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!

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.

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.

2008

2008 - So many doctors, medicines, "lets try this" or "maybe it's that" all with no relief in sight!


May 2008, Final exam time quickly approaching and my head hurts... ok, more than hurts, I've had a headache for 3 or 4 days now. Losing track of just how long it's been there, I just know it's not going away.

I started keeping a bit of a journal, tracking the intensity of the pain, the ER visits, and the DR visits. I also tracked the suggested diagnosis and treatments at the time.

Started out with swollen neck glands, ear and headaches - Went to Urgent Care (walk in clinic) and was tested for Strep (negative), diagnosed with sinus infection and to take Advil Cold & Sinus formula.  About a week later,  back into the clinic with same symptoms.  This time, doctor isn't sure what's wrong, prescribed  Naproxen (nonsteroidal anti-inflammatory drugs (NSAIDs)). No improvements, some pain control using OTC (over the counter) acetaminophen with codeine.

A week later, same symptoms, however pain meds aren't touching it.  Went to local ER - Doctor's diagnosis was dehydration -  drink more water and stop medicating.  I drink a lot of water to begin with, at the time I was drinking anywhere from 5-8 500ml bottles a day, so dehydration definitely wasn't the problem. 

This pattern continued for another month, with additional symptoms such as severe pain over temple areas, inability to tolerate lights, movement or noise.  I also discovered that my scalp was extremely painful to touch, brushing my hair was torture.  I was rating my pain 10 on the pain scale. 

Wong-Baker FACES Pain Rating Scale

A trip to ER once again. I was given a pain cocktail consisting of Stemetil, Toradol and Benadryl

Stemetil belongs to a group of medicines called phenothiazines. It helps to correct chemical imbalances in the brain, allowing it to function correctly. These chemicals may also affect the parts of the brain which control nausea (feeling sick) and vomiting...used to treat nausea, vomiting and dizziness due to various causes, including migraine (severe headache). 

Toradol is in a group of drugs called nonsteroidal anti-inflammatory drugs (NSAIDs). It works by reducing hormones that cause inflammation and pain in the body. 

Benadryl is an antihistamine. Diphenhydramine blocks the effects of the naturally occurring chemical histamine in the body... used to treat sneezing; runny nose; itching, watery eyes; hives; rashes; itching; and other symptoms of allergies and the common cold...also used to suppress coughs, to treat motion sickness, to induce sleep, and to treat mild forms of Parkinson's disease. 

This cocktail got the pain down to around a 2 on the scale, tolerable though the headache continued to linger.  Within 12 hours, I was back in the ER with the headache back full force, including sharp stabbing sensations at the temples and the intolerance to light and movement worse than earlier that day.  Treated with an IV of Stemetil and Cogentin, no reduction in pain level (9/10) Toradol added, still no change.  Doctor sent me home with 2 Percocet tablets after giving me 2 in hospital.

Cogentin is an anticholinergic. It works by decreasing the effects of acetylcholine, a chemical in the brain. This results in decreased tremors or muscle stiffness.

Percocet contains a combination of acetaminophen and oxycodone. Oxycodone is an opioid pain medication. An opioid is sometimes called a narcotic. Acetaminophen is a less potent pain reliever that increases the effects of oxycodone. 

The next morning the headache was still intense and I followed up with the 2nd dosage of Percocets and the Naproxen that was prescribed previously. Symptoms didn't ease until later that afternoon when the headache finally returned to a lingering 2 on the scale. 

This pattern continued for the next couple of months. Different cocktails were tried with little to no relief in pain as the intensity of the pain continued to rise and fall.  As the pain got higher, I got to the point where I had nausea or vomiting all the time.

I'm sure within the first 5 ER visits, a "drugseeker" flag had been placed on my charts, then I encountered Dr. Kelly.  He listened, he actually heard that I wasn't there for narcotics, I just wanted relief.  He ordered my first CT scan after ordering the typical Stemetil, Toradol, Benadryl IV.  This lowered the pain to about 7/10, so he added more Stemetil and tried adding Decadron to the mix. 

Decadron is a brand name for dexamethasone, a prescription drug that works on the immune system to help reduce itching, swelling, and inflammation.It is used to treat a variety of health conditions, including allergies, arthritis, problems with blood or bone marrow, skin problems, and flare-ups of multiple sclerosis (MS).Dexamethasone is a corticosteroid, a class of steroid hormone.

This combo moved the pain scale back down to 2/10.  Dr. Kelly was happy with that result, provided a prescription for Percocets and sent me home with a referral to a Neurologist.  It should be noted that the CT performed was non-contrast, and did not show any significant findings.  

While at home, I did have some time of little to no pain, but did notice that my co-ordination seemed "off" and I was having difficulty with recalling how to spell simple names or even write some numbers, apparently a side effect of the Percocets.  This combined with the nausea and vomiting while taking Percocets was enough to determine I had a sensitivity to the drug and would prefer to not be given it again.  My pain levels did continue to vary and by this time I was waiting on some follow up appointments. 

In addition to the Neurology appointment, my family doctor noticed concerning levels in regards to Thyroid and Red Blood Counts, resulting in referrals to Endocrinology and Haematology.
By September, I had seen the Neurologist and had tried a course of high dose Prednisone (100mg/day x 5 days) and had started taking Amitriptyline. While on the 5 day course of Prednisone my right eye and the orbital area surrounding it began to swell excessively with a slight protrusion of my eyeball. I truly wish I had thought to take pictures, but I had a tendency to avoid the camera whenever possible. I was also sent for my first biopsy.  A temporal artery biopsy was performed to check for inflammation.  The results were negative, ruling out yet another possible diagnosis. One thing I've learned by this point, this is an illness of elimination. Most of the tests and treatments attempted have been in an effort to rule out one possible thing or another, hopefully resulting in the eventual diagnosis.

Prednisone is a corticosteroid. It prevents the release of substances in the body that cause inflammation. It also suppresses the immune system. Prednisone is used as an anti-inflammatory or an immunosuppressant medication.

Amitriptyline is a good initial choice for migraine prophylaxis. Although it is not licensed for migraine, amitriptyline is the only antidepressant with the consistent evidence supporting its effectiveness in migraines. Amitriptyline downregulates serotonin receptors, increases the levels of synaptic norepinephrine and enhances endogenous opioid receptor actions

The course of treatment through the Haematology department was a series of IV Iron infusions for Iron deficiency resulting in altered red blood cells including a higher than normal platelet count.

The Endocrinologist prescribed thyroid medication for Hypothyroidism, however none of these follow-up specialists had any results in regards to the treatment or easing of the headache.

By year end, the Neurologist determined that the source of my headaches was outside of his area of speciality and he would be referring me to a colleague who specialized specifically in headaches.  Dr. Giammarco of Hamilton.