. I had my usual prime rib dinner and found myself barely able to eat it. Of course, the meal includes an amazing salad bar and soup so I ended up taking the main portion of my meal home for the next day. First time I've ever walked out of that restaurant with a doggie bag!

March 5 was my next follow up with the bariatric clinic. We discussed my tolerance of Victoza and I was advised that I seemed to be doing better than many on the drug. My weight loss was continuing at an acceptable level and I was provided with a 3 month prescription with the instructions to continue the dosage of 1.2 for a month then to increase to 1.8mg.

March 9th was my follow up with Dr. Rodriguez.  My eyesight has remained stable.

I met with Dr. Khalidi on March 24.  As we discussed my current medical situation, it was decided that a sleep study would be a good course of action. My symptoms of headache, nausea, and vomiting were ongoing, I advised him of my use of Victoza and my ongoing weight loss.

I held off increasing my dose of Victoza until after my sleep study which was Friday April 10th. It wasn't too bad considering I typically have a lot of difficulty in falling asleep. I was of course advised to not consume any caffeine after 6pm.  I arrived at the hospital where the study was to be done for 8pm. By about 8:30 I was wired up and left to relax.  I had my e-reader with me, so spent the evening reading and waiting for bed time. I had a control pad that was placed on the bed beside me when I was ready to sleep. I settled in for the night about 11:30 or so as they had to get the study started. Falling asleep was my usual pattern, I had trouble, so I continued to read until I eventually drifted off. At one point during the night, I was awakened when the nurse came in to turn on the room fan, apparently I was breaking out into night sweats. I haven't received my results yet, expect they will be available at my next appointment with Dr. Khalidi.

All wired up for sleep study

Typically, I was making medication changes on Sunday, this time, we decided I would increase my dose on Saturday so hopefully the side effects would have tapered off in time for me to walk Rachel to and from school on Monday. Well, that didn't happen, increasing the dosage once more seemed to exceed my limits. The side effect of vomiting and nausea went far beyond the day or 2 I had anticipated.

I could do nothing more than lay down, even that resulted in getting sick if I happened to roll over or move too quickly. Rob ended up having to leave work, drop Rachel off with me at home and head back to work. I couldn't summon the strength to walk across the street to her school.  Other side effects continued to occur as well, especially heart burn and indigestion. I couldn't sleep, couldn't eat, couldn't do much of anything.

I did achieve one goal though! April 11th I weighed myself and discovered that I had reached my first major weight loss milestone.  100 pounds gone from my worst weight to my current weight.

Comparison of highest weight to current

April 29th, my headaches were bad, but I knew it wasn't increased pressure, I didn't have any swooshing noises in my head and rather than the outward pressure from being too full, my head felt as it if were squeezed in a vice, the only relief I got from the headache was to resort to my heat packs and stay laying down. Sitting upright for too long just made things worse and increased the nausea and vomiting exponentially. I tried stopping ibuprofen knowing it can increase stomach issues, but that made the pain even more intolerable. Antacids did nothing to control the burning and I was having difficult keeping even water down which had the potential for dehydration adding onto the current problems.

Given my experiences in 2008 when everything started up, I had come to distrust finding satisfactory results from a trip to the emergency department. Robert was getting concerned because I was in worse shape than normal, so he made a call to Dr. Klurfan. When he received no answer and discovered her voice mail was full, we decided waiting was out of the question. It was time to visit the ER. The first decision we faced was if we should go to the local hospital, or make the drive to Hamilton.

Hamilton ended up being the choice we made.  My records from the past 3 years would be readily available through Hamilton Health Sciences and provide a history for the doctors, a resource the local hospital wouldn't have, virtually putting me back at square one. I wasn't too long waiting to get into the emergency room, they were busy and low on bed space but they were able to find an overflow spot for me.  It wasn't long before I saw the ER doctor and she got me started on an IV for pain with Ondansetron added for the nausea. A neurology consult was brought in and after reviewing my history the decision was made to admit me in order to determine if my shunts were failing or if there was something else going on.

I spent the night in the ER then was moved to the Clinical Neurosurgical Unit. On the 30th, doctors decided that we could be looking at a shunt revision being required and I was made NPO in anticipation of surgery. I had elevated white blood counts, reflective of possible infection and a specialist from Infectious Diseases was brought in for consultation.

NPO: Nil per os (alternatively nihil/non/nulla per os) (npo or NPO) is a medical instruction meaning to withhold oral food and fluids from a patient for various reasons. It is a Latin phrase whose usual English expansion is nothing by mouth (most literally, "nothing through the mouth"). Variants include nil by mouth (NBM).

An infectious disease (ID) specialist is a doctor of internal medicine (or, in some cases, pediatrics) who is qualified as an expert in the diagnosis and treatment of infectious diseases. After seven or more years of medical school and postgraduate training, ID specialists complete two to three years of additional training in infectious diseases.

ID specialists have expertise in infections of the sinuses, heart, brain, lungs, urinary tract, bowel, bones and pelvic organs. Their extensive training focuses on all kinds of infections, including those caused by bacteria, viruses, fungi and parasites.

Dilaudid (hydromorphone) is an opioid pain medication. An opioid is sometimes called a narcotic. Dilaudid is used to treat moderate to severe pain.

Abdominal ultrasound and shunt x-ray series were ordered to verify no physical or technical issues were resulting from the drainage catheter in my stomach. I continued to receive Dilaudid and was placed on an alternating schedule of Ondansetron and Gravol to control the nausea and vomiting.

For the first 3 days I was NPO for most of the day as there was still a possibility of a shunt revision being done. Ironically, the NPO orders were reversed after dinner had been served on the ward. By this time I was finding the thought of food a little more appealing thanks to the anti-nausea medication.

May 2nd I saw one of the neurology residents and was advised that an LP would be done later that day. I didn't actually get the LP done until the next morning. The doctor came in around 3 am to say she was having a busy night and would be in to see me in the morning to do the spinal tap.

The tap on May 3rd didn't go well for me. It was performed at the bedside by the resident.  She had difficulty getting the catheter into the proper space and it took 3 attempts including having another resident come in to assist.

Shortly after 10am I sent a message to Rob, letting him know that my LP came back with a very low pressure reading (13, which agreed with the symptoms that prompted me to visit the ER in the first place) and the doctors had decided to tie off the left side shunt and send me home.

That afternoon I was moved into the procedure room to have my shunt tied. I did have some concern about the success of the procedure based on my experience with having the right side tied off only to be reversed a week later. Previously, the tie off procedure was quick and relatively painless, I expected the same this time. I quickly found out I was wrong!

After freezing, an incision was made at my collar bone on the left side and the resident proceeded to search for the tubing in order to place a couple sutures around it. This took longer than he anticipated and I could feel the freezing wearing off and could feel tearing sensations as he probed for the tube. Freezing was re-applied 4 times before the resident decided that he would close off the incision and start again. I recall him going through 4 changes of gowns and gloves, something I don't believe to be typical during a procedure, but I guess he felt it was necessary, or perhaps just needed to cool down before proceeding.  Additional pain and the resulting nausea required more medication added to my IV. By this point, I had reached my limitations, I was not tolerating the procedure well and found myself shaking and in tears, an unusual reaction for me given what I had learned to put up with over the past 7 years.

May 3rd
Incision at collarbone for shunt procedure

A second site was prepared and a small incision made on my neck below my ear. The tubing was located relatively quickly this time, but I experienced a lot of pain that seemed to be nerve related. When the tubing was shifted to allow for the tie to be placed I felt burning sensations along the top of my ear and felt as though my hair was being pulled out by the roots. After a few minutes of this, the ties were placed and the resident closed the incision with steri-strips.

 We celebrated Rob's birthday with my return home. My sister, Mandee stayed with us for a few days to help out with Rachel and look after meals while I was recovering.

I received a call booking a CT scan and follow up with the Ambulatory Neuroscience clinic for June 18th.

Mother's Day - Rachel presented me with a project she had made at school. It really hits home just how my daughter perceives my illness, and that Rachel has never known me to be 100% well.  I have been lucky, she really does her best to help out around the house and helps care for me when I'm having a bad day.

Front

Rear of Rachel's Mother's Day gift
Thanks Krystal for the added coffee inspired decoration ;-)

My headache eased a bit shortly after having the shunt tied but I soon found myself experiencing occasional swooshing noises. I continued to experience nausea and vomiting and the resulting weaknesses right up until May 15th. The previous day I made the decision to stop taking Victoza to see if it was adding to my symptoms.

On the 15th I had my sutures removed from my incision. On the 16th we had a gathering of family in a combination celebration of Rob's Birthday, Mother's Day, and my upcoming birthday.  It was also a day for my mother and Krystal to relax and enjoy the quite time following a busy tax season. I have to confess that perhaps once again Rob was right, and the Victoza was contributing to the nausea. Within 2 days of stopping the injection, my nausea had eased drastically and I was able to enjoy a prime rib dinner.

May 15
Sutures out

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!

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.

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!

In Memory ~ Coco (1996- 2010)

2010 started out poorly for me not only medically but also emotionally. 

Coco (sitting up) and his sister Mokka

Coco was born August 14, 1996 and I was there to help him into the world as I also owned his mother Bubbles.  At 5 weeks of age, Coco was involved in an accident while playing with his father, resulting in an artificial cleft palate. This prevented him from being sold to his potential owners and instead he remained with my family his entire life which actually was a number of years longer than originally anticipated.

He was operated on at 9 months after getting his weight over 4lbs.  The vet attempted a closure of the palate, but by the time the surgery was performed, he had already learned to keep the opening between  his palate and his nose cleared. This meant that he also managed to clear the sutures that the vet had placed.  The end result was a reduction in the size, but a permanent hole remained in the roof of his mouth.

His disabilities didn't stop him though. Coco was a saucy and demanding little pup, earning himself the nickname "Little $hit".  If his food or water dish were empty and he wanted them filled, he would pick up his dish and toss it around until you filled it.  If he wanted attention and wasn't getting it immediately, he would make all kinds of noises, chattering and whining at you until you picked him up or made room on your lap.

Coco liked to steal things!
The soother belonged to a relatives son, picture taken in 1998

Coco and Rachel

When Rachel was born, he became her protector. If she was on a blanket on the floor, he was right there next to her. At times though, he did display a bit of jealousy. I remember a few times when Rachel would be snuggled up next to me and he would be standing on her pawing at the blankets trying to get in with us.  It was a good thing he never weighed over 5 lbs with the exception of preparing for his surgery.

In the picture here, he's actually a lot of fur, as he got older, it became more difficult to keep him trimmed down as it created additional stress on his breathing.  We believe the vibrations of the clippers created some problems with vibration in his palate.

On the morning of January 11th, 2010 we woke up to what we believed was Coco in the midst of a seizure.  Once again, we were faced with the difficult decision of having to say good-bye to a well loved family member.  He was also 14 years old.  Another loved boy gone but never forgotten.