Five recommendations for people with NF2
The following is a blog by Dr. Luiz Rodrigues . Originally posted in Portuguese on March 27, 2017. Additions and translation were provided by Dr. Mata-Machado from the NF Clinic at Amita Health/St. Alexis.
Question: I received an NF2 diagnosis today. I’ve never really had anything serious. I’m 34 years old and have a 2-year-old daughter. A few days after she was born I had a ringing in my ears (but couldn’t hear on my left side. But I’m not sure when that happened). I went to an ENT specialist, who barely examined me and told me that I was constipated only. I reported that I had not heard from the left ear for a while and the right was with that buzzing, but he did not seem concerned, as the buzzing ended. So I thought everything was okay. I went on with my life, without caring about it. About 3 weeks ago, I had a new buzz, and I listened very quietly to the only ear I had left. I had moved and decided to look for a new ENT. He asked me for a lot of tests, including a brain MRI, which came with the diagnosis of NF2. I would like your help and guidance.
Here is our advice:
First – NF2 is usually a slowly progressive disease, auditory tumors are ALWAYS benign and only grow about 1 mm per year on average and the symptoms (hearing loss, tinnitus, dizziness) are not always related to the the size of the tumors.
Second – We only perform surgical intervention when the benefit is greater than the risks. That is, we do not operate a tumor just because it is there and we do not prescribe chemotherapy or radiation therapy on NF2 tumors (see expert opinion below).
Third – The fact that your first symptoms appeared only after the age of 30 indicates that you probably have the least severe form of the disease, a slower progression, and fewer complications. The later the first symptom, the less severe the NF2.
Fourth – Your daughter’s chance of having inherited the NF2 from you is less than 50%, and may be as low as 25%, depending on some signs we find on the clinical examination. Heredity in NF2 is complex because in many cases, there is a mosaic form meaning only one part of your body has the mutation.
Fifth – We can now evaluate you and your daughter by performing a relatively simple, quick and safe ophthalmic examination, which is called optical coherence tomography (see HERE for more information on this test), which allows us to better classify the presence or absence of NF2 and how complex it is so that we can act more calmly in the future.
NF2 Tumor Treatments – Expert Opinion For healthcare professionals who read in English and want to know one of the latest scientific papers reviewing the treatment of tumors in NF2, I recommend clicking HERE. In this article we can extract some interesting observations.
1) Surgery and radiotherapy that physicians commonly use in vestibular schwannomas on people without NF2 do not work well in people with NF2. In fact, the side effects of radiotherapy in people with NF2 are sufficient to contraindicate this form of treatment.
2) Since the surgical treatment of vestibular schwannomas is also less efficient in people with NF2, It must be postponed until you are sure that the benefits will outweigh the risks (see HERE ).
3) Meningiomas should also only be treated with surgery in people with NF2 when they present important symptoms, and for them, radiation therapy is also contraindicated.
4) There are several drug studies for the purpose of reducing vestibular schwannomas in people with NF2, but there is no certainty that they should be indicated for the time being. In special cases bevacizumab has been tried, with encouraging results (see HERE ). And radiation therapy is also contraindicated for them.
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My husband has mosaic NF2. One UVS removed at 27 years. He is now 50. Scans have been good although he hasn’t had one for 5 years. Recently experiencing random phantasmia. 3 month wait for an MRI. I am worried about this symptom. Is it NF2 related? Is 3 months too long to wait for an MRI? Thank you for your help
We’re not physicians these are questions for your doctor. We hope you have one that has a good knowledge of NF Type 2. If your husband having any odd symptoms you should let his physician know and ask about moving an MRI up. Sometimes it’s just a matter of speaking up and making sure your physician is aware of all your concerns.
Hello. I am a troubled parent of three that their mother has severe nf2. Her family new of it and decided to withhold info to mates of their eight children who them all had large families unaware. Now I must build a huge foundation for all to handle all lifelong matters. I need also legal and business help for the situation any ideas for resources ? Thanks.
We’re sorry that we don’t have any means to help and suggest that you look for help locally and with your county.
I’m Mara from srilanka . i have nf2 diesese for last 2 year I am completely deaf .. I haven’t treatment yet
My tumor almost 5 cm
Give me advice ..
Mara, We’re not sure how medical care works in your country. Tumors from NF2 often take hearing. All we can advise is to keep looiking for the best doctor in your area unless you have the means to travel.
You need to update the article “Five recommendations for people with NF2” as some of the comments are misleading (I will just point out one). It is true that the non-NF2 tumour is different than the NF2 tumour but to say “In fact, the side effects of radiotherapy in people with NF2 are sufficient to contraindicate this form of treatment” is plainly wrong. I and many other NF2ers have had successful radiotherapy when given in the right situation. Oddly, surgery too has side effects and I had a stroke myself.
Thanks. We’ll have someone look at it. It was written by a NF doc. So it his opinion at the time – or perhaps his wording was bad.
I am Bharti from India i have nf2 diesese for last 10 year I am completely deaf I want me to hear as before is there any treatment for this iam 40
Bharti, You will have to talk to your doctors about options.
Just went to neurologist, and they said that they believe nf2 is the diagnosis, should I see a geneticist to confirm nf2? I’m 50yo male with no previous signs, my hearing just turned off, if that explains anything. So after 2 MRIs and the discovery of 5 tumors, all the size of a dime. I’m now nf2? What steps do you recommend?
Tony, You should check back with the neurologist to see if he is aware of the diagnostic criteria for NF2 and ask them to clarify how they are confirming NF2. You may not need genetic testing if you meet the clinical diagnostic criteria. With a diagnosis of NF2, we’d recommend that you try to see a doctor who is very experienced with the disorder. Where to you live? You can search for experienced docs at http://www.nfcollective.org.
Where are you located? I am in Kentucky. Do you work with the V.A.?
I have had NF2 for several years and currently have a very hard time with balance. I use a wheelchair or walker always. I have bilateral hearing loss with a 30% disability rating from the V.A.
I am 64.
Recently diagnosed with NF2. I’m 52 and have severe tinnitus and hearing loss in right ear. I have 4 children.
Wes, We’re sorry to hear about your struggles. Do you need someone to reach out to you? Where do you live? You can contact us at http://www.nfmidwest.org/contact.
I’m a newly diagnosed 33 year old female. I’m scared to death because recently I have had multiple symptoms.
Erica, Please email us at and let us know where you live.
My 10 year daughter has Nf2. Blind in her Right eye. Bilateral vestibular neuromas. Many tumors in her brain and spine. Any new treatments coming out and who would you want following her medically if it were your daughter? We live in Columbus, Ohio and are being followed here. We have met with Dr. Plotkin and Dr. Welling at Mass. General and are going to trying to get seen by CHOP in Pennsylvania. She is so young and there is a long road ahead any suggestions would be helpful. My husband is a physician and I am a nurse so we are very aware of her situation.
Thank you for your time.
Stephanie, it looks like you are well informed and that we don’t have a lot to add. We can put you in touch with others if you’d like. I know someone who has a daughter was diagnosed at a similar age and has a lot of experience.