Case Study # 17, Lori Cramton Tells us About her Vagal Schwannoma (Tenth Cranial Nerve) Removal

Hello everyone!  Today I am pleased to offer our 17th case study - this is one that we have been seeking for quite some time, the story of someone who has gone through the process of having a Vagal Schwannoma removed.  Friend of the page Lori Cramton was kind enough to tell us her story, which is also important because she had her removal done by a very promising method called Intracapsular Enucleation, which is a style of removal in which the Schwannoma is removed by excising it from its own enclosing capsule.   Lori also had her surgery done by the very well renowned Dr. Netterville of Vanderbilt University Medical center in Tennessee - and Dr. Netterville has gained a reputation as one of the best in treating this kind of Schwannoma.  We thank Lori for her assistance, and we wish her the best on her continued recovery!  (PS. I have heard her sing, and her voice sounds amazing - even after surgery - which is quite a wonderful thing since Vagal Schwannomas are known for ravaging a person's voice, and this example gives promise to this kind of treatment.)
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1)  How old were you at the time of your diagnosis?

I was 38 when the tumor was found.  

2)  Do you have any history of Schwannomas in your family?

None that I am aware of.

3)  Where was your Schwannoma located?  And what were the symptoms that lead to your diagnosis?

Schwannoma on the 10th Cranial Nerve (Vagal Nerve) at the base of my skull/neck, pressing against the jugular vein on the left side (under the ear close to jawline).

I was a "sickly" child with a history of severe migraines from the age of 9 (& still have them on a severe level).  While in college I had a very bad case of mono.  After having mono I began to get sinus infections quite often.  The infections got progressively worse and I was always at the doctor's office for either migraine or sinus stuff.  At 38, after noticing for like a year, that it appeared than one of my "lymph nodes" stayed swollen so I mentioned it to the doctor and she stated it was concerning her too.  She sent me to an ENT who poked and prodded then sat down and said "I don't think it's a lymph node".  By the end of the next day I had an MRI and he personally called to tell me the results.  That it was a Vagal Schwannoma.  He said he would like to remove it and informed me of the long term consequences/side effects.  I was in shock so I agreed to talk to the nurse to schedule the surgery.  After hanging up and talking to my dad he told me that we were going to find another doctor and get a second opinion.  I ended up going to the Chief  Surgeon of Otolaryngology at Duke.  This  doctor stated he would NOT remove it b/c of the damage it could do and suggested we had enough time to watch and wait as they are not normally malignant.  HE said "if I do surgery I WILL hurt you"  So I watched and waited for about 4 years but it started to bother me...pain in my neck...as well as ongoing dizziness, blood pressure and stomach issues (though at the time I didn't know the connection).  It was extremely uncomfortable and although it ended up not growing very fast it started to be visible.  I started to do research online and found a lady named Rhonda Edwards who had a webpage ( http://www.vagalschwannoma.com/ ) and from there I joined the Facebook group she had started and that's how I found Dr. Netterville!  

4)   Could you describe, in whatever detail possible, what kind of surgical treatment was performed on your Schwannoma, and if you would consider it successful? (Or describe what you may know about the surgery that may yet be performed, if you haven't been operated on yet)

I had Intracapsular Enucleation done to remove the tumor by Dr. James Netterville at Vanderbilt Medical Center (Skull-Based Surgery Center).  My surgery was very successful.  Dr. Netterville stated he didn't know what kind of tumor it was 100% until he went in.  He stated that many tumors that are thought to be Vagal nerve tumors are actually Sympathetic nerve tumors, which sometimes have a better outcome.  Due to the location of mine he felt it was Vagal and if so I might have no voice or barely a voice for 6 months to a year.  There was the possibility of needing a a feeding tube due to swallowing issues that often arise.  He felt all of these things would be temporary and get better over time.  I was told to plan to be out of work 6-8 weeks. And if I lost my voice completely there were treatment options.  I had it removed March 3, 2013.  It was a 6 hour surgery.  I woke up talking (even though I sounded like i had laryngitis) and asking for food...which i was able to eat as soon as they transported me to ICU.  I was there for 2-3 days (can't remember)...and even though I was in pain I recovered well.  The left side of my head was numb (especially my ear) for about 3 months before I could feel anything.  The sensations slowly came back and the nerve would "jump" a lot...I would have these spells where i would experience a lot of activity (even acne on that part of my neck) and then it would be better than before.  My follow up appointment at 6 months showed slow blood flow in the jugular vein but the doctor was not concerned.  He said that was common.  I went back to work in 3 weeks but should have waited the entire 6 weeks.  My biggest issue was that b/c my vocal cords didn't "close" correctly I lost a lot of air just moving around so I would get out of breath easy. This would cause me to get dizzy and the extra exertion of trying to talk exhausted me at work.  I had serious blood pressure issues at first but that got better...matter of fact my BP was really bad before surgery but they couldn't get it regulated...it would go to low on meds.  Now I still have spikes but NOTHING like i was experiencing before.  It confused the doctors bc they said if it was bc of tumor it should have dropped but that vaso-vagal response I think for me was hypersensitive and reversed or something I don't know.  Anyway, after surgery it was like that, they thought, because the nerve was healing and it actually got better the more I healed.  Also...by a year I pretty much had my voice back completely.  At my 1 year check-up it was showing that my vocal cords were at 50% (which stunned them and made them excited).  The only symptom I have now is neck pain/pressure (which will trigger a migraine) some pain due to scar tissue, and numbness.  I also had a lot of stomach issues before surgery...they improved about 75%...same with BP.  If I didn't have the migraines I might be OK but I struggle with those which they are certain did not come from the tumor.  

5)   Having gone through the experience, what do you think are the most critical questions for someone to ask their doctor about surgery and treatment of this kind of schwannoma?

Questions about: risks  and long-term side effects, how long it takes to heal as well as reasonable recovery time (the intern that came in to see me on rounds the day I left said in 2 weeks I could return to work...but when I asked the Resident and Dr. N they said to take as long as I needed; he even asked on a follow-up phone call if he needed to take me back out of work so if you go to a teaching hospital you should make sure that you are expressing any discomfort or lack of understanding to everyone including the surgeon...the interns and residents are still learning...and they are learning from you! 

Ask what would happen should the surgeon feel like he couldn't save the nerve (Dr. N assured me that if it was that bad he would close me up and we would possibly do some radiation). 

Ask what to expect as far as normal and not-normal "side effects" (They really don't prepare you for how long it takes to heal...I notice in these groups people asking how long before this gets better and I was the same way.  Even my surgeon and residents didn't fully prepare me probably bc each experience is different and they don't experience it themselves so they really don't know since you leave the hospital and only call if there is a major issue.  

their success rate (get some success stories)

I WOULD TELL EVERYONE TO GO TO VANDERBILT.  YOU CAN'T LOSE GOING THERE...HE SPENDS A TON OF TIME EXPLAINING THE ENTIRE PROCESS. 

6)   Can you describe what the recovery process was like for you – if you feel you  have made a positive recovery…and what things you have done that have been of most help to you?  (Also, you are free to describe what doctors tell you to expect from your recovery if you haven't been in surgery yet)

I've described a lot in the questions above but I didn't mention the drain tube.  They will show your family how to remove and when but be sitting down because you might pass out...I did.  My daughter cut the stitches and did exactly as they taught her but because the tube is near the ear under the skin it gave me a very sick queasy dizzy feeling and I passed out....LOL. That still makes me sick to think about it.   I will say that I would be hard on myself if I laid around too much and I expected my body to heal way faster than it could.  So I would say take the max time out of work...don't push yourself...and recognize that it may take 2 years or more to finish healing...I feel like I'm still healing.  Don't be afraid to ask for help with pain in the beginning...and you may have trouble sleeping because you cant sleep on that one side for awhile because you can't feel it.  What got me through was noticing little changes, as well as stuff I could do to keep from going stir crazy while resting...good books, xbox, my tablet, music.  And don't be afraid to call your surgeon to ask questions.  You never know if what you are experiencing is normal or not unless you ask.  

7)  Are you able to do all or most the things you did before? (Also feel free to describe your abilities even if you are still living with the tumor or tumors)

After 2 years I can now say "yes".  It took a long time to feel like I was completely back to myself.  I still get out of breath easier that before and have issues with the area around the tumor "aching" and I lose my voice quicker at games and concerts but for the most part I'm back to myself.   And I get dizzy if I have any pressure in that area (like if I hug someone tall...lol) The most significant part of my recovery is that I am able to sing again, which I was told my voice would most likely not be back the way it was before but it is! 

Intracapsular Enucleation, A Short Blog on a Promising Treatment - Especially for Vagal Schwannomas

Sometimes it happens that people get the unwelcome news that their schwannoma is in a difficult location - or that the prospects for complete removal are unlikely.  In those cases, schwannoma patients have to consider their options, whether that may be any form of less invasive procedure, like Cyber-Knife, or even a partial removal or 'de-bulking' of their tumor.

Another kind of surgical treatment that has shown great promise, especially in the treatment of Vagal Schwannomas (schwannomas of the vagus nerve) is called Intracapsular Enucleation, or I.E., for short.  Vagal schwannomas (The Vagus nerve is also known formally as the tenth cranial nerve) present themselves often as a mass in the neck, and though a variety of symptoms are often present, a hoarseness of voice is one such symptom that is commonly reported, along with some dizziness and vertigo issues.  (I know of the case of a young male you had a Vagal schwannoma that displaced his jugular vein, causing woozy feelings as his blood flow was being interrupted by the mass pressing into the jugular vein)

However one doctor's name has come up over and over again when researching the issue of vagal schwannomas, and this is Dr. James Netterville, of Vanderbilt University Medical center in Tennessee.  Dr Netterville has become a leading expert in the usage of Intracapsular Enucleation treatment of Vagal Schwannomas, and people who have been treated by him report often better than average results.  You can see his page and profile here at:

http://www.vicc.org/dd/display.php?person=james.netterville

One lady who is going to soon do a case study for the Schwannoma Survivors & Schwannoma Fighters blog stated that she had her Vagal Schwannoma removed by Dr Netterville, using I.E. surgical treatment, and she was still even able to sing afterwards!  In other cases when conventional removals of Vagal Schwannomas have been done - people sometimes emerge from surgery often with a voice that is very weak.  I have read cases where people could not raise their voices above a whisper - or that their voice was no longer normal, and constantly sounded hoarse.  

Why is it important for Intracapsular Enucleation to be well known for Schwannomas - especially Vagal Schwannomas?  From what I can glean from the medical literature it works like this; when the I.E. treatment is performed, the surgeon makes an incision in the "capsule" that surrounds the schwannoma.  (By capsule, we could say in layman's terms that it is something like a thin lining around the exterior of the tumor), and then he or she proceeds to slowly excise the schwannoma from its own capsule.  The schwannoma is then removed, and the theory of why I.E. works appears to demonstrate that often less damage to the surrounding nerve fibers themselves is done by this method, than by the excision of the entire schwannoma, capsule and all - in a more conventional style surgical removal.  Rhonda Baksh, who runs the Schwannomas of the Head and Neck Facebook page, offered her feedback and did reiterate that when schwannomas are removed by this technique, some risk of regrowth is present - and in some patients Gamma Knife or Cyber Knife treatment may be performed to finish off any remaining Schwann cells from the original tumor.  As well, do ask your consulting physician about the risks of regrowth, and what the plan might be should that occur.

So, what are the lessons to be learned?  In short, if you have a vagal (or Tenth-Nerve) schwannoma - please inquire if there is a doctor in your region who uses Intracapsular Enucleation treatment for removal of vagal schwannomas.  Also, if you have the means to do so, inquire with Dr. Netterville's office to see if you are able to go and meet with him for a consult.  I have also been told of the names of three different neurosurgeons on staff at Johns Hopkins, in Baltimore, MD - who all practice Intracapsular Enucleation treatments.  These are Dr. Tamargo, Dr Weingart, and Dr Brem. 

Here are some articles from medical journals that have been very informative:

Schwannoma in Head and Neck: Preoperative ImagingStudy and Intracapsular Enucleationfor Functional Nerve Preservation 

Advantages of intra-capsular micro-enucleation of schwannoma arising from extremities

(A few notes to end this article...I have been in touch with Dr. Netterville and we hope to collaborate on a Q and A article, and get some of his feedback on the necessary information contained in this article.  We've just not yet been able to manage it, schedule wise.  I have also spoken to a surgeon at Johns Hopkins on this same subject.  I plan to amend this article with further information as it comes in to me - but I feel that it is critical to share necessary information as soon as possible.  Expect that this article may be amended and updated - as it is a personal policy of mine to update any published blog article with new, better, or more accurate information, as soon as I learn of it.  Neil @ the Schwannoma Survivors & Schwannoma Fighters page.)

Alguns Fatos Básicos Sobre Schwannomas Para Aqueles que são Diagnosticados com Esses Tumors

Introdução

 Trabalho com o grupo "Schwannoma Survivors & Schwannoma Fighters" e respondo a muitas perguntas e mensagens na tentativa de esclarecer questões básicas sobre Schwannomas para as pessoas que são recém-diagnosticados. Algumas das perguntas e respostas são muitas vezes bastante semelhantes, então pensei que seria uma boa idéia simplesmente colocar alguns dos princípios básicos sobre Schwannomas, uma espécie de guia introdutório para uma pessoa que nada sabe sobre Schwannomas. Abaixo segue o básico que eu posso compartilhar para ajudar aqueles recém-diagnosticados! - Neil

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1) O que é um Schwannoma?

 

O Schwannoma é um tumor da bainha do nervo que é composta de células de Schwann. Estas células são conhecidas, em casos raros, por crescer anormalmente e causar os tumores que conhecemos como schwannomas.

 

2) Quais são os sintomas de um Schwannoma?

 

Como uma pessoa que não é médico, mas que é um sobrevivente e alguém que trabalha com sobreviventes - Eu posso compartilhar com vocês que os sintomas mais comuns que ouço são dor extrema, com dormência, perda de sensibilidade nos nervos, e, por vezes, extrema fraqueza nas áreas do corpo afetadas pela Schwannoma. As pessoas não relatam choques nervosos, e sentimentos como sensação de formigueiro. (Eu tinha tudo isso enquanto eu estava sofrendo com um Schwannoma)

 

3) Os Schwannoma são benigno ou maligno?

 

 Neurocirurgiões me afirmaram que a grande maioria dos Schwannomas são benignos. Porém devemos enfatizar que eles ainda podem causar problemas na vida de uma pessoa. Geralmente eles aparecem como um unico tumor e não vários. Embora seja rara a malignidade é, a natureza exacta do tumor é geralmente confirmado por biópsia que é conduzida depois que alguma parte ou todo o tumor seja removido por meio de cirurgia. (Em alguns casos, quando chegar ao tumor é mais fácil, uma biopsia pode ser realizada antes da cirurgia para determinar a natureza exacta do tumor).

 

4) Os Schwannomas se transformam em câncer?

 

Na grande maioria dos casos, câncer e Schwannoma não estão relacionados. Um neurocirurgião confirmou-me que normalmente só aqueles que têm a condição de neurofibromatose estão em um risco mais elevado de desenvolver tumores com células canceriginas. Quando as pessoas ouvem a palavra 'tumor' eles tendem a pensar CÂNCER! Schwannomas são diferente. Obviamente, você deve consultar com o seu médico sobre a natureza exata de seu próprio tumor, se você foi diagnosticado - mas para nosso propósito de falar em termos gerais, é suficiente dizer que casos de Schwannomas e câncer é extremamente raro. Os médicos vão usar todos os meios à sua disposição para tentar determinar com precisão a natureza do tumor, e biópsias pós-operatórias são normalmente realizadas para determinar com certeza o tipo de tumor.

 

5) Se eu tiver vários Schwannomas, o que está acontecendo?

 

Embora esta é uma pergunta melhor respondida por um médico com experiência, se você tiver sido diagnosticado com múltiplos Schwannomas, existe uma chance de que você possa ter a condição chamada Schwannomatosis. Schwannomatosis, em geral é uma condição genética que predispõe uma pessoa a múltiplos Schwannomas que podem ocorrer ao longo de quase todo o sistema nervoso - exceto ao longo dos nervos auditivos neuromas acústicos onde / schwannomas vestibulares às vezes crescem. Um teste genético está disponível para ajudar a confirmar Schwannomatosis - no entanto, me foi dito que essa condição pode ser diagnosticada clinicamente pelo médico e tratada de acordo, tendo-se feito ou não o teste genético.

 

6) Onde os Schwannomas podem crescer no corpo?

 

Simplificando, eles podem, teoricamente, crescer ao longo de todo o systema nervoso. Trabalhando com os sobreviventes tenho visto que isso é verdade, e os tratamentos têm de ser orientados para as pessoas de forma diferente com base em onde seu Schwannoma está localizado, e como ele está afetando o corpo.

 

7) Quais são as opções de tratamento para Schwannoma?

 

A opção de tratamento mais comum é a cirurgia. Opções de tratamento mais recentes, como tratamentos de Cyber knife e tratamentos minimamente invasivos estão se tornando mais comum - no entanto, o que eu vejo no grupo de sobreviventes é que um número considerável de pessoas ainda têm a cirurgia convencional como alternative para remover seus Schwannomas. As opções de tratamento são sempre decididas com a ajuda de seu médico - a estratégia pode ser diferente com base na localização da Schwannoma, o tamanho, e na medida em que afecta a qualidade de vida de uma pessoa. E medicação é muitas vezes necessária para controlar a dor causada pelo tumor, e para tratar as interferências no funcionamento normal do nervo por a compressão do tumor no nervo.

 

8) Uma pessoa pode ter uma boa qualidade de vida após a cirurgia de remoção de um Schwannoma?

 

Sim, isso definitivamente é possível. Eu, por exemplo, tenho uma qualidade de vida muito melhor sem a dor extrema do Schwannoma que eu sofria. No entanto, uma cirurgia em em qualquer parte do corpo é arriscado, e haverá sempre algum risco de perda de função ou dor residual resultante da cirurgia. Também depende da pressão que o próprio Schwanoma exerceu no nervo afetado. A pessoa tem que avaliar as perspectivas de resultado da cirurgia em consulta com o seu médico. Muitas das pessoas no grupo “Schwannoma Survivors” ainda têm dor residual e alguns problemas mesmo após a cirurgia de remoção do Schwannoma - eu digo às pessoas que eu sou cerca de 85% tão bom como eu estava nos meus melhores dias, antes de ser afetado pela Schwannoma. Isso é certamente aceitável para mim, pessoalmente. Não, nem todas as histórias de remoção são feliz - mas há razões para ficar otimista, devido ao número significativo de remoções de sucesso através de cirurgia, e as pessoas que tiveram uma boa recuperação de sua qualidade de vida após a cirurgia.

(Translation from English by Vitor de Souza Carneiro)

Que Devrait-on Demander au Médecin si on Vient Juste d’être Diagnostiqué Avec un Schwannome?

Bonjour à tous! Mon nom est Neil et je suis coordinateur du groupe "Schwannoma Survivors & Schwannoma Fighters". En 2009, lorsqu’ on m'a diagnostiqué avec un schwannome,  je disais souvent aux gens que malgré le fait que je suis un gars avec de très bonnes connaissances générales, je n’avais JAMAIS entendu le mot ‘’schwannome’’ de ma vie. En effet, il s’agît de quelque chose qui n’est vraiment pas bien connu du grand public. J’ai donc créé la page et le blog Schwannoma Survivors sur Facebook dans le but de lever le voile sur les schwannomes et les nouveaux traitements disponibles. Par le fait même, cela permet de mieux répondre à des questions pratiques dans le but que nous, en tant que patients, soyons davantage informés quand nous nous asseyons pour parler avec notre propre médecin. 

J’ai donc pensé qu’il serait utile d’élaborer ce blog avec des questions simples et utiles qu'une personne nouvellement diagnostiquée souhaiterait peut-être demander à son médecin. Voici quelques suggestions: _________________________________________________________________ 

1)  Qu'est-ce qu'un schwannome et combien en avez-vous examiné durant vos années de pratique médicale? 

Pourquoi cette question? Je pose cette question parce que les médecins ont divers degrés d'expérience en ce qui concerne leur propre capacité à traiter les schwannomes. Certaines personnes m’ont confirmé, certains dans des termes sans équivoque, qu'ils ont rencontrés des médecins qui avaient très peu d'expérience à l’égard des schwannomes. En général, ce sont les neurochirurgiens qui ont les meilleurs résultats dans le traitement ou la suppression des schwannomes, car ils sont ceux avec le plus d'etudes de cas avec ce qui peut être nécessaire pour traiter le schwannome que vous avez. 

2) Où est situé mon schwannome? 

Pourquoi cette question? L’endroit où est localisé un schwannome a beaucoup à voir avec une foule d’éléments, y compris le degré de difficulté à être retiré. La localisation du schwannome a un degré d'impact sur les nerfs environnants. Tout dépendant ou il se trouve, cela fait varier le déficit que cela peut engendrer. Par conséquent, le pronostic du patient  après l'ablation chirurgicale peut varier en rapport à la position du schawannome dans le corps. Le meilleur moyen pour évaluer la situaton est de passer une IRM avec contraste. 

3) Pourquoi est-ce que je ressens ces symptômes? 

Pourquoi cette question? Via cette interrogation, le médecin pourra, espérons-le, nous aider à comprendre pourquoi ou comment le schwannome interfère avec le bon fonctionnement des nerfs touchés dans notre corps.  

4)  Quelle est la taille du schwannome que j’ai? 

Pourquoi cette question? C'est un élément essentiel : la taille du schwannome est un facteur critique dans la difficulté ou la facilité de son retrait et le degré d’impact avec lequel il interfère avec vos nerfs ou d'autres organes avoisinants sur lequel il peut créer de la pression lorsqu’il grossit. Ceci est également important dans l’établissement d'un pronostic post chirurgical. 

5)  Quelles sont mes options chirurgicales? Et est-ce que le Cyber-couteau une option de traitement viable pour moi? 

Pourquoi ces questions? Dans la grande majorité des cas, la résection, à savoir, la chirurgie conventionnelle est utilisée pour retirer le Schwannome du nerf affecté. De nouveaux traitements sont disponibles, dont le traitement Cyber-couteau. J'ai personnellement entendu très peu de cas traités avec le Cyber-couteau, mais il est raisonnable de penser que ce genre de procédure moins invasive sera plus avancée dans l'avenir, et sera utilisé avec plus de régularité. Il est toujours souhaitable de demander si une forme moins invasive de chirurgie ou de traitement est une option viable. Vous pouvez également demander si le traitement chirugical par énucléation intracapsulaire (E.I.) est une option si vous avez un schwannome dans un endroit particulièrement restreint et difficile à atteindre. En effet, ce type de traitement chirurgical (E.I) a été utilisé à plusieurs reprises avec succès sur les schwannomes affectant le nerf vague, ainsi que sur certains autres schwannomes qui se trouvent dans des endroits ou la chirurgie conventionnelle est plus problématique ou très risqué. 

6)  Combien avez-vous retiré schwannomes? 

Pourquoi cette question? Un autre chirurgien m'a dit que cela est une bonne question à poser, en particulier à votre neurochirurgien. Ce dernier devrait immédiatement dire à un patient si oui ou non ils ont auront un chirurgien qui est expérimenté avec schwannomes. La logique veut que vous ne voulez pas être le cobaye de quelqu'un. Donc, posez la question! ...cela vaut la peine!  

7) Quel pourcentage de mes capacités puis-je retrouver suite à la chirurgie? 

Pourquoi cette question? Pour être tout à fait honnête, beaucoup d'entre nous retrouvons l'ensemble de nos capacités après une chirurgie - mais beaucoup d'entre nous (moi y compris) ne sont pas revenu à 100%. Être en forme parfaite après une chirurgie de schwannome est une chose rare. Il est donc logique de vouloir savoir, autant que possible, ce que votre qualité de vie sera après ce genre de traitement. 

8) Quels sont les risques de la chirurgie? 

Pourquoi cette question? Toutes les chirurgies comportent un niveau de risque. Une intervention chirurgicale sur des nerfs cruciaux comme la moelle épinière par exemple, contient davantage de risques. Un chirurgien m'a déjà dit une fois que certains schwannomes se détachent spontanément. D'autres doivent être découpées. D'autres se révèlent trop difficile à enlever complètement et sont partiellement éliminés. Il est important que le médecin vous réponde, avec autant de précision que possible, quels sont les risques de la chirurgie. Cette information éclaircira l'acte inévitable d'avoir à équilibrer les risques et les récompenses avant de décider de subir une intervention chirurgicale ou d'opter pour une autre alternative. 

9) Quel sera mon temps de récupération? 

Il s'agît d'une question épineuse et il se peut qu'il n'y ait pas de réponse exacte - mais il est toujours bon de la demander au spécialiste. Nous voulons tous savoir combien de temps de notre vie sera hypothèquée pour se remettre d'une chirurgie. Basé sur mon expérience de récupération de chirurgies de schwannome (celle retirées de la moelle épinière ou neurinomes plus particulièrement), cela peut être très long . Les temps de récupération peuvent varier, mais dans l'ensemble elles sont prolongées.

(Translation from English by Mr. Christian Nassar )

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Mon nom est Neil Myers et je suis un artiste demeurant à Tucson, en Arizona. Je suis également le coordinateur du blog et de la page" Schwannoma survivors and Schwannoma  Fighters " sur Facebook. Ce groupe est un réseau de soutien aux patients qui cherchent à obtenir de bons conseils  et qui sont à la recherche de traitements pour les schwannomes . (Je me suis moi-même un survivant ) Bien que nous cherchons toujours à promouvoir des conseils médicaux acceptés et qu'un chirurgien me consulte les informations sur cette page- je tiens à répéter que ces éléments ne sont pas un remplacement par rapport à l'avis de son propre médecin. La page FB ''Schwannoma survivors and Schwannoma Fighters'' et le blog déclinent toute responsabilité pour ses propres décisions médicales personnelles - et tout médecin offrant ses conseils à la page et le blog n'accepte aucune responsabilité . S'il vous plaît prendre toutes les décisions médicales majeures avec votre propre médecin. 

Case Study # 16, Susan Hicks and the Story of Her Left Orbital Schwannoma

Hello everyone! As we have been published Case Studies telling that individual stories of those who have had Schwannomas - we have strived, where possible, to tell the stories of the kinds of Schwannomas that are both common, and some which are less common or downright rare.  A friend of the Schwannoma Survivor page Susan Hicks got in contact with me regarding a rare Left Orbital Schwannoma (This is a Schwannoma behind the eye)...and since hers was one of the first such cases I had heard of, I asked her if she would do a Case Study for us.  She graciously agreed to tell us her story - and we publish it here with the hopes that it may help others.  We thank Susan for her time, and wish her the best in her recovery - Neil
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1) How old were you at the time of your diagnosis? 
I was 51 years old. 2) Do you have any history of Schwannomas in your family? 
No. 3) Where was your Schwannoma located? 
The schwannoma was located in the left orbit. Initially it was labeled as an angioma. And what were the symptoms that lead to your diagnosis?
We had moved recently. I have glaucoma and the new doctor did not like the look of my optic nerve. I have always been told they are small, almost childlike. I went for the MRI he ordered in January 2013. The MRI discovered a tumor by accident; it was not what was expected. 4) Could you describe, in whatever detail possible, what kind of surgical treatment was performed on your Schwannoma, and if you would consider it successful? (Or describe what you may know about the surgery that may yet be performed, if you haven't been operated on yet) 
Initially, they were going in for an angioma, so the procedure was as an outpatient. The Doctor went in at the corner of my left eye. He removed a piece of the skull to get behind they eye. After he got inside he realized it was not an angioma. He removed the tumor and sent it off for biopsy. I was put back together with a titanium plate in the temple. He told me he got 100%. I had double vision for 3 months and the pupil was dilated for 6 months. Then the pupil shrank smaller than normal and no longer responds to light normally. Fast forward 15 months, I was having problems with the left eye. They were minor complaints. Intermittently I would have trouble keeping the eye lid open, at times the eye itself felt like it was pulsing, and increased light sensitivity. I was sent back to the surgeon. He ordered an MRI. There was a 7 MM tumor. Here is where the potential for malignancy is brought up by the doctor. The surgeon sent me back to the neurologist. We waited another 3 months to try and determine if it was growing or what was left behind in the first surgery. We never did a post-surgery MRI. The next MRI showed growth, the tumor was now 1 CM. Now the surgeon and neurologist think malignancy is a real potential. They want to wait another 3 months. I sought out a second opinion at Johns Hopkins. It does take a while to be seen here. At this point, I began to have pain on the left side that would wake me when I slept. Also, a bright room would cause a horrible headache. The next MRI shows marginal growth, now 1.1 CM. Now they want to wait 6 months. I am uncomfortable with this. I kept my Johns Hopkins appointment. This doctor was sure it was a schwannoma again. We discussed the risks of doing nothing, the risk of surgery, and the very off hand chance of malignancy. He recommended surgery. We decided to take this path. I had surgery in March. I was scheduled for an hour but it actually lasted 4 hours. He followed a similar path as the first surgery. The titanium plate was removed and the same pieces of skull were removed. There was so much scar tissue and the tumor was very ingrained in the lateral muscle controlling left and right movement, it turned into more of an event than expected. He also chose not to put the titanium plate back in when he replaced the pieces of skull; he said I would heal faster and with less pain without it. I stayed in the hospital overnight. I can see from that eye but it no longer moves causing double vision. I am also cross eyed on the left. If this does not improve, I will see yet another surgeon in September to center the eye. I may always have some amount of double vision to the side even after the next surgery. He was only able to remove 95% of the tumor. He says it will return but estimates 10 to 20 years. He said sometimes with schwannoma’s you only get only one chance to completely remove them. So we monitor with routine MRI’s. Biopsy reveled it was a schwannoma again. 5) Having gone through the experience, what do you think are the most critical questions for someone to ask their doctor about surgery and treatment of this kind of schwannoma? 
Getting the right doctor with the correct experience is critical. I was misdiagnosed initially. Ask about all the possibilities. Also, get second and third opinions. Ask about their experience with all the possibilities. If you have a tumor in the orbit, seek out a neuro-ophthalmologist! 6) Can you describe what the recovery process was like for you – if you feel you have made a positive recovery…and what things you have done that have been of most help to you? (Also, you are free to describe what doctors tell you to expect from your recovery if you haven't been in surgery yet) 
The first surgery was rough. I was home and probably should not have been. I was not able to function. I was in this state at least two weeks. Someone had to be around constantly. The white of the eye was gone, replaced with blood. My left eye no longer responded to light normally. There was significant damage to the left optic nerve. The left eye was rotated a bit; the new glasses had to have a 10 degree rotation in the left lens. I returned to work 6 weeks post-surgery. The second one was easier. I was up and moving without assistance within 8 hours post-surgery. The eye was crossed but looked fairly normal. There was white to the eye and no blood at all. I was back to work at 2 weeks post-surgery, from home. I began driving at 3 weeks post-surgery. The doctor told us how to see just one image. Place scotch tape over the left lens of the glasses. It works great. At 4 weeks post-surgery, I was back at the office. 7) Are you able to do all or most the things you did before? (Also feel free to describe your abilities even if you are still living with the tumor or tumors)
Most things, I still miss seeing from the left side but it will return. I have significant optic nerve damage future. The tumor will also regrow another unknown. I choose not to sit and worry about any of this. Instead, I choose to live and deal with the bumps as they pop up on my journey.
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A Letter with Info on the After-Surgery Phase, for Acoustic Neuroma Survivors

Hi everyone!  One of the goals of the Schwannoma Survivors page and blog is to try to work to fill in the gaps in our knowledge and understanding about various kinds of Schwannomas.  One such gap - something we hadn't explored as much as we would have liked, is what a person goes through after Acoustic Neuroma / Vestibular Schwannoma surgery.  We have two case studies online where people spoke about Acoustic Neuroma, but recently I got more information on some of the treatments and therapies used after surgery, from a lady who had been through it.  The lady who is speaking in the narrative prefers to stay anonymous, though she gave us permission to publish the content of what she has shared on the blog.  The information that she wrote to another friend of the page was something that I think would be useful to others.  So with thanks to her - and she knows who she is :), I present to you this first person account - a kind of informal letter with some details on the treatments and therapies used after Acoustic Neuroma surgery - Neil
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At the time it was found (in late April / early May 2005), my tumor was the size of an apricot, adhered to the spinal lining, grown into the acoustic nerve, and beginning to tear the facial nerve.  (All I knew until the tumor was discovered was that I was always very subtly off balance, had lost much of my hearing in my left ear, and was feeling very gentle spiders walking along the area near my upper left lip.  I understand only after the second surgery that the spiders-walking feeling was the facial nerve being pressed.  I got the tumor headaches about two weeks after discovering the tumor.)  

In June  I was in surgery to reduce the size of the tumor so that we could do Cyberknife radiation treatment on it.   I was in a lot of pain.  I think I was in the hospital for at least four nights, at my grandmothers house for a night as a staging way to get me back home.   At home I was bedridden for at least a couple more days — I could walk down the hall, barely, using the wall to keep me upright.  Over the next three weeks I walk a little bit further each day.  Getting to the end of the drive with my husband was HUGE, then I went back to bed.   I went back to work on week 5, but only half time for the first two or three weeks.  I’d get to a point around 11am where an easy jig-saw-like task that would typically take me 15 minutes became impossible to figure out.

In November I was back in surgery because even though they pulled out 80% of the tumor the first time, it still wasn’t small enough for Cyberknife.   When I woke from that surgery, the left side of my face was completely unresponsive and I couldn’t hear at all out of my left ear.   I was in much less pain by the time I got home — barely used any of the pain pills.  Some of the experience I described in my email to (name kept anonymous).  I’m happy to give you more info if it helps as when I sent her was related specifically to the paralysis and the physical therapy.  Note, though, my doctor told me not to tape my eye shut as it could scratch the cornea.

In January (2006) I did the Cyberknife treatments.   (Off topic — have I ever shown you the mask made for me for the Cyberknife?  Freaky-cool.   Like Geiger molded my face.)  I was still recovering; I had some very interesting logical lapses during January.  I probably went back to work (the second time) in February.  I probably went to the doctors in March or April (so 4 to 5 months after surgery).    

We think the Cyberknife treatments finished off whatever hearing I had left.   If it means the tumor won’t grow again (which was the aim of the radiation), it’s a good trade.

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*Note from Neil, here is a 2nd part that was written, which she also gave permission to share

Neil and I talk sometimes, in general terms, about the schwannoma page he runs. He mentioned that you were looking for information about what to do post-op, related to facial paralysis. Here’s a little background on me: I underwent surgery for an acoustic neuroma about 10 years ago. Actually, two surgeries, to reduce the size of the tumor. After the second surgery the left side of my face was completely paralyzed. (Don’t blame the doctors. It was a miracle I didn’t have problems earlier.) The doctors and hospital staff were very uninformed about it — the best they had was “most of the time things return to normal in about 3 months.” Four months later I was in to see the nurse practitioner for an unrelated visit. She gave me the name of the physical therapist who worked on facial paralysis (whom she knew about because her son mentioned his therapist did facial work…) So I have no reference for how to find people who deal in facial paralysis but I can tell you some of what he and I did together to improve the movement and symmetry in my face.

We started with a nerve conduction test (I don’t promise that’s what it was actually called, though) which indicated which areas were least / most damaged and therefore most / least likely recoverable. He video taped me talking so we could view facial movement more objectively and selectively.

In our visits he showed me exercises to do which targeted specific muscle movements to work on at home. When I first began, I had no movement in the left side of my mouth outside of what moving my jaw produced. I had to focus on one movement. I think we began with the corner of my mouth. He demonstrated the movement. (Watch someone smile gently, lips closed, with just one side of their mouth.) My job was to relax every muscle in my face then move just the corner of my mouth up. I had to practice this in the mirror every day. I don’t remember if we did just the corner of the mouth then two weeks later did the upper lip movement, then… or if he showed me how to isolate each and I practiced it all every night. But I do remember it was very frustrating, and very difficult to convince myself to go back to it day after day. I also remember when — two weeks later? three? — the corner of my mouth started to move.

At nights I slept attached to a device that sent periodic pulses of electricity to the muscles as a way to stimulate the nerves. The sensation was a bit like being tapped lightly. I can’t remember what the device was called - or really how long I had it. My husband and I recall the timing for this differently. He thinks “not long”, but I’m fairly sure I had this device for close to a year.

The therapist also had me work with a small biofeedback machine for a couple of months. I stuck a pad to a target area on my face and attached the pad to the machine, then focused on muscle movement. We began biofeedback after some of my mouth muscle began moving, and used it as a continuation of that work. So, if I attached it a little above the left side of my mouth, I then relaxed all the muscles in my face and focused on raising the top part of my lip in such a way it registered on the machine.

When I had more muscle movement, he had me practice certain phrases and tongue twisters in the mirror with the focus on keeping the mouth symmetrical when pronouncing letters such as p and r.

The last piece that I recall came several months later, when I’d regained muscle movement and symmetry. We did more muscle isolation exercises specific to counteracting the synkinesis he saw. (Synkinesis, if that’s unfamiliar, is when the nerves get a bit mis-wired and moving one area causes a completely unrelated area to move. For a couple weeks, for me, raising my right (unaffected) eyebrow also caused the left corner of my mouth to move a small amount.)

I don’t remember how long I worked with the therapist, but I’m sure it was over a year. It may have been as long as two. My face is still partially paralyzed, but it is a huge improvement over what I had just after surgery. My eye still doesn’t blink quickly, but that was one of the areas the first test showed unlikely to recover. However, while I cannot grin, I can smile. I can pronounce words properly (just after surgery I couldn’t pronounce my phone number. About the time I began with the therapist I could but needed to speak it slowly.) My face is still asymmetrical, but is not nearly as pronounced as it was before. And I have a suspicion that I’d have much more muscle movement if only I’d been a bit better at convincing myself to do those muscle isolation exercises.

I hope there is some piece here that helps you find the answers to your questions. Facial paralysis I found deeply saddening at first, then just an annoyance.
My best to you as you heal.
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Our thanks to the person who allowed us to share some of this correspondence.  We hope it is of assistance to those of you recovering from acoustic neuroma! - Neil

Schwannomas, a Story of Discounted Tumors and Misunderstood Pain

'Well at least it wasn't cancer!'

'But your tumor was benign, right?'

We've all heard these things said before.  To be quite honest, they are usually said by very well meaning people who try to be encouraging.  (I have also heard it said by doctors who have a more specific reason, wanting to clearly make the medical distinction of what a Schwannoma is, versus cancer).

Certain things have become quite clear to me, after having worked on Schwannoma issues and survived one myself - and that is, that Schwannomas are frequently discounted.  And by that I mean they are not taken as seriously as other tumors or conditions.  I have frequently talked to people on the Schwannoma Survivors page who mention having difficulty helping family and loved ones understand just what they are going through.  The truth is, these tumors can be very, very bad.  They can impact your life and bend every part of your daily experience around the pain and neurological deficits that they create.  So why is it that people discount Schwannomas so frequently?

Part of the problem is the simple fact that they are not very well known.  Go pick ten people off the street and ask them what a Schwannoma is, and there is a good chance that you will run 0 for 10, and find absolutely nobody who knows what they are.  So just making these tumors better known is a paramount goal that we should all try to aspire to.  We have to share our stories, because raising awareness for rare diseases, conditions, and tumors - is always a good thing.  And we will never suffer for our becoming wiser in that regard.

But the ugly truth still prevails...most people have no idea what a Schwannoma is.  So it follows that they will not likely have any kind of mental architecture for coming up with a way to encourage and support someone who has a Schwannoma.  If we mention Cancer, Diabetes, Heart Disease, etc - there is immediately something that people can grasp and there are even entire support networks there to inform and assist families.  Very little, to almost none of that - exists for Schwannoma Survivors.  So, strange as it may seem - it is up to the person with the Schwannoma to often try educate others or help people understand what these insidious tumors can do.  So as we suffer, we have to explain.  We have to try to share what the reality of our lives is like, and we have to pull that word "Schwannoma" out of the dark and put it up in lights.

Awhile back I was doing more online reading on Schwannomas and I came across a discussion where several people mentioned that they didn't like the word "survivor" being used in association with Schwannomas.  Our page and blog are called "Schwannoma Survivors & Schwannoma Fighters" so I took some time to think about it.  Of course, a person may self-identify any way they wish.  They can call themselves a flying goat headed crocodile zombie if they like! :)  How a person self-identifies is very personal, and they have that right to assert whatever label they wish in a non-harmful manner.  All of this called to mind the first thought I had when I thought about Schwannomas being discounted tumors - and that is, that if we have no mental frame of reference for understanding a Schwannoma, especially on a societal level - it would stand to reason that some people may not understand why some of us prefer to call ourselves 'Survivors'.  (And for some, no amount of suffering is enough to raise to this level.)

The simple truth is this; Schwannomas CAN be that bad.  I have read so many examples of the relentless, grinding misery these tumors can cause - I learned early on not to discount them the way others do.  For example, when more than one person says to me that they were at the point of contemplating ending their lives just to get OUT of the pain caused by Schwannomas, that would make most people to sit up and take notice.  These are usually very normal, well adjusted people who love life - but when they have told me that the pain was so unbearable that they were to the point of even contemplating ending their own lives, then we must understand how serious that is.

When people write me and tell me that they have the condition of Schwannomatosis - and that they have more Schwannomas than a doctor can count in their spinal cord alone, then it is really that serious.  When you hear of them speak of multiple surgeries, and still struggling with pain and neurological deficits in multiple points of their bodies, then you can't cheapen or lighten that story.

When someone has an acoustic neuroma...and they detail the experience of loosing hearing on one side, or having their facial muscles paralyzed.  Having to tape their eyelid shut just to sleep.  Having balance problems, nausea, and most of all - carrying on as the wonderful people they are while presenting to the world a face that has been affected by facial paralysis - then it is that serious.  And this is a tumor that often has to be surgically operated on near the base of the skull, just below or beside the brain.  Most of us with normal faces can't properly contemplate the struggle and the courage that it takes to show themselves to the world, while hearing impaired or deaf on one side, struggling to maintain balance, and still mustering the courage, beauty, and dignity to present themselves to the world.  And these amazing people do it again and again each day.  Yes, these tumors are that serious.

When a person can take a strong, narcotic medication in a desperate attempt to reduce pain - and it does almost nothing to reduce the pain...then yes, they can be that serious.

When people like myself tell their stories of spinal cord Schwannomas - and they talk about lying curled up on the bed in pain, unable to lie down flat at night - and therefore, unable to sleep...the twin effects of sleep deprivation and chronic pain ravaging tired bodies and minds, sometimes for years...

When a person tells you about their Cervical Spinal Schwannoma - which leaves shooting pain and weakness down their arms.  When the weakness and nerve shocks affect everything below their necks...

When a person tells you about the damage done by a particularly bad Lumbar or Sacral Schwannoma which leaves them with a permanently debilitated bowel and bladder...

When a neurosurgeon tells one of our page members that the worst Spinal Cord Schwannomas can feel like "The pain of terminal cancer, without the relief of death," then they are really that serious.  (Don't misunderstand me friends, I do not, in return, seek to cheapen the experience of having cancer.  I too have had multiple family members lost to this scourge of a disease.  Almost all Schwannoma Survivors will emerge from the experience with our lives - however the point about the pain Schwannomas cause is striking, and worth taking note.)

When people have Schwannomas in their arms and legs, and the million small gestures we all take for granted...reaching out to pick up something, lifting a leg, standing up, become acts of pain and grit...

Take it from me, the guy who has read the entire inbox of the Schwannoma Survivors & Schwannoma Fighters page - these tumors can be that serious.  No, not all are serious in that way, or to the same extent - but the simple truth is that they CAN be.  A certain number of people have very successful Schwannoma removals, suffer minimal side affects, and are able to resume a healthy and vigorous life.  And for those folks, if the winds of fortune were kinder to you - then we celebrate that and are delighted that you passed through the storm with minimal damage.

But the argument I make is that if a person with a Schwannoma wishes to call themselves a 'Survivor' then that should be respected.  You do not have to call yourself that, if you wish not to.  But respect it if another person does.

For Schwannoma Survivors we have no charity walks.  We have no telethons.  We have no celebrities advocating our cause.  If we are diagnosed with a Schwannoma, it is an extreme rarity if we will even have any relative, friend, or loved one, who even knows what we, or our doctors, are talking about.  And so it's not a stretch to realize, often to our dismay, that finding others to relate to is not easy - and that is why I created the Schwannoma Survivors page and blog.  And that is why I have continued to be inspired and amazed by the gritty, beautiful, and fascinating people that I have met on the page.

We are people who have been handed a burden that almost no one else understands.  We have often felt that we were walking in the dark.  Slowly, as a result of efforts made in the years since, lights have appeared and illuminated other Schwannoma Survivors - and we can look across the space of the internet and at least take comfort in the fact that we are not alone.  There are still those spaces of darkness, ignorance, misunderstanding - and spaces filled with that giant, often prevailing notion that Schwannomas are discounted tumors.  It will be up to us to light those spaces up and help others understand that these tumors can be extremely serious, and they can have far ranging, lasting affects on innocent people's lives.

We can call ourselves whatever we like...

Myself, I am a proud 'Schwannoma Survivor.'

- Neil

*This blog is dedicated to one amazing anonymous survivor and contributor to the Schwannoma Survivors Facebook page and blog.  You know who you are :)