News

NF2 BioSolutions

3 weeks ago

We are proud to announce our newest NF2 BioSolutions Ambassador for New York, Chantelle Nobis. Both Chantelle's husband and daughter have NF2, and this is their story;

Amelia Nobis is an eight-year-old third grader in upstate New York who adores her little brother and sister, loves to read, play soccer, write, dance and sing. When Amelia was 4-years-old, her mom Chantelle noticed what looked like a lazy eye. Feeling that something was off with her daughter, Chantelle insisted that Amelia have a CT scan at the ER. The image on the scan was horrifying. Amelia had a golf ball-sized tumor in the left ventricle of her brain. She was then rushed to a local children’s hospital for emergency brain surgery that evening. And the news just got worse: the tumor was a grade 3 meningioma — a rare and malignant tumor. Almost overnight, the entire family’s life turned upside down. Doctors quickly diagnosed Amelia with neurofibromatosis type 2 (NF2) and since then, Amelia had a full resection of that tumor in 2015 and that part of her brain has remained stable.
In addition, Amelia also has a cataract in one eye, several spinal tumors - including a large cervical spine tumor - and cafe au lait marks.
Amelia has quarterly MRIs and is followed by a medical team that includes an oncologist, neurologist, audiologist and ophthalmologist and a Neurofibromatosis specialist in Boston. Amelia is on “active surveillance.”
The family also learned that Amelia’s dad Pete has NF2 and that the Bell's palsy his had was actually a misdiagnosis. Basically, when Pete was 31-years-old, he went to the ER for what he thought was Bell's palsy and the doctors ordered a MRI after a second case. The MRI discovered vestibular schwannomas, a non-cancerous tumor on the main nerve leading from the inner ear to the brain.
Since then, Pete underwent Gamma Knife radiosurgery in 2009 and had to have steroid injections into his ear drums. Pete has slight muscle weakness on one side of his face, and a slight hearing deficit on the side with the larger tumor along with balance issues. His diagnosis of NF2 was later confirmed with genetic testing and Pete currently sees a neurologist and also travels to Boston to see a specialist.

Since their diagnosis with NF2, Chantelle has made it her mission to find others living with the same disorder and fight for a cure. Chantelle truly believes that gene therapy is the answer to curing NF2, Amelia is the reason why she decided to join and support NF2 BioSolutions.

Chantelle looks forward to meeting and working with other families affected by NF2, so we can educate, spread awareness, and fundraise for research and a cure. “Alone we can do so little. Together we can do so much.”

Please contact Chantelle at Chantelle@NF2biosolutions.org if you are in the New York area. ...

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NF2 BioSolutions

4 weeks ago

The following is the story of Rachael Morris, who died fighting NF2 at the age of 32 on July 14, 2014. What has been shared here is Rachael's life story and her worldview in her own words.
What you are about to read is what she penned at the age of 27.

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I was 14, and it was the summer before my freshman year of high school. While away from home at camp, I started experiencing horrible lower back pain that extended down through my legs. It would initially come at night after being active all day. At the time, I could not really describe the pain. However, if I were asked to do so now, I'd describe it as a sharp nerve pain and deep burning in my muscles. This pain led to me spending 2 days in the camp infirmary sleeping, eating bananas, and sleeping some more. The camp doctor was just treating me as if my body was lacking potassium - this explains the banana binge - and said I had a “Charlie Horse”. I felt better after a lot of rest and was able to finish my camp session.

When I got home I visited my pediatrician. The pain would come and go, coming mostly after dance class or theatre rehearsals. My doctor just agreed with the diagnosis of the one at my summer camp and said that my body got tired and then said that I needed bananas.

However, it eventually it got worse and this led to many sleepless nights for the entire family and school days missed and I was ultimately referred to a neurosurgeon at Wake Forest Baptist Medical Center. MRI scans were done, and the neurosurgeon then made the diagnosis of Neurofibromatosis Type 2. I had a thumb size tumor putting pressure on my spine causing the pain and my first surgery was December 1996.
NF2 is a disorder characterized by the growth of benign tumors throughout the nervous system. The most common tumors associated with Neurofibromatosis Type 2 are called vestibular schwannomas. These growths develop along the nerve that carries information from the inner ear to the brain, also known as the auditory nerve. Tumors that occur on nerves in other areas of the brain or spinal cord are also commonly seen with this condition. I have had tumors treated throughout my entire body resulting in various impairments, some temporary and some permanent.
After my initial diagnosis, I saw my new doctor and had MRI scans every six months in Winston-Salem. When I would see my scans and hear all about this tumor disease I was like “HUH?”, “Neuro-what?”

I was told all about what could happen with this disease, but little did I know that this is what would happen! The warnings of possibly going deaf were out. I pretty much had the mentality that "this will never happen to me “. I really did not think much about it. I was a freshman in high school. There were more important things to ponder like whether the Backstreet Boys or N*SYNC gonna be #1 on TRL that day!
Reality hit when I was 20 & Deaf in my left ear due to Gamma Knife Therapy to treat a growing tumor in my brain. Of course, it was an adjustment and I still had good hearing in my right ear. I just had to make sure when I played the childhood game “telephone”, the person next to me whispered in my right ear, not the left - and it's a good thing that was not too often.
I was lucky enough to make it through all four years of high school & two years of college without too many NF2 interruptions

Unfortunately, an acoustic tumor on my right side began to grow when I was 23. My hearing was suffering and I had to get hearing aids. They were pink and very “in-style” (or as much as they could be). The aids did not last long because the tumor was growing quickly, also causing severe balance and dizziness. I was going to be Deaf whether or not I had the tumor removed. I needed TV & radio volume almost on full blast. People talking sounded like Charlie Browns teacher to me. All I heard was “Wha wha wha wha wha” and sound became a headache to me. This is where things really get juicy, complicated, and I turn 80 years old physically.
In February 2006 I underwent brain surgery at the House Ear Institute in Los Angeles, California. I had an acoustic neuroma removed and an Auditory Brainstem Implant (ABI) implanted on the left side of my head. I was in California. for 2 weeks before returning to recuperate at home.
Following that, in April and May 2006, I received radiation therapy to my brain for other inoperable lesions and went through physical therapy before having more surgery in July of the same year to repair a damaged facial nerve.
Then, in August I addressed the deteriorating hearing in my right ear.I was hoping it would hang on until I fulfilled my student teaching requirement set for the beginning of August 2006 but weeks prior to starting, my hearing was completely gone in both ears and I had to withdraw from school.
I then went through more radiation and physical therapy in November and December 2006 and that was when I also took a beginner sign language class along with my friends and family.
However, that was not the end for me. I returned to Los Angeles on Jan 9 2007, where I had my right vestibular schwannoma removed and another auditory brainstem implant put in. Again, I spent two weeks in California before returning home to recuperate.
This was followed by another round of sign language classes in the spring of 2007. I was in and out of the hospital during this period to treat my hydrocephalus, and at the time I had severe headaches, dry & unfocused eyes and I lost most function and feeling in my right hand. I also had extreme balance & weakness difficulties that resulted in being unable to walk. I had a walker. Life was difficult then!
Then in September 2007, I began receiving a chemotherapy drug called Tarceva. However, I discontinued the use of Tarceva in May 2008 because the side effects were getting worse and worse. I wanted to feel good for an eventful summer. Tumors appeared stable since initially beginning Tarceva and continue to be to now

My two biggest struggles now are that I am completely deaf and that I have severe balance problems. I have taken many sign courses but if you do not use it enough, you lose it. I am just not around anyone else who is fluent to the point where I can completely communicate through sign even though my friends & family all know some. I lip read mostly and finger-spell. Because of my balance, I have trouble walking. I can not run, jump, skip, & play like I use to and I have a walker that helps me a great deal. If I don’t have my walker, I need some other kind of support like a wall to lean on or an arm to hold onto. I struggle in crowded, congested, dark areas the most.
However, this has not stopped me from living my life; l drive and go to places by myself - I just have to prepare. In addition to these impairments, I also have difficulty using my right hand and difficulty speaking. Using my right hand is especially difficult because I am right handed. My handwriting has suffered some & I have had to learn to sign with my left. Both of my facial nerves have been damaged from tumor removal & radiation therapy. I can not move my lips and I use my hand a lot when I speak to move my lower lip so that people can understand me better and I have learned that just have to speak slower and more clearly. I also think I should mention that I see doctors in Los Angeles, Boston, Winston-Salem, & High Point. Basically, I see some kind of doctor at least once a week and I have to have periodic MRI scans done to check growths.

I am 27 now and would say my biggest challenges are still being deaf, followed by bad balance. I have Auditory Brainstem Implants (ABI’s) on both sides of my brain to assist with determining sound. ABI’s are complicated and do not work for everyone. Mine, I feel, are more of an annoyance than anything and as such I do not wear them.
Thinking about life, we really do take things for granted! Being late-Deafened is life changing, far different than being born Deaf! In many ways I feel totally different than the person I was before loosing my hearing. I never was a quiet, shy person! I loved being the center of attention (usually entertaining in some way) & being involved in constant conversation! Being Deaf is lonely! Over time, I have learned to deal with the emotions I face. It has gotten easier but will never be easy. I am still adjusting and all I can do is make the best of it. This is how it is going to be from now on.
I miss sound! I really miss music! I have always been one to break out randomly in song or dance. I still do so, around people I know of course. The song is always something from my past like a “New Kids on the Block” hit or a TV show theme song. It makes people laugh, hard! I love it! I joke and say “Oh, I’m just listening to my iPod, my implanted iPod”. My iPod is constantly in full swing! I have a friend that will send me text messages saying “Ray, what’s playing on your iPod today?”

Because of what NF2 has done to me, yes, my social life has suffered! I do not live like your typical girl in her 20’s. Group situations are the hardest as I feel left out of conversation most of the time. I am a great lip reader so I look around trying hard to lip read the conversation but end up just getting lost. It is just as annoying for me to interrupt by saying “What are y’all talking about” as it is for the speakers who have been interrupted! In a lot of situations I get very stressed out and I shut down. This happens with friends and family. It is so hard to see people hysterically laughing together. My reaction is always delayed and after the moment, it is no longer funny. It hurts deep but there is nothing anyone can do. Everyone really does their best to include me. We have ALL learned some signing but are not fluent as we do not live in a strong Deaf community. As such, I kind of feel like I am the only one. Most of my friends do not live in High Point. We all still text and email like crazy, I just don’t see them nearly enough. I can not do a lot of things my peers do. For example; concerts, parties, shopping, bars, etc. are all difficult for me. My entire body gets so tired. My stamina has improved a great deal over the years but still is not 100%.

I am currently not working and living at home with Mom, Dad, our dog Smokey and our cat Butter Frances. My world was put on hold my final year of college because of NF2 and I had to withdraw for brain surgery. After ups, downs, and loop-dee-loops, I was able to get back into school this past year. I graduated with my BS degree, majoring in Professions in Deafness & concentrating in Auditory-Oral Deaf Education from the University of NC at Greensboro (UNCG) in May’09. I am kind of lost and don’t know where to go now. When I began college, I was majoring in Auditory-Oral Deaf Education. I was unable to student teach so I am lacking my teaching license, resulting in the general degree. Soon, I will be starting in a volunteer position to get my feet wet and gain some experience. I am excited and I look forward to having somewhat of a schedule. Right now my weekdays consist of errands, household duties, working out, and a at least one doctor's appointment every week.

I get bummed, bored, and throw a pity party from time to time but I strongly believe in keeping my glass half full and staying optimistic. Life is a roller coaster, but still good! I do not like to be sad and dwell on my disabilities. I would rather celebrate my abilities, everything I have overcome, and who I am NOW. Life is too short and you have to take it one day at a time.
By sharing my story, I think I make a great mentor as motivating others who may or may not be struggling with something, is what I am most passionate about! I try so hard not to dwell on how Neurofibromatosis has changed my life. I have been through more than most people go through in a lifetime. It has been a complete turn around, especially in the past four years. I have had to adjust to a life very different than that of my peers & accept a lot of changes. I am very lucky though because I am blessed with wonderful friends & family whom I share a close bond of unconditional love, support, and strength with. We have stuck together and fought together. That’s my story and I’m stickin’ to it. ...

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