ja_mageia

Can you hear the silence?

I can. Always. I’m deafened since I was 17 due to Neurofibromatosis Type 2 and since then live in complete silence. What proved at first an enormous strike in life, I have now found ways to cope with. The first step was acceptance of this fate. Not that I have swept it aside and carry on living but rather I've found ways of lessening the pain which deafness brings. At first, there was the language part. If you want to enjoy your time with somebody else, you need a shared language to achieve this. For me and my family this meant learning sign language and I, over time, taught this to my closest friends, so they can find a way back to my real self. Of course this is a small world, but it’s better than being completely isolated, because no doubt this is what happens after ‘the strike’. What you thought to be friends, move away from you except very few close ones if you’re lucky. You lose the grip of the world you belong to and with it your identity until only your family is left. NF2 is so different in every individual though and many will have different perceptions and experience, but this is how I experienced it after becoming deaf.

So, suddenly without being part of either world; Nor the hearing one, neither the one of the deaf (from birth) community, I seemed and still seem a bit lost when it comes to where I belong. I would say I’m from in-between the two worlds and that sadly is quite a small margin. In public I find myself being avoided, it is almost impossible to meet new people. They all avoid me, but when I take that thought further I realise it’s not me directly, but rather communication with me. So, I keep telling myself it is their loss, I like myself. Of course though, it is mine, too. Due to the failing smalltalk (who thought this would be important?) I feel shut out from personalities, from information, from other human-beings in general. I always have to work hard and get to know many people, because there might be just be someone who will not follow this general pattern. After all I have interesting things to tell and in some way or another I can slip something maybe interesting about myself (not my disease etc.) in to provoke further interest. So, actually it is an on-going fight about re-integrating myself back into society or maybe it is just a straw of my past and true identity which I do not want let go.

Another basic of life, along with health, communication and a working social environment is that of the family. I do not know what I would do without them, just like anybody who hears says the same for his ears. Although they support me in any way possible, despite their helplessness, I know I would move on without them, because I must, because I have no other choice than trying making the best of it. Of course we all felt completely lost when we suddenly had to communicate by pen and paper after the first surgery. But after a few years we all began to understand the real meaning of the saying ‘time heals all wounds’. It is really hard to keep your spirits up if the society’s behaviour just pulls them down again. NF2 has really redefined true family love within mine and in some way or another I am even grateful for this fate. It enabled me to meet many nice people, to make new experiences, to learn what life is worth. I had one of the best childhood’s you could wish for and that can never be taken away. I have taken these words as my new motto: ‘Don’t cry because it’s over. Smile because it happened.’

I am a language student at Southampton University, which has, having grown up in Germany, always been my dream (before deafening). I thought to myself ‘why should the deafness stop me?’, so I just continued with it and it works well. Having me as a student means extra work for the tutors etc. and of course for myself, but I’m up for it. There is lots of support available, all you have to do is raise your hand. Generally I enjoy challenges and that’s what keeps me going. I see NF2 not as a disability itself (the feeling of being disadvantaged is created by society) but as a challenge. And I won’t give up before it is resolved. Many people complain about not knowing to do with their lives, having no essence. For me NF2 is a sense of life. It is certainly not boring and made me what I am today. And when I look around me, without being arrogant I believe I am a better person than some, who are just not aware of the value of life, who just lead the same boring life as always and being somewhat superficial. In Shantaram, the best book I have ever read, a sentence goes ‘If you can’t do anything about something, you have to accept that things could be worse and that you are completely helpless in the face of it’ but Voltaire said ‘the longer we dwell on misfortunes, the greater is their power to harm us.'

So yeah, guess I gotta keep moving.

My name is Miranda. I am 7. I have NF1. Having NF1 makes me feel unique. It makes me feel unique because not that many people have NF. I don’t know anyone else with NF. I like to twist on the monkey bars. I like to play goalie, defense and offense in soccer.  I am good at school. My favorite thing in school is math. I want to be a veterinarian when I grow up. I like to draw animals like hawks, peacocks, cats and horses. I have scoliosis that makes my back sore sometimes. I have lots of café au lait marks. We call them beauty marks. I have to get a needle poked through my arm for my MRI’s. I see lots of doctors so they can check my NF. People don’t know lots about NF. Other kids think they can catch it from me but they can’t. If I met another kid with NF, I would tell them that it gets better in life and you get used to having NF.

The above was written by my beautiful, compassionate and intelligent daughter. It is the first time she has expressed her feelings about her NF – in her own words. I asked questions and she answered them. She was diagnosed with a missense spontaneous mutation of NF1 when she was almost 5. We were stunned, confused and worried when the diagnosis came to us and had no idea what to expect. We firmly believe that knowledge is power. Thanks to CTF.org and Miranda’s battalion of doctors, we know that it is as much a part of the wonderful person who wrote the paragraph above as any other genetically determined trait. We know how lucky we are that her manifestations have been mild. We know that if we make it something to stress out about, she will stress out about it. If we treat it as a normal part of her life, she will to.

- Miranda's mom, Jessica

I was diagnosed with NF2 at just 6 months old.  I don’t remember a time when I didn’t know how to say Neurofibromatosis. My earliest memories are of my Grandmother and Uncle, completely wasted away, paralyzed and deaf. My Mom was afflicted as well and I remember sitting in ICU waiting rooms with my Dad for hours every time she would have yet another surgery, and then bringing her home and dealing with the devastating aftermath.  Over the years all three died before the age of 40.

I had my first spine surgery in 4^th grade, and my back never quite healed correctly. By high school I had a vestibular schwannoma in the left side of my brain. I had major surgery Junior year, and woke up completely deaf on that side. High school was a blur of cruel kids and bad decisions, by the time I graduated my VS had grown back, as well as another one on the right side.

At this point I began having MRIs every 6 months instead of once a year, and it seemed that every time there were more tumors. Over the years I have had clumps of tumors removed repeatedly down my entire spine, have had Gamma Knife radiation on 10 different brain tumors during 6 different procedures, and had Cyber Knife radiation on 6 spine tumors during 3 different procedures. I’ve had bone removed from my spine and cannot stand up straight any more, I have weakness in all of my extremities, no reflexes, double vision and am completely deaf.

In 2002 against all advice I gave birth to my son JT, who was soon diagnosed with NF2 as well. As complicated as the situation was, he is the best thing I have ever done in my life. I stopped waiting to die and started living for my son. I graduated college, got married, and had my daughter Mica, who is the first person in my family in 4 generations to not have NF2!

I don’t even remember how I first heard about the NF Endurance Team, I ran my first marathon with them in 2009 and have not slowed down since. Being a part of the team has been incredible. I am stronger both physically and spiritually, and I am now a proud Team Captain of the Long Beach NF Endurance Team for the 3^rd year running!

As happy as my life is today, NF2 will always threaten my horizon. I want to live to see my children graduate, I want to retire with my husband and grow old next to him, and I know I am not alone. There are a lot of us all waiting for that cure, and I will never give up hope that I will see it in my lifetime.

Ed Note: The Children's Tumor Foundation, in conjunction with the Mayo Clinic, is excited to be offering a Health Fair for individuals living with NF2 and Schwannomatosis at this year's NF Forum.  Click here for more information on this opportunity to receive an evaluation from the foremost experts in NF2 and schwannomatosis care and management.

To read more about Olivia and her quest to find a cure for NF, visit her blog here. 

My name is Sarah, I am twenty-six years old and live in British Columbia, Canada with my husband Hans and our dog and two cats.

I was diagnosed with NF2 when I was thirteen years old. At this time I was having a lot of trouble walking and I was rushed to Children's Hospital in Vancouver, BC where I was given an MRI. The doctors discovered my brain and spine were loaded with benign tumors, and this is how NF2 all began for me. I had emergency surgery to remove several tumors from my spine at this time. I was lucky that with time, patience, and physical therapy, I regained my ability to walk.

But my fight with NF2 was far from over…

Two years later when I was fifteen years old, my left vestibular schwannoma (hearing/balance nerve tumor) had grown large enough to leave me profoundly deaf in my left ear. I underwent stereotactic radiosurgery that year to attempt to stop further growth of the tumor.

In 2007 I underwent my second spinal surgery to remove multiple tumors which had grown large enough to compress my spine and be cause for concern.

In November, 2008, my right vestibular schwannoma had ballooned up and was compressing my brainstem. In the year prior, my hearing in that ear had gotten progressively worse. This surgery would leave me deaf, but there was some hope. I was sent to the House Ear Institute in Los Angeles for the surgery. The surgeons who operated on me there removed the tumor and also implanted a hearing implant similar to a cochlear implant called an auditory brainstem implant (ABI). The ABI gives me sound awareness and helps me to communicate with people when combined with lip reading. It does not mimic "normal hearing", but it is still a huge help and has been a major blessing.

Since 2008, I have been participating in a NF2 natural history study at the National Institutes of Health (NIH) in Bethesda, Maryland. The purpose of this study is for the researchers to learn more about NF2 in hopes of better treatment options. I go to Maryland every six months for testing which includes MRIs, vision testing, blood work, and exams/consultations with research doctors.

At my latest NIH trip in April, 2011, I had eight tumors surgically removed from my right arm. I unfortunately also learned that a tumor at the base of my skull near the brainstem is once again growing. It is in a very risky location and has already been operated on once. This summer I will have to undergo my third major spinal surgery to remove this dangerous tumor.

NF2 will be with me forever. It is a lifetime of waiting and watching, of MRI scans and of deciding when surgery is worth the risk of losing another function. I have too many tumors to count throughout my spine, in my brain, and in various other parts of my body. NF2 and the treatments/surgeries that I have undergone have left me deaf, with chronic back pain, drop foot, poor balance, nerve pain, vision loss, inability to open one eye, facial paralysis and many, many scars.

Despite all this, I try to live life to the fullest, one day at a time, and never give up hope that we will one day have a cure!