Prednisone and Dental Issues

More fun! I get to experience yet another issue with long term prednisone usage!
It's a great medication, it's supposed to help with so many different things, but sometimes I have to wonder if it's truly worth it!

I was out grocery shopping yesterday, something I haven't done as much as I should. I tend to let Rob take care of the groceries, especially if I'm having a bad day. While we were out I was feeling the typical low blood sugar issues so picked up a chocolate bar to split with Rachel. Just a simple bar, no nuts, nougat, not sticky, just the typical soft bar similar to a Mars or 3 Musketeers.  Take a bite and I started thinking right away that somehow nuts had gotten into the bar during manufacture. Nope, turns out it's a chunk out of one of my molars.  I think that puts the count up to about 3 teeth  now starting to crumble and break.

I did some searching and apparently it is a common issue among long term prednisone users. Prednisone causes calcium absorption issues and the teeth need calcium to maintain health.  There are a number of other drugs that can cause dental issues.  A short list (not inclusive) of those can be found at Medication and Dental Issues.  

The fun part for me, ever since I was little, I have had an innate fear of the dentist! I had a bad experience once, and that was it for me. I brush regularly, I go to the dentist when it is absolutely necessary..aka pain to the point of intolerance, but if I can avoid that trip I will ... every time!

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!