A Neurofibromatosis story

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Over the years I have had a few people ask me why I haven't given up.  This really bugs me because there are other people who don't think it is fair that persons with disabilities (or major health problems) get 'special' accommodations, or they think we are lazy because we are not working, they just don't get it.  Most people in a similar position to me (who have a disability) want to work.  I say to these people, put the shoe or the other foot.  Walk a mile in my shoes and then let's see what you call fair. 
 
Birth to Grade 12 (Health)
 
I was born in Calgary in May of 1977.  We moved to New Brunswick when I was about 2½.  Before moving to NB, I was diagnosed with Neurofibromatosis.
 
_What is Neurofibromatosis?_
 
Neurofibromatosis encompasses a set of distinct genetic disorders that causes tumors to grow along various types of nerves.  NF can also affect the development of non-nervous tissues such as bones and skin. Neurofibromatosis causes tumors to grow anywhere on or in the body.  Neurofibromatosis effects about 1 in 3,500 people. 
 
I was diagnosed with NF Type 1 because I had a number of café-au-lait spots and because my head was large.  Apart from some developmental milestones like sitting up and walking I didn't have any NF related problems until the fall of 1988, I was in grade 6.
 
I can remember having lots of trouble trying to copy down spelling words from the blackboard.  I went to see an Ophthalmologist who did some tests (eye exam and visual field) and then sent me for a CT.  I remember the CT taking FOREVER (because they found something).  I had a tumour on my optic nerve.
 
On March 14, 1989 (yes I remember the date) I had neurosurgery.  Turns out they couldn't remove the mass, so they took a piece of it for a biopsy.  Had they taken it out, I would have been blind.  I think I spent about 10 days in hospital.  After going home and re-cooperating a bit I went through the process to get ready for radiation.  I have a lovely scar from ear to ear.
Getting reading for the radiation treatments included more doctor visits, having a mask made (which I actually still have, it's this big clunky plastic thing), and patiently laying on a table with the mask on while they painstakingly did measurements to make sure the radiation beams were being targeted in the correct location (my two temples and the middle of my forehead).  I had 30 days of radiation treatments.  I can remember that I was tired for the longest time.  It also took quite a while for all my hair (from the surgery) to grow back it so it was all the same length again.
 
The surgery cause some nerve damage, since nerves heal, my vision improved.  But the tumor in my optic chiasm was there for good.  It is called an optic nerve glioma. This is a diagnostic characteristic of NF.  The radiation caused a calcium crust to grow around the tumour.
 
I broke my arm in grade 9 falling on the ice at school.  We were told it was a bone cyst.  I have no idea whether this was related to NF or not because some people with NF do have bone problems.  I haven't had any since, so I think it was just a regular bone cyst.
 
Things were all good until I was in grade 10.  I started getting this horrible pains in the right side of my face.  It took them quite a while to figure out the cause.  At first they thought it was TMJ (temporomandibular joint).  Then they thought it was because of a cyst by my wisdom teeth, which I then had removed (and was bruised from my neck down to my chest).  Eventually I saw an Ear Nose Throat specialist who sent me to get an MRI.
 
The MRI revealed a mass on my cranial nerves.  On the 2nd day of grade 11 (September 1993) I had a plexiform neuroma removed from my cranial nerves.  The doctor compared a plexiform neuroma to a tree root.  I spent a few days in the hospital and a few more recovering at home before returning to school.  As a result of the surgery the cranial nerve which controls the voice box was severed on one side.
 
Everything was okay until I was in grade 12.  I started having pains again.  Another MRI was done and it was found that the tumour was back.  So, in the late summer of 1995 I had another surgery to remove this tumor.
 
Birth – Grade 12 (Education)
 
I was a socially awkward kid, not sure if this was because I was teased (harassed) all the time by my peers to different degrees.  I can still remember the day I came back to school in grade 6 after having my neurosurgery.  I was petrified to go into the classroom.  I remember wanting to wait outside the classroom until the teacher came until one of my classmates told the class she'd beat the crap out of them if they made fun of me (thank you Sonia).  It was also hard overhearing a classmate say the wished that something had gone wrong.  Kids can be very mean.  The teasing from the girls had basically stopped by junior high, although there were a few of them who still poked fun behind my back.  High school was a breeze compared to elementary and junior high.
 
Hindsight being 20/20 I wish I had had the courage to tell them to stop.  I remember being in grade 9 and it was Winter Carnival week.  They had a slave auction.  Since the girl who was supposed to have been a slave didn't come to school that day, I was 'next in line'.  So I had to be the slave of one of my tormentors.  I vaguely remember at one point he wanted me to tie his shoes.   I refused.  I went to the teacher and told her I wasn't doing it.  I don't think this type of event would be taking place today.  One of the things that bugs me is that the teachers saw them doing it, but never did/said anything.  Maybe since I just ignored them they thought it didn't bother me.  I think part of me being so introverted is due to the tormenting/harassing I went through in elementary.
 
I was a horribly messy kid.  Very disorganized.  Because I was visually impaired, I was assigned to an iterant teacher.  I remember the day 2 gentleman from APSEA came by the school to do an assessment.  I wasn't having a very good day, so I was relieved when I was 'pulled' out of the classroom.  In the Fall of grade 7, I was introduced to the lady who was a great mentor and friend.  Not sure what she did (or how she did it) but she helped me because a much neater, or organized individual.  One of her jobs was to try and inform the teachers on what kinds of accommodations would help me.
 
I wasn't the best student.  School work was not my forte, I was very middle of the road.  I decided to do a Bachelor of Arts, majoring in psychology.  I applied to a couple of universities in Ontario.  My parents were both U of T grads and they wanted their kids to GO AWAY to school.  I remember getting the rejection letter from U of T and being heart broken.  I had also applied to Glendon College, a bilingual campus which is part of York University.  It was the day of grade 12 graduation, I got a phone call from the guidance counselor telling me that I had been accepted to Glendon College, I was relieved to hear this.
 
Bachelor's Degree (Health)
 
So, about 2 weeks before I went off to Ontario, I had another surgery.  I think I was good for a little while before the pains came back again.  I don't remember the course of events exactly, but one of my trips home I had another MRI which revealed the tumor was back.  So, at the end of first year, they took out the tumour.  It was either this one, or the previous one that resulted in my 12th cranial nerve being severed on the right side, I wasn't able to stick my tongue out straight (not that ladies should be sticking out their tongues).
 
The pains came back almost immediately.  The pains were really bad.  I remember one night in February some idiot pulled or set off the fire alarm.  It was freezing out.  I was huddled in my winter coat shaking and in tears because I was in so much pain.  The cold was a trigger.  I remember when my Don got permission for me to go inside.  I remember puking up stomach acid all over the floor, that's how bad the pains could get.  I used to choke on my food a lot too (grossed out my brother).
 
My ENT here in Saint John didn't know what to do with me, so he referred me to a doctor in Toronto who he had studied under.  After meeting this doctor and having another MRI, we decided to wait until the school year was over to do the surgery.  I remember one of the girls in my house asking me one night if I was afraid to die, that did it, I was a mess. 
 
On May 2, 1997 (2 days before my 20th birthday) had had massive surgery at the Toronto General Hospital.  To get the tumour out they had to break my jaw (I have a titanium plate, dentist said it looks like a chainsaw), I had a trachea and I had a feeding tube up my nose.  I was supposed to have been in the hospital for about a week.  Well….things did not go as planned.  I was in the hospital for closer to 3½ weeks.  This was by far the WORST experience of my life. 
 
I had a student nurse call me a wimp when she was suctioning out my trachea.  I would have like to smack her upside the head, but I was in no shape to do such a thing.  I wasn't able to say anything because I couldn't talk.  I had to communicate with hand gestures and by writing messages.
 
One night I had an allergic reaction to heparin.  I can't remember if I my trachea was corked or not, but I let the nurses know I was bleeding from the wounds in my mouth, I was spitting out all kinds of blood into a spittoon.  She just gave me some gauze and told me not to spit.  She did not seem remotely concerned.  When my (now) brother in law came by that night, and saw what was going on, he told them that my sister had low platelets.  I don't know what the heck would have happened had he not been there.  They gave me some platelets and I seemed to be okay.  However, I recall on one or two occasions nurses coming by with very suspicious looking needles.  I was still bleeding from previous injection sites.  The reaction I had is called mobilization of the platelets. 
 
I think because of this allergic reaction my healing was dramatically slowed down.  As a result, I had lost the ability to swallow.  I remember one day going for a barium swallow test (it is really gross).  The speech pathologist administering the test yelled at me because I couldn't swallow the liquid or anything else.  When I ran into this individual in the fall on a follow up appointment, she basically called me fat and ugly (she was referring to how I looked before the surgery).
 
Because I wasn't able to swallow, and the nose feeding tube is only temporary, the decided to put tube in my stomach.  This was awful.  They didn't give me enough sedation, I FELT THE WHOLE THING.
 
I remember having to call my Aunt in Edmonton a few times to ask her what something met.  Some of the staff were not very good at explaining the procedure they were doing. 
 
After about 3 weeks in the hospital I was allowed to go home.  I was still being tube fed because I was unable to swallow.  In the beginning the doctor wondered why I couldn’t keep anything down.  Turns out I was on 3 times as much iron as I should have been.  Eventually I learned how to swallow again.  It took quite a while to do this without a lot of effort.  I still had to plug my nose when I drank otherwise the liquid would come out.  I had a small hole between my oral and nasal cavities.  My voice was a royal mess.  I was hyper nasal and my speech was very garbled.
 
I was well enough to return to Ontario for 3rd year.  I finished off my BA and graduated in the spring of 1999.

 
 
Bachelor's Degree (Education and Family
 
University was a rough time.  I had professors who were total idiots.  They university didn't really have good procedures in place to help students with special needs.  I remember getting to an exam to find out they didn't have one for me in large print.  I had test anxiety up the wazoo, so that didn't help.  Also, psychology profs liked multiple choice.  I loathe multiple choice.  I had a few professors who were really good.  They picked up on this fact and changed the format of the exam for me.
 
Spring of 2nd year was very stressful, the professors were on strike and I knew I was having major surgery at the end of term.  I had one professor nearly bite my head off when I asked her about writing an exam early (to accommodate my surgery).
 
Third and fourth year were equally as hard, my speech was at times very difficult to understand, I feel as though some of my professors discriminated against me because of the quality of my voice.  I was trying to continue in the specialized psychology program, but was denied because my marks were not high enough.  The university administration didn’t care that I was a student who probably worked twice as hard as most students and that I had just fought the battle of my life.  I got my B.A in the Spring of 1999.
 
Not that I can't make friends, I'm just not an extroverted person.  I had a few friends at university who helped me through the tough times.  My parents have been there, but they've never really gotten it.  I was very close with my Aunt in Edmonton.  She and I would provide support to one another, she had MS.  Since my parents hadn't ever really talked to me about what I was going through, I had to find my own support network, and joined the Neurofibromatosis Society of Ontario.
 
1999-2004 (Employment)
 
In the summer of 1999 I noticed my friend, who is also visually impaired, with a white cane.  I watched her use it and thought that it might be very helpful for me to get one for myself.  I wish I had discovered this years ago.  I’m not sure why it wasn’t ever recommended.  Having a white cane in Toronto would have been very helpful.  I remember one day being in the subway station, having to go down a set of concrete stairs which were poorly lit, and holding on for dear life.  I knew that the rise and run on stairs are supposed to be equal.  Trying to get my brain to send this message to my eyes (and vice versa) just wasn’t happening.
 
Finding my first job was a bit of a challenge.  Because I am a person with a disability I had an employment counselor.  My voice was still a mess.  I applied to all kinds of places, wrote lots of letters, had a few interviews.  On one of these interviews my employment counselor accompanied me.  The person doing interview later made a comment to her about why I was not hired.  'Their clients might think I was drunk'.  This same person was worried that I might hurt my voice.
 
I eventually got a job working as a Psychometrist (administering psychological questionnaires) in a workers' rehabilitation centre.  I loved this job.  I had always thought I wanted to work with kids, but this changed my mind.  I was there for about a year when I was let go for lack of work.  I was called back a few times to finish off a project I had initiated.  Since I was unemployed, I spent a bunch of time volunteering and looking for work.
 
1999-2004 (Health)
 
Sometime in 1999 I started having pains in my face again.  When an MRI was done, I was told the tumour had returned for the 5th time.  It was decided that rather than take it out, we'd we wait and see what happened.
 
For pain, I tried acupuncture for a little while, that didn't work, then I tried Neurontin, that didn't work, then I tried Lyrica, that didn't work.  I have had good days and not so good days, pain wise, I lived with it in varying degrees for over 20 years, so it is basically part of my life. 
 
In 2003, I investigated improving my voice.  I had a pharyngealplasty done to repair the hole between my oral and nasal cavities, that made a huge difference in the quality of my voice.  Not only was my speech MUCH better, I didn't have to plug my nose any time I drank liquids.  I can't remember exactly when but I also had a very minor procedure to insert a silicon block in my neck (which pushed over my paralyzed vocal cord).
 
Master's in Adult Education
 
In 2003, my Aunt who lived in Edmonton was diagnosed with cancer.  She and I had been very close.  She had MS, so she understood some of the struggles I was going through.  Watching the only person in my family who I could truly connect with die of cancer was very hard.
 
On one of my visits to Edmonton, we started cleaning out my Aunts apartment, I learned that she had a Master's in Adult Education, and thought this is something I might investigate.
 
I did some investigating to discover what I needed to do get into the M.ED program at the University of New Brunswick.  Before I could start my M.ED I had to prove to the university that I was able to do it.  My marks from my B.A weren’t so great.  I had done horribly on multiple choice tests and wanted to figure out why.
 
In order to get accommodations at the university I was attending, I had to have a letter from a Psychologist stating what my needs were.  The results of this assessment revealed that I did much better on recall than recognition and that for someone with my level of education and experience I read and wrote much more slowly than would be expected.
 
In the fall of 2004 I started doing some upgrading.  I found this a much easier time than my previous university experience.  The policies at this institution required students needing accommodations and their instructors to sit down and come up with a solution.  The format of my exams were changed and I was allowed to write my exam in a private room.
 
I was accepted into the M.ED program and started my M.ED in Adult Education in the fall of 2005.  This was one of the easiest learning experiences I have ever had.  I had one of those ah ha moments when I finally started understanding how I learn.  I finished my M. Ed in the Spring of 2007.
 
Spring of 2007 (Health)
 
In the December of 2006 I went on a cruise with my sister and her family.  One day my brother-in-law noticed a bunch of bruises on my legs.  Since I tended to bruise really easily I thought nothing of it.  Around Christmas time the bruises were still there and more were appearing.  I was on Lyrica at the time so I did a Google search to see if there was anything on bruising and Lyrica.  What I read suggested I contact my primary care physician. 
 
On the first Monday in January of 2007 I went to see my GP who ordered a battery of blood work.  That afternoon I got a phone call asking me to get to the hospital ASAP because there was something wrong with my bloodwork…oh great, now what? 
 
I went to the ER and eventually saw a hematologist.  I was told I had TTP.  My platelet count was at 11, it should be at least 150.  I was told I was the sickest person in the hospital.  I was really lucky that it was caught before any real damage was done. 
 
_What is TTP?_
 
Thrombotic Thrombocytopenic Purpura is a rare autoimmune blood disorder that is considered a true medical emergency. TTP is diagnosed at a rate of 3-4 in 1 million people per year. Potentially fatal complications can result from internal blood clotting with damage to critical organs such as the brain, heart and kidneys.
 
 
After waiting in the ER for hours, I was finally admitted.  The next day a central line was inserted so they could do plasmapheresis.  When they brought me down to the procedure room and told me what they were going to do, I was in tears.  I explained what happened at the TGH, they assured me that that would not happen.  I had a nasty reaction to the sedative they gave me and was very nauseous.  Anesthetic often makes me very nauseous.  That night I got up to go to the bathroom, and was still very nauseous, I was retching so violently that the nurses had to intervene because they were afraid I'd dislodge the tube.  They basically put several pieces of huge bandage over the tube so it wouldn't come out. 
 
For 5 days I had plasmapheresis, basically an oil change.  They took out all my blood, separated it in this machine, put back the good part along with donor plasma.  The side effects were awful.  My senses were all out of whack, I was seeing spots, and lines would move, I was hearing things, food tasted absolutely gross, I had a constant urge to go to the bathroom but never could and I didn't sleep the whole time I was in the hospital.  Luckily the first round worked and I was allowed to go home.
 
I had to have my blood check every day for about 2 weeks just to make sure, than it was once a week, and then once a month.  I now have it tested once a year.  Every time I see unexplained bruising I start freaking out.  I don't want a repeat of that.
 
The blood work I had done also revealed that I had an underactive thyroid.  One day I felt I lump under my chin so my GP ordered an ultra sound of my thyroid.  Turns out I have a very lumpy goiter.  I have this scanned every few year.  I also stopped taking Lyrica because it wasn’t working.
 
Finding a Job
 
After doing a bunch of different things including: tutoring, proofreading/providing input on software, writing manuals, designing learning curriculum geared to adults with learning challenges, and lots of volunteering, I am now working part time for a family owned business. 
 
Present Health
 
Although the tumor in my optic nerve has been stable for over 20 years, we think that the radiation is starting to show its effects.  My peripheral vision has slowly been getting worse.  In June of 2014 I saw a specialist in Halifax who ran a battery of tests.  My vision has changed so gradually that I don't really notice any changes.
 
I have been having MRI’s every year since 2000.  I have gone back to Ontario to see an NF specialist who has basically told me he wanted my life to be down the toilet before he felt it warranted to do anything.  I overhead one doctor tell another on the phone that nobody wants to touch it.  This same doctor told me that taking it out is going to be much worse than the symptoms it might be causing.  I’m in no hurry to go through another massive surgery, which could have serious complications.  I have pain, but it isn’t anywhere near as bad as it has been.  I can go for weeks with no bad days or I might have a couple bad days in a row.
 
I keep track of my symptoms so I stay on top of things.  I have had theses 'strange' episodes including one where I was smelling burnt toast for a while.
 

This is my story thus far.  As I said at the beginning of this story, people have asked me why I haven't given up.  I try and take one day at a time and cross each bridge as I come to it.  It is all I can do.  My Dad told me a long time ago to always look at the glass half full.  I read a quote someplace that I really like…’it is better to live for today because yesterday is already gone and tomorrow may never come’.
 

Po