My NF2 history

This post is for anyone new to NF2 or if they are unfamilar with when this all started.

When, where, and how did this all start?

Well it started as early as the winter of 1998 but I was completely unaware. I was working at an environmental lab and when I spoke to Harley over the phone I was having trouble understanding some of the things he said. I attributed it to a crappy portable phone but he thought I was starting to experience hearing loss. At the time I was also hearing crickets (tinnitus....ringing in the ears that sometims takes on different sounds) at night in an urban portion of Seattle where we resided. Since crickets are not uncommon in the midwest, I thought that what I was hearing was normal until I it occured one night during the pouring rain.

Then one day while I was watching tv in our apartment I noticed that the volume was too loud when I was laying on my left side. I turned it down and changed positions onto my right side. When I did this I had trouble hearing the tv and discovered that I had a definite hearing loss in my left ear. Shortly after, we bought our first home. I did not go to the doctor because I did not have insurance at the time but decided to get some in the fall of 1998.

The next winter (Jan-March 1999) I worked for the Seattle library downtown and began to notice more problems with my hearing. I often could not understand the secretary who sat across from me (maybe 10 feet away) and the director I worked for. It was terrible because the director would often give me instructions with her back turned towards me and while she was walking away. I had to scurry after her like a little animal with pen and paper, and ask her to repeat herself (which I could tell she did not appreciate).

Then came the day I had to interview for my own position (I had been hired as a temp but led to believe it would lead to permanant employment). It was terrible and humiliating! There were three women directors who interviewed me and asked questions. The room had very high ceilings and was surrounded by windows. Thus the acoustics were terrible! While one director asked questions the other two were fidgeting with the paper in front of them which contained the questions. This made it very difficult to hear the very soft spoken director. On 2 or 3 occasions I had to ask for a repeat of the question. When I did so, the women glared at each other like I lacked focus. This was really disheartening and there was nothing I could do. It was rather insulting as I find myself an intelligent person but was percieved as spacey in the interview.

After I was let go, I returned to my job with the WSDA in the spring where I was appreciated and respected by my colleagues. I have many good memories with them. The only problem is that it was seasonal and there was no position mobility due to the dependence on federal and state funding which was slowly dwindling.

In April of 1999 my hearing loss was becoming more noticeable to others and I had an audiogram done in May or June of 1999. My hearing was very abnormal and bad for someone my age. The audiologist refered me to an ENT who decided to order an MRI due to my previous bout with cancer.

The time frame is sketchy to me but sometime that summer I had a cat scan, spinal tap, and genetic blood test for NF2 (what the ENT theorized as the source of my hearing loss). I remember going to my 10 year class reunion over the 4th of July but I do not recall knowing about the tumors just yet. At the reunion we sat at round tables and I remember having difficulty hearing my classmates who sat across from me. Shortly after the reunion I had a confirmed diagnosis of NF2 in July 1999.

It was horrifying and scary because I thought my vanity was going to be the death of me! While going through chemotherapy for Hodgkin's Disease in fall 1992/winter 1993, I was extremely upset over the thought of going bald. I put up a really big stink about it. Therefore, the doctors allowed me to wear an ice cap on my head to freeze my hair folicles. I had wondered during treatments why I was the only one doing this. A college friend who had the same cancer as I, told me that her doctors did not want her to do wear ice on her head for fear that tumors would spread to the brain. I learned that with certain cancers such as leukemia and breast cancer it is not uncommon for tumors to migrate to the brain. However, I was assured that it was very uncommon for this to happen with my cancer.

In fact, NF2 and Hodgkin's disease are thought to be completely unrelated. NF2 is a mutation of the tumor suppressor gene and is present at the moment of conception. At the time of birth, my DNA had already determined for me that I would have NF2. Since the tumors are slow growing, it is usually not detected until the early 20s or later when the person undergoes a hearing loss. Many people are misdiagnosed if their primary care doctors are not familiar with the condition. I was lucky due to the fact that I had cancer. Therefore, it caused a doctor (ENT) to have suspicions of possible brain tumors and order an MRI. My oncologist totally overlooked this and thought it was no big deal. Imagine his surprise then they sent me back to him to confirm that the tumors were not malignant! The darn fool!

So I had a diagnosis....what then????
The next few years were difficult because I had to do a lot of research on my own. I was sent to a neurologist who we felt was not very proactive in my care and did not help in gathering information. He also did not tell me all my options. I had to look into it myself and inquire. Also, he always sounded so glum when I would see him.

I was told that Gamma Knife was the way to go to preserve my hearing because the tumors were small (6 mm and 8 mm). After researching GK, I discovered that some people had died due to brain swelling or stroke. That really scared me. Plus they had to screw this halo head frame into the skull that I read was not pleasant. Still, I consulted with the leading GK neurosurgeon here. We liked him as he seemed rather upfront and honest. He could not guarantee preserving my hearing and said that I could possibly become deaf within a year after the procedure. As a matter of fact, I was often told to prepare for deafness as that is often the natural progression of NF2.

At this point I was not even wearing hearing aides. The thought of going deaf was just horrible to me and when someone mentioned learning sign language I started crying. It was also painful to attend certain functions where I could not understand what was being said. First of all I would become very bored. The frustration of not knowing what was being said was a reminder of all the tumors in my head and the possible fate that I might succumb to later.

I started to wear a hearing aide in April of 2000. The hearing in my left ear was distorted and amplification was not going to help the problem. At first I hated it, and during the trial period I barely wore the aide. Sounds were different than I knew them and I did not like it (especially listening to music). Plus, the audiologist had programmed my digital aide too loudly so that the sound of shutting a car door was painful and the crunch of feet on gravel made my skin crawl. Once getting over that hump however, I grew to love wearing a hearing aide (most of the time) and could not imagine getting along without it (now it seems much easier and a relief which I will use a different post to explain at a later date).

Still what to do??? I had researched many things: surgery, FSR (fractionated stereotactic radiosurgery, GK (gamma knife), Peacock IMRT, the Linear Acclerator at Loma Linda, and I read of cyberknife being tested in Cleveland, Ohio. The choice of what, where, and when to get treatment was one of the most difficult decisions I have ever made in my life. The HMO was very reluctant to send me out of their providers for the alternative radiosugeries where I had read they had good results.

I got married in July of 2000. In the midst of going working and doing research, I was also trying to plan a wedding. I think it was effective in taking my mind off the situation as it became too overwhelming for me. I did not want to have treatment prior to the wedding or before our honeymoon. We traveled and dove in central america so I did not want to be immuno-compromised in any way or have a problem that would interfere with dive plans. We agreed to postponing treatment and using the "wait and watch" method until we got the wedding and honeymoon out of the way. We also, agreed that if the tumor had substantial growth then I would pursue treatment.

After we returned (we vacationed for 5 weeks in the fall of 2000), I tried to decide what to do. In July my MRI revealed very little growth (1-2 mm) over 6 months. It seemed like I could buy myself time to wait for better medical advances and published results. However, my hearing seemed to digress dignificantly even though the growth was minimal. At that time I was trying to decide what I would do with my life as the funding with my seasonal job seemed to be getting less each year. I took a career interest survey which revealed the same interests that I had while in high school when I was trying to decide what degree to pursue. The results were a teacher, scientist, or social worker. I decided that I would go back to school to pursue science teaching seeing it would allow me to dabble in all sciences instead of picking just one to focus on (my undergrad degree is in zoology).

After looking into the program requirements, a counselor and I determined that I would need to go back to school to aquire endorsement credits prior to applying and being accepted into the UW graduate program. I started taking classes in the Winter of 2001 and that is when I started to learn more about life as a HOH (hard of hearing) person and what accommodations were (such as assistive listening devices or FM systems).

I have to admit, going back to school helped me put things on the backburner. The HMO was still reluctant to allow me to get treated with FSR which I was most interested in. Then I discovered that they were getting it later that year (2001) and wanted me to wait. First of all, they were real nay sayers about FSR. Then once they found out they were getting the technology, they wanted me to get treated there (mind you I would be the first one and they had no experience with it). I said the heck with being somebody's guinee pig! I felt like I was never getting a straight story or answer from the HMO. First they wanted me to get GK. Then it was "surgery is the only way to go". Next they told me it was best to do nothing when I wanted FSR treatment. Then they told me to get FSR if I chose them. When I did not want to go there but somewhere else for FSR, they went back to the advise of "do nothing". What a crazy political world we live in! I have found that you must often be your own doctor and do the research yourself because sometimes it is hard to get an honest answer.

In February of 2001 I lost a great deal of hearing in my left ear even though growth of the tumor was very minimal. I think in winter of 2002 or 2003 is when I lost the remaining hearing in the left ear. This was actually ok because the sound in that ear was very distorted and almost devilish sounding. If I was lying on my right side, the voices from a tv sounded liked something from an exhorsist movie! So I was really happy to get rid of it! However, I had to learn to adjust to hearing in one ear only. Even though the sound in my left was distorted, it greatly assisted me in speech reading and my awareness of my surroundings. Similiar to wearing a hearing aide for the first time, I had to relearn how to hear again.

Oh yeah, in fall of 2002 I warmed up to the idea of learning sign language finally. Harley pointed out that it would be very useful while scuba diving and that we could have our own secret language. I took the class at HCC and did very well in it. However, I took a job in Seattle and an online class in the winter. I was not sure if I could swing taking the second part of ASL without being late for class due to taffic. Thus my study of sign language was put on hold for until I finished my graduate degree. Since the spring of 2004, I have taken 4 sign language classes.

I was accepted into the UW program and while going to school it seemed there was never an opportune time for treatment (plus I was told by my doctors that the "wait and watch" method was the best thing to do until the tumors got bigger). During this time, a fellow NF2 friend sent me information about cyberknife treatment. I had asked my neurologist about it briefly before and he told me that it was not an option because it was in clinical trials (actually it was for areas of the body OUTSIDE the brain. The FDA approved it for use on brain tumors in the late 90s....maybe 1999).

I really liked the philosophy of the doctors at Stanford Medical Center in California. The seemed sincere about patient "quality of life" and rather honest in their willingness to provide both opinions on surgery and radiation. Prior, I found it challenging to find an unbiased view on surgery and radiation. It seemed like doctors were swayed one way or another depending on what their expertise was. I think politics and funding have a lot to do with this. So it was refreshing to read about a facility and doctors who could go either way.

Over all this time my tumors did continue to grow 1-2 mm every 6 months. For a long time I really thought I was one of the lucky ones and would have some form of hearing. However, realistically (outside of my denial) when I looked at the facts I could predict the onset of deafness by the time I graduated or just after. I would look at the growth and compare each of my audiograms from every 6 months. At the rate I was losing hearing, I would be lucky to have any residual hearing left when I graduated.

This proved true as in the fall of 2003 when I was a student teacher, my speech discrimination in the right ear (only remaining hearing ear) was at 12% without the aide of speech (lip) reading and 28% with speech reading. What that means is that I was only understanding 12% of conversations when not looking at people and 28% understanding when I focused intently on their lips. By April of 2004 I had 0% speech discrimination without speech reading and 44% with speech reading. I could hear sounds but not understand any conversation unless looking at people's lips. Even this was hard because there are many factors that influence speech reading. For one, it helps if you know the person and their voice already. It also makes a HUGE difference if you are only listening to one person's voice or if you are in a noisy setting with lots of background noise.

Before, when I was still trying to live like a hearing person I would tell people I could read lips which only made things harder on myself. Why? Well most often hearing people have the misconception that a hearing aide or an FM system helps you to hear normally like you are restored to new. NOT TRUE! Often, just having those devices raised people's expectations of me and they would think I understood everything they said. Second, more often than not, I have found that hearing people think speech reading is some magic talent and if you can do it then you understand EVERYTHING that is being said even if the person does not move their lips much or has a large mustache.

I am amazed how many people (strangers) everywhere ask me if I can lip read even though I have stated that I am deaf and need information writen down. They just don't want to do it! Sometimes it takes me 3 times of repeating myself before someone writes something down! So unless I know the person well, I do not admit that I can lip read a little. It just totally complicates the matter and I can see how hearing people treat those who are deaf with disrespect.

One day I got so frustrated being asked this question that I instinctively shouted at a gas station attendant "NO! I CAN'T LIP READ! YOU NEED TO WRITE DOWN YOUR QUESTION OR STATEMENT!" LOL it was funny because he is the only person who actually paused and visually thought about it for a moment. In order to me to have answered the question I had to lip read him. After thinking for awhile he proceeded to write down for me that the car wash closed at 7 pm. I did not realize until after I left that I was probably shouting at him.

For the record, when I started graduate school I still utilized a handheld FM system for one on one commmunication but for lectures and class discussions I needed and utilized real time captioning. Real time captioning is when a person types verbatum what is being said and I read it off a computer screen on a laptop. The captioners use the same device as court reporters and some of them work as such. Because the documentation must be fast, they do not use a standard keyboard and instead use a keypad device that combines groups of letters to form words. They are amazing people and I do not know how they do it as I think my brain would get confused trying to think of letter combinations so quickly.

In March 2004 (the day of my graduate presentation) I received the disappointing results from my MRI. The right side tumor had suddenly grown rapidly. Instead of 1 mm, it grew 1 cm! If I wanted radiosurgery, I had no choice but to seek treatment that spring or summer. The tumor was 2.8 cm and tumors over 3 cm are typically viewed as too large for the radiosurgery.

Seeing as I had been researching all my options for years, I was pretty sure what I wanted so it was just a matter of consulting with the staff and getting coverage. I printed out the contact information and facilities I was where interested in getting treated, but my neurologist would not even look at the paper. Apparently he felt the best choice of action for me was surgery and he wanted me to meet with their surgeon who had no experience removing the tumors of NF2 patients as far as I knew.

Therefore, since I had double coverage now that I was under my husband's insurance, I decided that the HMO was no longer of use to me and that I would refer myself to the facility I thought would be best. I chose Stanford because they invented the device and seemed to have the most experience (most number of patients treated). In addition, they offered a doctor who did both radiosurgery and surgery (50/50). Thus I felt that I would get a pretty unbiased opinion on whether the tumor was too large and if I needed to get surgery instead.

In July of 2004 we went to Palo Alto, CA for cyberknife radiosurgery at Stanford University Medical Center. On July 14th, 15th, and 16th I had 3 half hour treatments at 20 gray. The radiation dose was large because the tumor was large. The team felt that in order to effectively have a shot at killing the tumor, they would have to use a high dose of radiation fractionated over 3 days. Given the tumor size, radiation dose, and that fact that I have NF2, we knew that preservation of my residual hearing was unlikely. The day after my last treatment I lost the remaining hearing. For AN patients I believe the chance of hearing preservation is 80%. With NF2 however, the chance of hearing preservation is 50% or less.

So there you have it! I will have to compose a different post from treatment up to present but for now this gives you the background. If there is something you feel is missing or that I should add please let me know and I will try my best to fill in the gap. I am working on compiling helpful links where you can learn more about NF2, hearing loss, and various treatments. However, seeing as the blog is so new to me, I have not figured out yet how to attach links to this page and to post photos. I am currently working on it.

Thanks for reading!